A subacute or chronic inflammatory disease of muscle and skin, marked by proximal muscle weakness and a characteristic skin rash. The illness occurs with approximately equal frequency in children and adults. The skin lesions usually take the form of a purplish rash (or less often an exfoliative dermatitis) involving the nose, cheeks, forehead, upper trunk, and arms. The disease is associated with a complement mediated intramuscular microangiopathy, leading to loss of capillaries, muscle ischemia, muscle-fiber necrosis, and perifascicular atrophy. The childhood form of this disease tends to evolve into a systemic vasculitis. Dermatomyositis may occur in association with malignant neoplasms. (From Adams et al., Principles of Neurology, 6th ed, pp1405-6)
Diseases characterized by inflammation involving multiple muscles. This may occur as an acute or chronic condition associated with medication toxicity (DRUG TOXICITY); CONNECTIVE TISSUE DISEASES; infections; malignant NEOPLASMS; and other disorders. The term polymyositis is frequently used to refer to a specific clinical entity characterized by subacute or slowly progressing symmetrical weakness primarily affecting the proximal limb and trunk muscles. The illness may occur at any age, but is most frequent in the fourth to sixth decade of life. Weakness of pharyngeal and laryngeal muscles, interstitial lung disease, and inflammation of the myocardium may also occur. Muscle biopsy reveals widespread destruction of segments of muscle fibers and an inflammatory cellular response. (Adams et al., Principles of Neurology, 6th ed, pp1404-9)
Inflammation of a muscle or muscle tissue.
In patients with neoplastic diseases a wide variety of clinical pictures which are indirect and usually remote effects produced by tumor cell metabolites or other products.
A diverse group of lung diseases that affect the lung parenchyma. They are characterized by an initial inflammation of PULMONARY ALVEOLI that extends to the interstitium and beyond leading to diffuse PULMONARY FIBROSIS. Interstitial lung diseases are classified by their etiology (known or unknown causes), and radiological-pathological features.
Presence of air in the mediastinal tissues due to leakage of air from the tracheobronchial tree, usually as a result of trauma.
General term for inflammation of adipose tissue, usually of the skin, characterized by reddened subcutaneous nodules.
A vague complaint of debility, fatigue, or exhaustion attributable to weakness of various muscles. The weakness can be characterized as subacute or chronic, often progressive, and is a manifestation of many muscle and neuromuscular diseases. (From Wyngaarden et al., Cecil Textbook of Medicine, 19th ed, p2251)
An enzyme that activates histidine with its specific transfer RNA. EC 6.1.1.21.
Antibodies that react with self-antigens (AUTOANTIGENS) of the organism that produced them.
A form of lupus erythematosus in which the skin may be the only organ involved or in which skin involvement precedes the spread into other body systems. It has been classified into three forms - acute (= LUPUS ERYTHEMATOSUS, SYSTEMIC with skin lesions), subacute, and chronic (= LUPUS ERYTHEMATOSUS, DISCOID).
A glucocorticoid with the general properties of the corticosteroids. It is the drug of choice for all conditions in which routine systemic corticosteroid therapy is indicated, except adrenal deficiency states.
A subtype of striated muscle, attached by TENDONS to the SKELETON. Skeletal muscles are innervated and their movement can be consciously controlled. They are also called voluntary muscles.
Disorders of connective tissue, especially the joints and related structures, characterized by inflammation, degeneration, or metabolic derangement.
Progressive myopathies characterized by the presence of inclusion bodies on muscle biopsy. Sporadic and hereditary forms have been described. The sporadic form is an acquired, adult-onset inflammatory vacuolar myopathy affecting proximal and distal muscles. Familial forms usually begin in childhood and lack inflammatory changes. Both forms feature intracytoplasmic and intranuclear inclusions in muscle tissue. (Adams et al., Principles of Neurology, 6th ed, pp1409-10)
Immunoglobulin preparations used in intravenous infusion, containing primarily IMMUNOGLOBULIN G. They are used to treat a variety of diseases associated with decreased or abnormal immunoglobulin levels including pediatric AIDS; primary HYPERGAMMAGLOBULINEMIA; SCID; CYTOMEGALOVIRUS infections in transplant recipients, LYMPHOCYTIC LEUKEMIA, CHRONIC; Kawasaki syndrome, infection in neonates, and IDIOPATHIC THROMBOCYTOPENIC PURPURA.
Agents that suppress immune function by one of several mechanisms of action. Classical cytotoxic immunosuppressants act by inhibiting DNA synthesis. Others may act through activation of T-CELLS or by inhibiting the activation of HELPER CELLS. While immunosuppression has been brought about in the past primarily to prevent rejection of transplanted organs, new applications involving mediation of the effects of INTERLEUKINS and other CYTOKINES are emerging.
A heterogeneous group of disorders, some hereditary, others acquired, characterized by abnormal structure or function of one or more of the elements of connective tissue, i.e., collagen, elastin, or the mucopolysaccharides.
Removal and pathologic examination of specimens in the form of small pieces of tissue from the living body.
The outer covering of the body that protects it from the environment. It is composed of the DERMIS and the EPIDERMIS.
The noninvasive microscopic examination of the microcirculation, commonly done in the nailbed or conjunctiva. In addition to the capillaries themselves, observations can be made of passing blood cells or intravenously injected substances. This is not the same as endoscopic examination of blood vessels (ANGIOSCOPY).
Permanent dilation of preexisting blood vessels (CAPILLARIES; ARTERIOLES; VENULES) creating small focal red lesions, most commonly in the skin or mucous membranes. It is characterized by the prominence of skin blood vessels, such as vascular spiders.
Works containing information articles on subjects in every field of knowledge, usually arranged in alphabetical order, or a similar work limited to a special field or subject. (From The ALA Glossary of Library and Information Science, 1983)
An immunosuppressive agent used in combination with cyclophosphamide and hydroxychloroquine in the treatment of rheumatoid arthritis. According to the Fourth Annual Report on Carcinogens (NTP 85-002, 1985), this substance has been listed as a known carcinogen. (Merck Index, 11th ed)
Devices, not affixed to the body, designed to help persons having musculoskeletal or neuromuscular disabilities to perform activities involving movement.

Fas and Fas ligand interaction induces apoptosis in inflammatory myopathies: CD4+ T cells cause muscle cell injury directly in polymyositis. (1/553)

OBJECTIVE: To investigate the involvement of the Fas/Fas ligand (Fas/FasL) system in the inflammatory myopathies. METHODS: Frozen muscle sections obtained from 7 patients with polymyositis (PM), 4 patients with dermatomyositis (DM), and 3 controls were studied by immunochemistry. Apoptosis was detected by DNA electrophoresis and in situ labeling using the TUNEL method. RESULTS: Fas was detected on muscle fibers and infiltrating mononuclear cells (MNC) in 6 PM patients and 2 DM patients. FasL was expressed mainly on CD4+ T cells and some CD8+ T cells, and on macrophages surrounding Fas-positive muscles in 4 PM patients and 1 DM patient. In 3 of the 5 patients with FasL-positive MNC, the TUNEL method showed that both invaded myonuclei and MNC underwent apoptosis. Chromosomal DNA from the muscle tissue of these patients showed ladder formation. CONCLUSION: Fas/FasL is involved in muscle cell apoptosis in at least 2 of the inflammatory myopathies, PM and DM. Although CD8+-mediated cytotoxicity is thought to be the main mechanism of muscle injury in PM, our data suggest that CD4+ T cells also directly cause muscle cell damage.  (+info)

Pneumocystis carinii pneumonia in patients with connective tissue diseases: the role of hospital experience in diagnosis and mortality. (2/553)

OBJECTIVE: Pneumonia due to Pneumocystis carinii has been increasingly reported in patients with connective tissue diseases, but the frequency of this complication is not known. We sought to determine the frequency of P carinii pneumonia (PCP) in patients with connective tissue diseases, and to determine the role that a hospital's acquired immunodeficiency syndrome (AIDS)-related experience may have in the diagnosis of PCP in these patients. METHODS: We used a state hospitalization registry to identify all patients with PCP and either rheumatoid arthritis, systemic lupus erythematosus, Wegener's granulomatosis, polymyositis, dermatomyositis, polyarteritis nodosa, or scleroderma who had an emergent or urgent hospitalization in California from 1983 to 1994. We compared patient and hospital characteristics between these patients and patients with connective tissue diseases hospitalized with other types of pneumonia. RESULTS: Two hundred twenty-three patients with connective tissue diseases were diagnosed with PCP in the 12-year study period. The frequency of PCP ranged from 89 cases/10,000 hospitalizations/year in patients with Wegener's granulomatosis to 2 cases/10,000 hospitalizations/year in patients with rheumatoid arthritis. Compared with 5,457 patients with connective tissue diseases and pneumonia due to other organisms, patients with PCP were more likely to be younger, to be male, to have private medical insurance, and to have systemic lupus erythematosus, Wegener's granulomatosis, inflammatory myopathy, or polyarteritis nodosa rather than rheumatoid arthritis, and were less likely to be African American. Hospital size, teaching status, urban/rural location, proportion of admissions due to AIDS or PCP, and proportion of patients with pneumonia undergoing bronchoscopy were each associated with the likelihood of diagnosis of PCP in univariate analyses, but only the number of patients with PCP being treated at a hospital (odds ratio [OR] 1.03 for each additional 10 cases/year, 95% confidence interval [95% CI] 1.01-1.05) was associated with the likelihood of diagnosis of PCP in multivariate analyses. Patients were also somewhat more likely to be diagnosed with PCP if there had previously been a case of PCP in a patient with a connective tissue disease at the same hospital (OR 135, 95% CI 0.98-1.85). In-hospital mortality was 45.7%, and was unrelated to hospital characteristics. CONCLUSION: PCP is an uncommon, but often fatal, occurrence in patients with connective tissue disease. A hospital's prior experience with patients with PCP is associated with the likelihood that this condition is diagnosed in patients with connective tissue diseases who present with pneumonia, suggesting that diagnostic suspicion is an important factor in the correct identification of affected patients.  (+info)

Dermatomyositis associated with invasive thymoma. (3/553)

We report a case of dermatomyositis (DM) associated with invasive thymoma in a 22-year-old woman who was admitted to our hospital complaining of dyspnea which required ventilation support. The reddened elevated scaly eruptions were prominent over the extensor surfaces. Chest X-ray and computed tomography showed mediastinal masses, which were diagnosed as mixed type thymoma. Muscle and skin biopsy specimens were compatible with DM. She was treated with methylprednisolone pulse therapy followed by extended removal of the anterior mediastinal tumor and subsequent radiotherapy. She has had a good clinical course without recurrence of thymoma or DM for more than 3 years. The role of thymoma in the development of DM is discussed.  (+info)

Prevalence and antigen specificity of anti-histone antibodies in patients with polymyositis/dermatomyositis. (4/553)

Anti-histone antibodies have been detected in the sera of patients with various autoimmune diseases. The existence of anti-histone antibodies in patients with polymyositis/dermatomyositis, however, has not been reported. We found anti-histone antibodies in eight (17%) of 46 sera from patients with polymyositis/dermatomyositis by an enzyme-linked immunosorbent assay. One serum was positive for both IgG anti-histone antibodies and IgM anti-histone antibodies. Six sera were positive only for IgG anti-histone antibodies. One serum was positive only for IgM anti-histone antibodies. An indirect immunofluorescence analysis using HEp-2 cells as the substrate showed that all sera positive for anti-histone antibodies produced homogeneous nuclear fluorescence. This immunofluorescence pattern disappeared after absorption of anti-histone activity with total histones. An immunoblotting analysis demonstrated that the anti-histone antibodies were predominantly directed against histone H1 in all seven sera with IgG anti-histone antibodies. Weak reactivity with H2B and H4 were also found in three sera from the patients with polymyositis/dermatomyositis. Sera from two patients with polymyositis/dermatomyositis displayed anti-H2A and H3 activity. One of the two sera showed IgM anti-histone antibodies in the enzyme-linked immunosorbent assay reacted with H1, H2A, H2B, H3, and H4, whereas the other serum reacted with no fractions of total histones. The activity of anti-histone antibodies disappeared in immunoblotting after absorption with total histones. All of the patients with anti-histone antibodies were free from lung fibrosis or internal malignancies. Thus, our data indicate that the presence of anti-histone antibodies is classified as one of the serologic abnormalities observed in polymyositis/dermatomyositis.  (+info)

Genetic risk and protective factors for idiopathic inflammatory myopathy in Koreans and American whites: a tale of two loci. (5/553)

OBJECTIVE: To better understand genetic contributions to autoimmunity, immunogenetic markers were studied in two racially discrete and geographically isolated populations of patients with idiopathic inflammatory myopathy (IIM). METHODS: Clinical characteristics, as well as clinical and autoantibody subsets, were defined in 151 American white patients and 50 Korean patients with IIM. HLA-DRB1 and DQA1 genotyping was performed on patients and racially matched controls by standard molecular techniques. Gm allotypes and phenotypes were determined by the hemagglutination-inhibition method. RESULTS: HLA-DRB1*0301, the linked allele DQA1*0501, and DRB1 alleles sharing the first hypervariable region motif 9EYSTS13 were major genetic risk factors for the development of myositis in whites (corrected P [Pcorr] < 0.0004, odds ratio [OR] 11.2, 4.5, and 3.1, respectively, for each factor versus controls). Although both the white and Korean patients had a similar distribution of clinical characteristics, autoantibody profiles, and clinical groups, no HLA-DRB1 nor DQA1 allele or motif was found to be a risk factor for IIM in the Korean patients. However, DRB1*14 was a protective factor in Korean patients without myositis-specific autoantibodies (Pcorr = 0.004, OR 0.046). In addition, although no Gm phenotype or allotype was identified as a risk factor in whites, Gm 21 was a protective factor for the development of IIM in Koreans (Pcorr = 0.024, OR 0.3). CONCLUSION: Although myositis patients in the US and Korea share similar clinical and serologic features, the immune response genes predisposing to and protecting from myositis in each of these ethnic groups differ at two chromosomal loci. These data suggest that multiple genetic loci should be studied to identify risk and protective factors for some autoimmune diseases in various ethnic populations.  (+info)

Autoantibodies to the extracellular matrix microfibrillar protein, fibrillin-1, in patients with scleroderma and other connective tissue diseases. (6/553)

A duplication in the fibrillin-1 gene has been implicated as the cause of the tight skin 1 (tsk1) phenotype, an animal model of scleroderma or systemic sclerosis (SSc). In addition to the production of abnormal fibrillin-1 protein, the tsk1 mouse also produces autoantibodies to fibrillin-1. Among a population of Choctaw Native Americans with the highest prevalence of SSc yet described, a chromosome 15q haplotype containing the fibrillin-1 gene has been strongly associated with SSc. With a recombinant human fibrillin-1 protein, autoantibodies to fibrillin-1 were detected in the sera of Native American SSc patients that correlated significantly with disease. Abs to fibrillin-1 also were detected in sera from Japanese, Caucasian, and African-American SSc patients. Compared with other ethnic groups, Japanese and Native American SSc patients had significantly higher frequencies of anti-fibrillin-1 Abs. Sera from patients with diffuse SSc, calcinosis, Raynaud's, esophageal dysmotility, sclerodactyly, and telangiectasias syndrome and mixed connective tissue disease also had significantly higher frequencies of anti-fibrillin-1 Abs than sera from controls or patients with other non-SSc connective tissue diseases (lupus, rheumatoid arthritis, and Sjogren's syndrome). Ab specificity for fibrillin-1 was demonstrated by the lack of binding to a panel of other purified autoantigens. The results presented demonstrate for the first time the presence of high levels of anti-fibrillin-1 Abs in a significant portion of patients with SSc.  (+info)

Autoantibodies in connective tissue diseases: clinical significance and analysis of target autoantigens. (7/553)

Systemic connective tissue diseases are characterized by the production of a number of autoantibodies directed against various cellular constituents. These autoantibodies are closely associated with certain diseases and clinical manifestations, and are therefore useful for clinical practice such as to diagnose diseases and to predict clinical subsets, disease activity and prognosis. To understand the etiology and pathogenic mechanisms of connective tissue diseases; it is particularly important to elucidate the structure and function of target autoantigens recognized by these disease-specific autoantibodies. In recent years, the nature of many target autoantigens have been identified using molecular biology approaches. Most of them are intracellular enzymes and regulatory factors necessary for important biological function involved in gene replication, transcription, RNA processing and protein translation. Thus, the studies of autoantibodies are useful not only in clinical medicine but also in basic cellular and molecular biology.  (+info)

Safety of a home exercise programme in patients with polymyositis and dermatomyositis: a pilot study. (8/553)

OBJECTIVES: To investigate whether a home exercise programme could safely be performed by patients with stable, inactive polymyositis (PM) and dermatomyositis (DM), regarding disease activity, muscle function, health status and pain. METHODS: Ten patients with reduced muscle function completed the study. A home exercise programme including exercises for strength in the upper and lower limbs, neck and trunk, for mobility in the upper limbs and moderate stretching was developed. The patients exercised for 15 min and took a 15 min walk 5 days a week during a 12 week period. Assessments included clinical evaluation of disease activity, serum creatinine phosphokinase (CPK) levels, magnetic resonance imaging (MRI) of the quadriceps, repeated muscle biopsy of the vastus lateralis, a muscle function index (FI), a walking test and a health status instrument (the SF 36) performed at the start of the study and after 12 weeks. RESULTS: After 12 weeks of exercise, there were no signs of increased disease activity as assessed clinically, by CPK values, MRI or muscle biopsy findings. On an individual basis, all patients improved regarding muscle function according to the FI, in six cases the improvement reached statistical significance (P < 0.05). A significant improvement regarding muscle function in the upper and lower limbs, walking distance and general health status was achieved. CONCLUSIONS: Our results indicate that this home exercise programme can be safely employed in patients with stable, inactive PM and DM, with beneficial effects on muscle function.  (+info)

Dermatomyositis is a medical condition characterized by inflammation and weakness in the muscles and skin. It is a type of inflammatory myopathy, which means that it causes muscle inflammation and damage. Dermatomyositis is often associated with a distinctive rash that affects the skin around the eyes, nose, mouth, fingers, and toes.

The symptoms of dermatomyositis can include:

* Progressive muscle weakness, particularly in the hips, thighs, shoulders, and neck
* Fatigue
* Difficulty swallowing or speaking
* Skin rash, which may be pink or purple and is often accompanied by itching
* Muscle pain and tenderness
* Joint pain and swelling
* Raynaud's phenomenon, a condition that affects blood flow to the fingers and toes

The exact cause of dermatomyositis is not known, but it is believed to be related to an autoimmune response in which the body's immune system mistakenly attacks healthy tissue. Treatment for dermatomyositis typically involves medications to reduce inflammation and suppress the immune system, as well as physical therapy to help maintain muscle strength and function.

Polymyositis is defined as a rare inflammatory disorder that causes muscle weakness and inflammation (swelling) of the muscles. It primarily affects the skeletal muscles, which are the muscles responsible for voluntary movements such as walking, talking, and swallowing. The onset of polymyositis can occur at any age but is most commonly seen in adults between 31 to 60 years old, with women being slightly more affected than men.

The exact cause of polymyositis remains unknown; however, it is believed to be an autoimmune disorder, where the body's immune system mistakenly attacks its own muscle tissue. Certain factors such as genetics, viral infections, and exposure to certain drugs may contribute to the development of this condition.

Polymyositis can cause various symptoms, including:
- Progressive muscle weakness and wasting, particularly affecting the proximal muscles (those closest to the trunk of the body) such as the hips, thighs, shoulders, and upper arms.
- Difficulty climbing stairs, lifting objects, or rising from a seated position.
- Fatigue and stiffness, especially after periods of inactivity.
- Joint pain and swelling.
- Difficulty swallowing or speaking.
- Shortness of breath due to weakened respiratory muscles.

Diagnosis of polymyositis typically involves a combination of medical history, physical examination, laboratory tests, electromyography (EMG), and muscle biopsy. Treatment usually includes medications such as corticosteroids and immunosuppressants to reduce inflammation and control the immune response. Physical therapy may also be recommended to help maintain muscle strength and flexibility.

If left untreated, polymyositis can lead to significant disability and complications, including respiratory failure, malnutrition, and cardiovascular disease. Early diagnosis and treatment are crucial for improving outcomes and preventing long-term complications.

Myositis is a medical term that refers to inflammation of the muscle tissue. This condition can cause various symptoms, including muscle weakness, pain, swelling, and stiffness. There are several types of myositis, such as polymyositis, dermatomyositis, and inclusion body myositis, which have different causes and characteristics.

Polymyositis is a type of myositis that affects multiple muscle groups, particularly those close to the trunk of the body. Dermatomyositis is characterized by muscle inflammation as well as a skin rash. Inclusion body myositis is a less common form of myositis that typically affects older adults and can cause both muscle weakness and wasting.

The causes of myositis vary depending on the type, but they can include autoimmune disorders, infections, medications, and other medical conditions. Treatment for myositis may involve medication to reduce inflammation, physical therapy to maintain muscle strength and flexibility, and lifestyle changes to manage symptoms and prevent complications.

Paraneoplastic syndromes refer to a group of rare disorders that are caused by an abnormal immune system response to a cancerous (malignant) tumor. These syndromes are characterized by symptoms or signs that do not result directly from the growth of the tumor itself, but rather from substances produced by the tumor or the body's immune system in response to the tumor.

Paraneoplastic syndromes can affect various organs and systems in the body, including the nervous system, endocrine system, skin, and joints. Examples of paraneoplastic syndromes include Lambert-Eaton myasthenic syndrome (LEMS), which affects nerve function and causes muscle weakness; cerebellar degeneration, which can cause difficulty with coordination and balance; and dermatomyositis, which is an inflammatory condition that affects the skin and muscles.

Paraneoplastic syndromes can occur in association with a variety of different types of cancer, including lung cancer, breast cancer, ovarian cancer, and lymphoma. Treatment typically involves addressing the underlying cancer, as well as managing the symptoms of the paraneoplastic syndrome.

Interstitial lung diseases (ILDs) are a group of disorders characterized by inflammation and scarring (fibrosis) in the interstitium, the tissue and space around the air sacs (alveoli) of the lungs. The interstitium is where the blood vessels that deliver oxygen to the lungs are located. ILDs can be caused by a variety of factors, including environmental exposures, medications, connective tissue diseases, and autoimmune disorders.

The scarring and inflammation in ILDs can make it difficult for the lungs to expand and contract normally, leading to symptoms such as shortness of breath, cough, and fatigue. The scarring can also make it harder for oxygen to move from the air sacs into the bloodstream.

There are many different types of ILDs, including:

* Idiopathic pulmonary fibrosis (IPF): a type of ILD that is caused by unknown factors and tends to progress rapidly
* Hypersensitivity pneumonitis: an ILD that is caused by an allergic reaction to inhaled substances, such as mold or bird droppings
* Connective tissue diseases: ILDs can be a complication of conditions such as rheumatoid arthritis and scleroderma
* Sarcoidosis: an inflammatory disorder that can affect multiple organs, including the lungs
* Asbestosis: an ILD caused by exposure to asbestos fibers

Treatment for ILDs depends on the specific type of disease and its underlying cause. Some treatments may include corticosteroids, immunosuppressive medications, and oxygen therapy. In some cases, a lung transplant may be necessary.

Mediastinal emphysema is a medical condition characterized by the presence of air or gas within the mediastinum, which is the central compartment of the thorax that contains the heart, esophagus, trachea, bronchi, thymus gland, and other associated structures.

In mediastinal emphysema, the air accumulates in the mediastinal tissues and spaces, leading to their abnormal distention or swelling. This condition can result from various causes, including:

* Pulmonary trauma or barotrauma (e.g., mechanical ventilation, scuba diving)
* Infections that cause gas-forming organisms (e.g., pneumomediastinum)
* Air leakage from the lungs or airways (e.g., bronchial rupture, esophageal perforation)
* Certain medical procedures (e.g., mediastinoscopy, tracheostomy)

Mediastinal emphysema can cause symptoms such as chest pain, cough, difficulty breathing, and swallowing problems. In severe cases, it may lead to life-threatening complications, including tension pneumothorax or mediastinitis. Treatment depends on the underlying cause and severity of the condition.

Panniculitis is a medical term that refers to inflammation of the subcutaneous fat, or the layer of fat located just beneath the skin. This condition can affect people of all ages and genders, although it is more commonly seen in middle-aged women. The inflammation can be caused by a variety of factors, including infections, autoimmune disorders, trauma, and medications.

The symptoms of panniculitis may include:

* Red, painful lumps or nodules under the skin
* Skin lesions that may be tender, warm, or bruised
* Swelling and redness in the affected area
* Fever, fatigue, and malaise (a general feeling of illness)

The diagnosis of panniculitis typically involves a physical examination, medical history, and sometimes a biopsy of the affected tissue. Treatment depends on the underlying cause of the inflammation and may include antibiotics, anti-inflammatory medications, or other therapies. In severe cases, hospitalization may be necessary to manage symptoms and prevent complications.

Muscle weakness is a condition in which muscles cannot develop the expected level of physical force or power. This results in reduced muscle function and can be caused by various factors, including nerve damage, muscle diseases, or hormonal imbalances. Muscle weakness may manifest as difficulty lifting objects, maintaining posture, or performing daily activities. It is essential to consult a healthcare professional for proper diagnosis and treatment of muscle weakness.

Histidine-tRNA ligase is an enzyme involved in the process of protein synthesis, specifically during the step of translation. Its primary function is to catalyze the attachment of the amino acid histidine to its corresponding transfer RNA (tRNA) molecule. This enzyme does this by forming a ester bond between the carboxyl group of histidine and the 3'-hydroxyl group of the tRNA, creating a charged histidine-tRNA complex.

The histidine-tRNA ligase enzyme plays a crucial role in maintaining the accuracy of protein synthesis, as it ensures that only the correct amino acid is attached to its specific tRNA. This helps to prevent errors in the genetic code and contributes to the proper folding and functioning of proteins.

The systematic name for this enzyme is "histidine:tRNA(His) ligase (AMP-forming)" and it belongs to the family of ligases, specifically the aminoacyl-tRNA ligases. The gene that encodes this enzyme in humans is known as HARS1 (Histidyl-tRNA Synthetase 1). Defects or mutations in this gene can lead to various genetic disorders, such as histidinemia and Charcot-Marie-Tooth disease.

Autoantibodies are defined as antibodies that are produced by the immune system and target the body's own cells, tissues, or organs. These antibodies mistakenly identify certain proteins or molecules in the body as foreign invaders and attack them, leading to an autoimmune response. Autoantibodies can be found in various autoimmune diseases such as rheumatoid arthritis, lupus, and thyroiditis. The presence of autoantibodies can also be used as a diagnostic marker for certain conditions.

Cutaneous Lupus Erythematosus (CLE) is a skin manifestation of Systemic Lupus Erythematosus (SLE), an autoimmune disease, but it can also occur without systemic involvement. It is characterized by various skin lesions that differ in appearance and distribution. The three main subtypes of CLE are:

1. Acute Cutaneous Lupus Erythematosus (ACLE): This form is typically associated with SLE and is characterized by a classic malar or "butterfly" rash on the face, which is often photosensitive and can be accompanied by discoid lesions. The rash may also appear on other sun-exposed areas of the body.

2. Chronic Cutaneous Lupus Erythematosus (CCLE): This subtype includes Discoid Lupus Erythematosus (DLE) and other less common forms such as lupus panniculitis and chilblain lupus. DLE is characterized by well-circumscribed, erythematous, scaly plaques that can cause scarring and pigmentation changes, often found on the face, scalp, and ears. Lupus panniculitis presents as deep subcutaneous nodules or indurated plaques, typically located on the trunk and proximal extremities. Chilblain lupus is characterized by violaceous, tender, and swollen lesions on acral areas, often triggered by cold exposure.

3. Subacute Cutaneous Lupus Erythematosus (SCLE): This form of CLE presents as non-scarring, papulosquamous or annular polycyclic rashes, often located on the trunk and proximal extremities. The lesions are typically photosensitive and may appear in patients with SLE or those with isolated cutaneous disease.

The diagnosis of Cutaneous Lupus Erythematosus is based on clinical presentation, histopathological findings, and sometimes direct immunofluorescence. Treatment depends on the severity and extent of skin involvement and may include topical therapies, antimalarials, corticosteroids, immunomodulatory agents, or photoprotection measures.

Prednisolone is a synthetic glucocorticoid drug, which is a class of steroid hormones. It is commonly used in the treatment of various inflammatory and autoimmune conditions due to its potent anti-inflammatory and immunosuppressive effects. Prednisolone works by binding to specific receptors in cells, leading to changes in gene expression that reduce the production of substances involved in inflammation, such as cytokines and prostaglandins.

Prednisolone is available in various forms, including tablets, syrups, and injectable solutions. It can be used to treat a wide range of medical conditions, including asthma, rheumatoid arthritis, inflammatory bowel disease, allergies, skin conditions, and certain types of cancer.

Like other steroid medications, prednisolone can have significant side effects if used in high doses or for long periods of time. These may include weight gain, mood changes, increased risk of infections, osteoporosis, diabetes, and adrenal suppression. As a result, the use of prednisolone should be closely monitored by a healthcare professional to ensure that its benefits outweigh its risks.

Skeletal muscle, also known as striated or voluntary muscle, is a type of muscle that is attached to bones by tendons or aponeuroses and functions to produce movements and support the posture of the body. It is composed of long, multinucleated fibers that are arranged in parallel bundles and are characterized by alternating light and dark bands, giving them a striped appearance under a microscope. Skeletal muscle is under voluntary control, meaning that it is consciously activated through signals from the nervous system. It is responsible for activities such as walking, running, jumping, and lifting objects.

Rheumatic diseases are a group of disorders that cause pain, stiffness, and swelling in the joints, muscles, tendons, ligaments, or bones. They include conditions such as rheumatoid arthritis, osteoarthritis, systemic lupus erythematosus (SLE), gout, ankylosing spondylitis, psoriatic arthritis, and many others. These diseases can also affect other body systems including the skin, eyes, lungs, heart, kidneys, and nervous system. Rheumatic diseases are often chronic and may be progressive, meaning they can worsen over time. They can cause significant pain, disability, and reduced quality of life if not properly diagnosed and managed. The exact causes of rheumatic diseases are not fully understood, but genetics, environmental factors, and immune system dysfunction are believed to play a role in their development.

Inclusion body myositis (IBM) is a rare inflammatory muscle disease characterized by progressive weakness and wasting (atrophy) of skeletal muscles. The term "inclusion body" refers to the presence of abnormal protein accumulations within muscle fibers, which are observed under a microscope during muscle biopsy. These inclusions are primarily composed of aggregated forms of amyloid-β and tau proteins, similar to those found in neurodegenerative disorders like Alzheimer's disease.

IBM typically affects individuals over 50 years old, and it is more common in men than women. The disease usually starts with weakness in the wrist and finger flexors, making it difficult to perform tasks such as gripping, buttoning shirts, or lifting objects. Over time, the weakness spreads to other muscle groups, including the thigh muscles (quadriceps), resulting in difficulty climbing stairs or rising from a seated position.

The exact cause of inclusion body myositis remains unclear; however, both immune-mediated and degenerative mechanisms are believed to contribute to its pathogenesis. Currently, there is no cure for IBM, and treatment options are primarily aimed at managing symptoms and improving quality of life. Immunosuppressive medications may be used to target the inflammatory component of the disease; however, their efficacy varies among patients. Physical therapy and exercise programs can help maintain muscle strength and function as much as possible.

Intravenous Immunoglobulins (IVIG) are a preparation of antibodies, specifically immunoglobulins, that are derived from the plasma of healthy donors. They are administered intravenously to provide passive immunity and help boost the immune system's response in individuals with weakened or compromised immune systems. IVIG can be used for various medical conditions such as primary immunodeficiency disorders, secondary immunodeficiencies, autoimmune diseases, and some infectious diseases. The administration of IVIG can help prevent infections, reduce the severity and frequency of infections, and manage the symptoms of certain autoimmune disorders. It is important to note that while IVIG provides temporary immunity, it does not replace a person's own immune system.

Immunosuppressive agents are medications that decrease the activity of the immune system. They are often used to prevent the rejection of transplanted organs and to treat autoimmune diseases, where the immune system mistakenly attacks the body's own tissues. These drugs work by interfering with the immune system's normal responses, which helps to reduce inflammation and damage to tissues. However, because they suppress the immune system, people who take immunosuppressive agents are at increased risk for infections and other complications. Examples of immunosuppressive agents include corticosteroids, azathioprine, cyclophosphamide, mycophenolate mofetil, tacrolimus, and sirolimus.

Connective tissue diseases (CTDs) are a group of disorders that involve the abnormal production and accumulation of abnormal connective tissues in various parts of the body. Connective tissues are the structural materials that support and bind other tissues and organs together. They include tendons, ligaments, cartilage, fat, and the material that fills the spaces between cells, called the extracellular matrix.

Connective tissue diseases can affect many different systems in the body, including the skin, joints, muscles, lungs, kidneys, gastrointestinal tract, and blood vessels. Some CTDs are autoimmune disorders, meaning that the immune system mistakenly attacks healthy connective tissues. Others may be caused by genetic mutations or environmental factors.

Some examples of connective tissue diseases include:

* Systemic lupus erythematosus (SLE)
* Rheumatoid arthritis (RA)
* Scleroderma
* Dermatomyositis/Polymyositis
* Mixed Connective Tissue Disease (MCTD)
* Sjogren's syndrome
* Ehlers-Danlos syndrome
* Marfan syndrome
* Osteogenesis imperfecta

The specific symptoms and treatment of connective tissue diseases vary depending on the type and severity of the condition. Treatment may include medications to reduce inflammation, suppress the immune system, or manage pain. In some cases, surgery may be necessary to repair or replace damaged tissues or organs.

A biopsy is a medical procedure in which a small sample of tissue is taken from the body to be examined under a microscope for the presence of disease. This can help doctors diagnose and monitor various medical conditions, such as cancer, infections, or autoimmune disorders. The type of biopsy performed will depend on the location and nature of the suspected condition. Some common types of biopsies include:

1. Incisional biopsy: In this procedure, a surgeon removes a piece of tissue from an abnormal area using a scalpel or other surgical instrument. This type of biopsy is often used when the lesion is too large to be removed entirely during the initial biopsy.

2. Excisional biopsy: An excisional biopsy involves removing the entire abnormal area, along with a margin of healthy tissue surrounding it. This technique is typically employed for smaller lesions or when cancer is suspected.

3. Needle biopsy: A needle biopsy uses a thin, hollow needle to extract cells or fluid from the body. There are two main types of needle biopsies: fine-needle aspiration (FNA) and core needle biopsy. FNA extracts loose cells, while a core needle biopsy removes a small piece of tissue.

4. Punch biopsy: In a punch biopsy, a round, sharp tool is used to remove a small cylindrical sample of skin tissue. This type of biopsy is often used for evaluating rashes or other skin abnormalities.

5. Shave biopsy: During a shave biopsy, a thin slice of tissue is removed from the surface of the skin using a sharp razor-like instrument. This technique is typically used for superficial lesions or growths on the skin.

After the biopsy sample has been collected, it is sent to a laboratory where a pathologist will examine the tissue under a microscope and provide a diagnosis based on their findings. The results of the biopsy can help guide further treatment decisions and determine the best course of action for managing the patient's condition.

In medical terms, the skin is the largest organ of the human body. It consists of two main layers: the epidermis (outer layer) and dermis (inner layer), as well as accessory structures like hair follicles, sweat glands, and oil glands. The skin plays a crucial role in protecting us from external factors such as bacteria, viruses, and environmental hazards, while also regulating body temperature and enabling the sense of touch.

Microscopic angioscopy is not a widely recognized or established medical term. However, based on the individual terms, it can be interpreted as the use of a microscope with an angioscope (a type of endoscope used for visualizing the interior of blood vessels) to examine the microscopic structures of the inner walls of blood vessels. This technique would allow for detailed examination of the vasculature at a cellular level, potentially providing valuable information for research and diagnosis of various vascular diseases. However, as this is not a standard medical procedure or term, it's essential to consult the relevant literature or experts in the field for more precise information.

Telangiectasia is a medical term that refers to the dilation and widening of small blood vessels called capillaries, leading to their visibility under the skin or mucous membranes. These dilated vessels often appear as tiny red lines or patterns, measuring less than 1 millimeter in diameter.

Telangiectasias can occur in various parts of the body, such as the face, nose, cheeks, legs, and fingers. They are typically harmless but may cause cosmetic concerns for some individuals. In certain cases, telangiectasias can be a sign of an underlying medical condition, like rosacea, hereditary hemorrhagic telangiectasia (HHT), or liver disease.

It is essential to consult with a healthcare professional if you notice any unusual changes in your skin or mucous membranes, as they can provide appropriate evaluation and treatment recommendations based on the underlying cause of the telangiectasias.

An encyclopedia is a comprehensive reference work containing articles on various topics, usually arranged in alphabetical order. In the context of medicine, a medical encyclopedia is a collection of articles that provide information about a wide range of medical topics, including diseases and conditions, treatments, tests, procedures, and anatomy and physiology. Medical encyclopedias may be published in print or electronic formats and are often used as a starting point for researching medical topics. They can provide reliable and accurate information on medical subjects, making them useful resources for healthcare professionals, students, and patients alike. Some well-known examples of medical encyclopedias include the Merck Manual and the Stedman's Medical Dictionary.

Azathioprine is an immunosuppressive medication that is used to prevent the rejection of transplanted organs and to treat autoimmune diseases such as rheumatoid arthritis, lupus, and inflammatory bowel disease. It works by suppressing the activity of the immune system, which helps to reduce inflammation and prevent the body from attacking its own tissues.

Azathioprine is a prodrug that is converted into its active form, 6-mercaptopurine, in the body. This medication can have significant side effects, including decreased white blood cell count, increased risk of infection, and liver damage. It may also increase the risk of certain types of cancer, particularly skin cancer and lymphoma.

Healthcare professionals must carefully monitor patients taking azathioprine for these potential side effects. They may need to adjust the dosage or stop the medication altogether if serious side effects occur. Patients should also take steps to reduce their risk of infection and skin cancer, such as practicing good hygiene, avoiding sun exposure, and using sunscreen.

Self-help devices, also known as assistive devices or adaptive equipment, are tools that help individuals perform activities of daily living (ADLs) that have become difficult or impossible due to disability, injury, or aging. These devices can help improve a person's independence, safety, and quality of life by reducing the physical demands of daily tasks and compensating for functional limitations.

Examples of self-help devices include:

1. Mobility aids: walkers, canes, crutches, wheelchairs, scooters, and prosthetics that help with mobility and balance.
2. Bathroom aids: raised toilet seats, shower chairs, grab bars, and non-slip mats that help with bathing and toileting.
3. Dressing aids: button hooks, zipper pulls, reachers, and dressing sticks that help with dressing and grooming.
4. Kitchen aids: easy-grip utensils, jar openers, and adapted cutting boards that help with meal preparation and cooking.
5. Communication aids: speech-generating devices, communication boards, and hearing aids that help with communication and social interaction.
6. Cognitive aids: memory aids, calendar organizers, and visual cues that help with memory, attention, and executive functioning.

It is important to consult with healthcare professionals, such as occupational therapists or physical therapists, to determine the appropriate self-help devices for an individual's specific needs and to ensure proper use and safety.

A form of this disorder that occurs prior to adulthood is known as juvenile dermatomyositis. No cure for dermatomyositis is ... A given case of dermatomyositis may be classified as amyopathic dermatomyositis if only skin is affected and no muscle weakness ... Third Edition "Dermatomyositis". Genetic and Rare Diseases Information Center (GARD). Retrieved 11 July 2021. "Dermatomyositis ... "Dermatomyositis (DM)". The Lecturio Medical Concept Library. Retrieved 11 July 2021. "Dermatomyositis Information". National ...
Dermatomyositis is a disease that involves muscle inflammation and a skin rash. Polymyositis is a similar inflammatory ... Dermatomyositis is a disease that involves muscle inflammation and a skin rash. Polymyositis is a similar inflammatory ... Dermatomyositis. rarediseases.org/rare-diseases/dermatomyositis/. Updated 2018. Accessed March 3, 2023. ... The cause of dermatomyositis is unknown. Experts think it may be due to a viral infection of the muscles or a problem with the ...
A Case of Dermatomyositis - Download as a PDF or view online for free ... Similar to A Case of Dermatomyositis(. 20. ). A Case of Hypothyroid Myopathy. Stanley Medical College, Department of Medicine• ... Similar to A Case of Dermatomyositis. A Case of Hypothyroid MyopathyStanley Medical College, Department of Medicine3K. views•47 ... Polymyositis dermatomyositis and inclusion body myositisNilesh Kucha6.7K. views•11. slides ...
Dermatomyositis is an idiopathic inflammatory myopathy (IIM) with characteristic cutaneous findings. It is a systemic disorder ... Calcinosis caused by dermatomyositis in childhood can be observed in patient who had active dermatomyositis 15 years before ... For discussion of dermatomyositis in pediatric patients, see Juvenile Dermatomyositis. For patient education information, see ... Incidence of dermatomyositis and clinically amyopathic dermatomyositis: a population-based study in Olmsted County, Minnesota. ...
... Pediatr Pulmonol. 2013 Oct;48(10):1016-25. doi: 10.1002/ppul.22742. Epub 2012 ... Background: Juvenile dermatomyositis (JDM) is the main cause of chronic idiopathic inflammatory myopathy of autoimmune origin ...
Dermatomyositis is an inflammatory myopathy characterized by symmetrical proximal muscle weakness and skin changes. Muscle ... Hypoxia triggers IFN-I production in muscle: Implications in dermatomyositis Sci Rep. 2017 Aug 17;7(1):8595. doi: 10.1038/ ... Dermatomyositis is an inflammatory myopathy characterized by symmetrical proximal muscle weakness and skin changes. Muscle ... is specifically overexpressed in perifascicular areas of dermatomyositis muscle. The aim of this work was to study if RIG-I ...
A to Z: Dermatomyositis. Dermatomyositis (dur-muh-toe-mi-uh-SY-tis) is an inflammatory muscle disease that weakens muscles and ... Dermatomyositis, a type of inflammatory myopathy, can occur at any age, but is most common in children age 5-15 and adults age ... Dermatomyositis cannot be cured, but it can be treated with anti-inflammatory and immunosuppressant medications, as well as ... Dermatomyositis is a serious disease that can result in life-threatening complications such as cancer, heart disease, lung ...
Current topic: Polymyositis and Dermatomyositis: Care Instructions Create your printer-friendly document by clicking topic ...
Dermatomyositis DMS*CHFadmin2023-08-12T20:40:20-04:00. Test Name: Dermatomyositis DMS. Dermatomyositis is an inflammatory ... See Collie Health 101 page on Dermatomyositis for details on this condition ... Leigh Anne Clark Presentation on Dermatomyositis & Interpreting Risk Assessment Results. The American Shetland Sheepdog ... I urge all Collie people to use this link to become more informed with Dermatomyositis, DMS. https:// ...
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Conclusions: Dermatomyositis and polymyositis are serious diseases with a disease-related mortality of at least 10%. In the ... Ng Y, Ouvrier RA, Wu T. Drug therapy in juvenile dermatomyositis: follow-up study. J Child Neurol1998;13:109-12. ... Disease course was defined as monocyclic, according to the criteria used by Huber et al32 in juvenile dermatomyositis, when the ... Adult onset polymyositis/dermatomyositis: an analysis of clinical and laboratory features and survival in 76 patients with a ...
Dermatomyositis is an idiopathic inflammatory myopathy (IIM) with characteristic cutaneous findings. It is a systemic disorder ... Calcinosis caused by dermatomyositis in childhood can be observed in patient who had active dermatomyositis 15 years before ... For discussion of dermatomyositis in pediatric patients, see Juvenile Dermatomyositis. For patient education information, see ... Incidence of dermatomyositis and clinically amyopathic dermatomyositis: a population-based study in Olmsted County, Minnesota. ...
To find out more about Dermatomyositis life insurance visit The Insurance Surgery today. We are the no.1 UK award winning ... Dermatomyositis life insurance. Underwriting for Dermatomyositis life insurance will depend on several criteria. Insurers may ... Does Dermatomyositis run in your familys medical history?. Some applications for life insurance with Dermatomyositis can be ... Dermatomyositis critical illness cover. In a similar way to life insurance, critical illness cover could be a useful option for ...
Ravulizumab LA is under clinical development by Alexion Pharmaceuticals and currently in Phase III for Dermatomyositis. ... Premium Insights Likelihood of Approval and Phase Transition Success Rate Model - Ravulizumab LA in Dermatomyositis Buy the ... Premium Insights Likelihood of Approval and Phase Transition Success Rate Model - Ravulizumab LA in Dermatomyositis. Buy the ... Ravulizumab LA is under clinical development by Alexion Pharmaceuticals and currently in Phase III for Dermatomyositis. ...
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Core Tip: Dermatomyositis is a rare autoimmune disease. Limited knowledge of the relationship between the disease and gastric ... In this study, we report 2 cases of gastric cancer with dermatomyositis as the first symptom. A literature review was performed ... Treatment of gastric cancer with dermatomyositis as the initial symptom: Two case reports and review of literature. World J ... Treatment of gastric cancer with dermatomyositis as the initial symptom: Two case reports and review of literature ...
Bethany was diagnosed with Juvenile dermatomyositis when she was four after an MRI scan showed that, without treatment, she ...
Cases of dermatomyositis evaluated in a single dermatologists adult autoimmunity practice over a 2.5-year period were ... The objective of this retrospective study was to analyze dermatomyositis skin biopsies for the presence of eosinophils and ... Quantitative Assessment of Eosinophils in Dermatomyositis Skin Biopsies With Correlation of Eosinophils to Pruritus and Other ... Quantitative Assessment of Eosinophils in Dermatomyositis Skin Biopsies With Correlation of Eosinophils to Pruritus and Other ...
We review the characteristics of other cases of anti-PD1 associated dermatomyositis and the recent literature to better ... We present a rare case of dermatomyositis associated with nivolumab therapy for melanoma. Nivolumab is an immune checkpoint ... We present a rare case of dermatomyositis associated with nivolumab therapy for melanoma. Nivolumab is an immune checkpoint ... We review the characteristics of other cases of anti-PD1 associated dermatomyositis and the recent literature to better ...
Dermatomyositis is a rare idiopathic autoimmune disorder of unknown cause affecting approximately 10 out of every million U.S. ... 1 - Dermatomyositis; U.S. Department of Health & Human Services, National Institutes of Health, National Center for ... To learn more about Octagam 10% for the treatment of dermatomyositis, please click here. ... Persistent premature mortality gap in dermatomyositis and polymyositis: a United Kingdom general population-based cohort study ...
... Nordal E.;Pistorio A.;Rygg M.;Giancane G.;Maghnie ... Growth and Puberty in Juvenile Dermatomyositis: A Longitudinal Cohort Study / Nordal E.; Pistorio A.; Rygg M.; Giancane G.; ... To study growth and puberty in a multinational longitudinal prospective cohort of children with juvenile dermatomyositis (DM). ... To study growth and puberty in a multinational longitudinal prospective cohort of children with juvenile dermatomyositis (DM). ...
Various dermatologic manifestations of dermatomyositis. Gottrons papules are seen in image A. Shawl sign is seen in image D. ... Suspect in known dermatomyositis with mild rhythm disturbances, CHF or myocardial fibrosis ... Frequency of specific cancer types in dermatomyositis and polymyositis: a population-based study. Lancet. 2001 Jan 13;357(9250 ... Koler RA and Montemarano A. Dermatomyositis. Am Fam Physician. 2001 Nov 1;64(9):1565-1573. ...
hypomyopathic dermatomyositis. * clinically amyopathic dermatomyositis (CADM) / amyopathic dermatomyositis*anti-melanoma ... Dermatomyositis - Ganzer Fall bei Radiopaedia Dixon A, Dermatomyositis. Case study, Radiopaedia.org (Accessed on 03 Dec 2022) ... Dermatomyositis - Ganzer Fall bei Radiopaedia Kuok Y, Dermatomyositis. Case study, Radiopaedia.org (Accessed on 03 Dec 2022) ... Dermatomyositis - Ganzer Fall bei Radiopaedia Jones J, Dermatomyositis. Case study, Radiopaedia.org (Accessed on 03 Dec 2022) ...
Eleni Tiniakou, a physician at the Johns Hopkins Myositis Center, explains the common symptoms of Dermatomyositis. ... Dermatomyositis presents symptoms in many ways; however, the most common signs are skin, muscle, and lung issues. The skin ... A common complication in any of the rheumatic diseases is Reynauds disease, and Dermatomyositis is no different. ... Eleni Tiniakou, a physician at the Johns Hopkins Myositis Center, explains the common symptoms of Dermatomyositis. ...
Classical dermatomyositisAmyopathic dermatomyositisAdermatopathic dermatomyositis. Disorder Group. Non-genetic systemic disease ... Dermatomyositis. Synonyms: Adult dermatomyositis. A rare idiopathic inflammatory myopathy (IIM) characterized by evocative skin ... Dermatomyositis?. Our RARE Concierge Services Guides are available to assist you by providing information, resources and ... Kezar Reports Disappointing Results from Phase 2 Trial of Zetomipzomib for the Treatment of Dermatomyositis and Polymyositis. ...
Dermatomyositis is an idiopathic inflammatory myopathy (IIM) with characteristic cutaneous findings. It is a systemic disorder ... TNF Blockers Treat Muscles, Skin in Juvenile Dermatomyositis * A Mild Form of Dermatomyositis as a Prodromal Sign of Lung ... Calcinosis caused by dermatomyositis in childhood can be observed in patient who had active dermatomyositis 15 years before ... Incidence of dermatomyositis and clinically amyopathic dermatomyositis: a population-based study in Olmsted County, Minnesota. ...
Juvenile dermatomyositis (JDM) is a systemic, autoimmune inflammatory muscle disorder and vasculopathy that affects children ... Hiketa T, Matsumoto Y, Ohashi M, Sasaki R. Juvenile dermatomyositis: a statistical study of 114 patients with dermatomyositis. ... encoded search term (Juvenile Dermatomyositis) and Juvenile Dermatomyositis What to Read Next on Medscape ... Polymyositis and dermatomyositis (first of two parts). N Engl J Med. 1975 Feb 13. 292(7):344-7. [QxMD MEDLINE Link]. ...
Eleni Tiniakou, a physician at the Johns Hopkins Myositis Center, discusses the process of diagnosing Dermatomyositis. ... Tagged With: Dermatomyositis, Dermatomyositis Education Series. Johns Hopkins Rheumatology. The Division of Rheumatology is a ... Dermatomyositis - Diagnosis , Johns Hopkins. August 1, 2018. By Johns Hopkins Rheumatology The enzymes that indicate ... Eleni Tiniakou, a physician at the Johns Hopkins Myositis Center, discusses the process of diagnosing Dermatomyositis. ...
  • Eighty percent of adults with adult-onset dermatomyositis have a myositis-specific antibody (MSA). (wikipedia.org)
  • Sixty percent of children with juvenile dermatomyositis have a myositis-specific antibody (MSA). (wikipedia.org)
  • Sontheimer RD. Would a new name hasten the acceptance of amyopathic dermatomyositis (dermatomyositis siné myositis) as a distinctive subset within the idiopathic inflammatory dermatomyopathies spectrum of clinical illness? (medscape.com)
  • Gerami P, Schope JM, McDonald L, Walling HW, Sontheimer RD. A systematic review of adult-onset clinically amyopathic dermatomyositis (dermatomyositis siné myositis): a missing link within the spectrum of the idiopathic inflammatory myopathies. (medscape.com)
  • Dermatomyositis (DM) is inflammatory myopathy or myositis characterized by muscle weakness and skin manifestations. (biomedcentral.com)
  • Dermatomyositis sine myositis Amyopathic dermatomyositis is a form of dermatomyositis characterized by the presence of typical skin findings without muscle weakness. (wikimd.com)
  • the International Myositis Assessment and Clinical Studies Group & Paediatric Rheumatology International Trials Organisation 2017, ' 2016 ACR-EULAR adult dermatomyositis and polymyositis and juvenile dermatomyositis response criteria-methodological aspects ', Rheumatology (United Kingdom) , vol. 56, no. 11, pp. 1884-1893. (elsevierpure.com)
  • Performance of the 2016 ACR-EULAR myositis response criteria in juvenile dermatomyositis therapeutic trials and consensus profiles. (bvsalud.org)
  • Bendewald MJ, Wetter DA, Li X, Davis MD. Incidence of dermatomyositis and clinically amyopathic dermatomyositis: a population-based study in Olmsted County, Minnesota. (medscape.com)
  • Sun Y, Liu Y, Yan B, Shi G. Interstitial lung disease in clinically amyopathic dermatomyositis (CADM) patients: a retrospective study of 41 Chinese Han patients. (medscape.com)
  • Provisional amyopathic dermatomyositis: between 6 to 23 months of skin disease without muscle involvement. (hss.edu)
  • While patients with amyopathic dermatomyositis should not have clinically evident muscle weakness, minor muscle abnormalities may be included. (wikimd.com)
  • Notably, this patient had a variant of dermatomyositis called amyopathic dermatomyositis, which manifests without muscle involvement. (wikimd.com)
  • Amyopathic dermatomyositis is a rare disease . (wikimd.com)
  • The patient presented with positive anti-MDA5 and anti-Ro-52 antibodies and a high ferritin level, all of which indicated the presence of clinically amyopathic dermatomyositis (CADM). (authorea.com)
  • Anti-MDA5 autoantibodies (Abs) have been associated with clinically amyopathic and classic dermatomyositis (DM), with rapidly progressive interstitial lung disease (ILD) and poor prognosis in Japanese patients (pts). (acrabstracts.org)
  • 13. Failure to over express MHC-CLASS-1 on muscle biopsy in a case of amyopathic juvenile dermatomyositis. (nih.gov)
  • 20. Juvenile amyopathic dermatomyositis. (nih.gov)
  • 8. A case of juvenile dermatomyositis manifesting inflammatory epidermal nevus-like skin lesions: unrecognized cutaneous manifestation of blaschkitis? (nih.gov)
  • Objective: To evaluate the impact of duration of untreated symptoms in children with juvenile dermatomyositis (JDM) on clinical and laboratory status at diagnosis. (northwestern.edu)
  • 5. Juvenile dermatomyositis at diagnosis: clinical characteristics of 79 children. (nih.gov)
  • How can I or my loved one help improve care for people with dermatomyositis? (nih.gov)
  • Tasks that use these muscles: standing from sitting, lifting, and climbing stairs, can become increasingly difficult for people with dermatomyositis. (wikipedia.org)
  • Scientists have already identified some of these molecular targets in people with dermatomyositis, and they include a protein called T1F1γ. (nih.gov)
  • Our findings support the idea that high levels of T1F1γ in regenerating muscle in people with dermatomyositis help drive the damaging inflammatory response," said Dr. Mammen. (nih.gov)
  • Two specific kinds are polymyositis and dermatomyositis. (nih.gov)
  • Polymyositis and dermatomyositis are first treated with high doses of a corticosteroid . (nih.gov)
  • Chow WH, Gridley G, Mellemkjaer L, McLaughlin JK, Olsen JH, Fraumeni JF Jr. Cancer risk following polymyositis and dermatomyositis: a nationwide cohort study in Denmark. (medscape.com)
  • Polymyositis and dermatomyositis (second of two parts). (medscape.com)
  • Several cases of polymyositis and dermatomyositis were reported as being triggered by the use of various statin drugs used to control blood cholesterol. (pinetreeacu.com)
  • Dermatomyositis is part of a group of rare diseases called the inflammatory myopathies that involve chronic (long-standing) muscle inflammation, muscle weakness, and in some cases, muscle pain. (nih.gov)
  • Dermatomyositis (DM) is a long-term inflammatory disorder which affects the skin and the muscles. (wikipedia.org)
  • Your child might need to take prescribed corticosteroids, methotrexate, or other anti-inflammatory medicines to treat inflammation caused by juvenile dermatomyositis. (gillettechildrens.org)
  • Other suspected triggers of dermatomyositis are certain drugs (eg, statins and nonsteroidal anti-inflammatory drugs ) and ultraviolet radiation exposure . (medscape.com)
  • Dermatomyositis is an idiopathic inflammatory myopathy that is characterized by muscle weakness and cutaneous manifestations , although it can also involve other organ systems (such as the pulmonary , cardiac , and gastrointestinal systems). (medscape.com)
  • Polymyositis (PM) and dermatomyositis (DM) are idiopathic inflammatory myopathies that are characterized by inflammation of the muscles, skin, lungs, and heart. (hindawi.com)
  • [2] Autoimmune disorders cause the body's immune system to attack its own cells and tissue, and dermatomyositis is a rare inflammatory disease marked by muscle weakness and a distinctive skin rash. (octapharma.com)
  • Objective: Dermatomyositis (DM) and juvenile dermatomyositis (JDM) are idiopathic inflammatory myopathies affecting multiple organs, including skeletal muscle and skin. (nih.gov)
  • Dermatomyositis (DM) is an inflammatory myopathy characterized by proximal muscle weakness, the presence of perifascicular atrophy (PFA) in the muscle biopsy and skin changes. (biomedcentral.com)
  • Juvenile Dermatomyositis (JDM) is a systemic autoimmune inflammatory muscle disorder and vasculopathy that affects children younger than 16 years old. (biomedcentral.com)
  • Dermatomyositis is an uncommon inflammatory muscular disease, which involves the degeneration of collagen, discoloration and swelling of the skin and underlying muscle. (ipl.org)
  • Dermatomyositis is one of a family of acquired inflammatory muscle diseases. (nih.gov)
  • Dermatomyositis is an idiopathic inflammatory myopathy characterized by skin changes and muscle weakness. (anesth-pain-med.org)
  • Dermatomyositis (DM) is a disease of unknown etiology characterized by typical skin lesions and bilateral, progressive, symmetrical proximal muscle weakness caused by perivascular non-supportive inflammatory processes [ 1 ]. (anesth-pain-med.org)
  • Data to date suggest that the drug may have clinical benefit as well as a beneficial impact on inflammatory and immunological markers in Phase 2 studies in diffuse cutaneous systemic sclerosis, dermatomyositis and cystic fibrosis. (corbuspharma.com)
  • Airio A, Pukkala E, Isomäki H. Elevated cancer incidence in patients with dermatomyositis: a population based study. (medscape.com)
  • Sigurgeirsson B, Lindelöf B, Edhag O, Allander E. Risk of cancer in patients with dermatomyositis or polymyositis. (medscape.com)
  • Factors associated with underlying malignancy in a retrospective cohort of 121 patients with dermatomyositis. (medscape.com)
  • A pilot trial of rituximab in the treatment of patients with dermatomyositis. (medscape.com)
  • Number, characteristics, and classification of patients with dermatomyositis seen by dermatology and rheumatology departments at a large tertiary medical center. (medscape.com)
  • An IFN gene expression "signature" is a candidate biomarker in patients with dermatomyositis (DM). (nih.gov)
  • Coexisting autoantibodies against transcription factor Sp4 are associated with decreased cancer risk in patients with dermatomyositis with anti-TIF1γ autoantibodies. (nih.gov)
  • Early identification and prompt initiation of treatment are needed to reduce the morbidity and mortality faced by patients with dermatomyositis. (medscape.com)
  • Some dermatomyositis patients have little pain, while in others (esp. (pinetreeacu.com)
  • Although the association of JDM and malignancies is not as common as in adult dermatomyositis, malignancies can occur in patients with JDM. (biomedcentral.com)
  • In patients with inflammation and dermatomyositis (DM) widespread across the skin, treatment with baricitinib proved efficacious as early as 4 weeks after therapy began, according to study findings published in a letter in the British Journal of Dermatology . (dermatologyadvisor.com)
  • The last study investigated intravenous immunoglobulins 2 g/kg body weight in 95 patients with dermatomyositis. (medscape.com)
  • To explore this question, Dr. Mammen's team looked for the protein in muscle cell biopsies taken from dermatomyositis patients. (nih.gov)
  • Several reports have documented normal or prolonged neuromuscular blockade following administration of different non-depolarizing neuromuscular blockers in patients with dermatomyositis. (anesth-pain-med.org)
  • The outcomes of this case indicate that response to the usual dosage of muscle relaxants in patients with dermatomyositis might be different from that in patients without this condition. (anesth-pain-med.org)
  • We describe the clinical features of 28 patients with juvenile dermatomyositis (JDM) and 1 patient with adult-onset dermatomyositis (DM), all of whom developed lipodystrophy (LD) that could be categorized into 1 of 3 phenotypes, generalized, partial, or focal, based on the pattern of fat loss distribution. (lvhn.org)
  • Is Anti-NXP2 Autoantibody a Risk Factor for Calcinosis and Poor Outcome in Juvenile Dermatomyositis Patients? (unige.it)
  • To investigate the role of adipokines in adult and pediatric patients with newly diagnosed dermatomyositis (DM), we analyzed peripheral blood and skeletal muscle gene expression of four adipokines: visfatin, leptin, adiponectin and resistin. (biomedcentral.com)
  • Several other measures have been developed and partially validated to assess disease activity and damage in certain target organs, such as the skin or muscle, or in certain populations, such as in patients with juvenile dermatomyositis. (nih.gov)
  • 14. Enzyme elevation in patients with juvenile dermatomyositis and steroid myopathy. (nih.gov)
  • Within 4 weeks of treatment with baricitinib, the Cutaneous Dermatomyositis Area and Severity Index version 2 and the Dermatology Life Quality Index indicated skin lesions showed recovery. (dermatologyadvisor.com)
  • Validation of the Cutaneous Dermatomyositis Disease Area and Severity Index: characterizing disease severity and assessing responsiveness to clinical change. (jamanetwork.com)
  • In some cases of dermatomyositis, the distal muscles (muscles in the forearms and around the ankles and wrists) may be affected as the disease progresses. (nih.gov)
  • Symptoms of Dermatomyositis The symptoms of dermatomyositis vary from person to person. (1-gsm.com)
  • Rituximab in the treatment of dermatomyositis: an open-label pilot study. (medscape.com)
  • Corbus Pharmaceuticals Receives Orphan Designation for Lenabasum for the Treatment of Dermatomyositis in the European Union : Corbus Pharmaceuticals Holdings, Inc. (corbuspharma.com)
  • Consider participating in a clinical trial so clinicians and scientists can learn more about dermatomyositis and related disorders. (nih.gov)
  • DeWane ME, Waldman R, Lu J. Dermatomyositis: Clinical features and pathogenesis. (medscape.com)
  • Clinical characteristics and outcomes of juvenile and adult dermatomyositis. (medscape.com)
  • The clinical phenotype of dermatomyositis (DM) is uniquely influenced by specific autoantibodies. (nih.gov)
  • Juvenile dermatomyositis (JDM) has a wide spectrum of clinical presentations. (unige.it)
  • 12. The Juvenile Dermatomyositis National Registry and Repository (UK and Ireland)--clinical characteristics of children recruited within the first 5 yr. (nih.gov)
  • Adult onset dermatomyositis has a strong association with cancer and affected individuals should be screened for an underlying cancer. (levydermatology.com)
  • Hyperferritinemia is frequently accompanied by rapidly progressive (RP) interstitial lung disease (ILD) with polymyositis (PM)/dermatomyositis (DM). (hindawi.com)
  • Hypomyopathic dermatomyositis: the patient has no symptoms, but does have positive lab findings that indicate inflammation of muscles. (hss.edu)
  • Juvenile dermatomyositis (JDM) is an autoimmune disorder that affects blood supply to muscles, and causes muscle inflammation. (gillettechildrens.org)
  • Sometimes juvenile dermatomyositis causes joint muscles to shorten (contract). (gillettechildrens.org)
  • Dermatomyositis (DM) is a connective-tissue disease related to polymyositis (PM) that is characterized by inflammation of the muscles and the skin. (pinetreeacu.com)
  • Dermatomyositis is a rare autoimmune disease that affects the muscles and skin. (1-gsm.com)
  • Depending on the involvement of various muscles, dermatomyositis can cause aspiration pneumonia, ventilatory impairment, and heart failure. (anesth-pain-med.org)
  • 16. Quantitative assessment of MRI T2 relaxation time of thigh muscles in juvenile dermatomyositis. (nih.gov)
  • Dermatomyositis is characterized by a skin rash that precedes or accompanies progressive muscle weakness. (nih.gov)
  • Dermatomyositis causes muscle weakness, plus a skin rash. (nih.gov)
  • As an example, Dr. Harp described the treatment of a 56-year-old patient with dermatomyositis, with involvement of the scalp, eyelid, and chest, as well as hand rash, in which each area that was affected was treated with different types and strengths of topical agents. (hss.edu)
  • The earliest sign of juvenile dermatomyositis is the gradual development of a skin rash. (gillettechildrens.org)
  • Some children start experiencing muscle weakness at the same time as they develop the dermatomyositis rash. (gillettechildrens.org)
  • Dermatomyositis (DM) is a debilitating autoimmune muscle disease causing muscle weakness and skin rash. (nih.gov)
  • Dermatomyositis is a rare disease that causes a skin rash and muscle weakness. (levydermatology.com)
  • Dermatomyositis is known by it's distinctive skin rash and muscle weakness. (ipl.org)
  • 7. [Two children with skin rash and muscle weakness: juvenile dermatomyositis]. (nih.gov)
  • If your child has juvenile dermatomyositis, the experts at Gillette Children's Specialty Healthcare in rheumatology and neuromuscular conditions can offer specialized care to help manage symptoms and improve function. (gillettechildrens.org)
  • Vista de Retrospective study of dermatomyositis. (dermatolarg.org.ar)
  • 11. Juvenile dermatomyositis: a retrospective review of a 30-year experience. (nih.gov)
  • 10. [Intravenous cyclophosphamide pulse therapy for refractory juvenile dermatomyositis]. (nih.gov)
  • Our objective in this review is to summarize the relationship between ILCs and diseases such as atopic dermatitis, psoriasis, systemic lupus erythematosus (SLE), multiple sclerosis, rheumatoid arthritis, irritable bowel disease (IBD), systemic sclerosis, and juvenile dermatomyositis (JDM). (jle.com)
  • It is commonly associated with diseases such as SLE, scleroderma, ulcerative colitis , rheumatoid arthritis and dermatomyositis . (digitalnaturopath.com)
  • We observed delayed onset of blockade and prolonged recovery following administration of 0.6 mg/kg rocuronium in a patient with dermatomyositis. (anesth-pain-med.org)
  • Scalp involvement in dermatomyositis. (medscape.com)
  • Dermatomyositis (DM) is commonly associated with scalp pruritus that can be severe. (umn.edu)
  • Juvenile dermatomyositis affects roughly 3,000 to 5,000 children in the U.S. Most cases of the condition occur in children ages 5 to 10. (gillettechildrens.org)
  • Dermatomyositis affects children and adults, but it usually affects children between the ages of five and fifteen, and it occurs in adults in their late forties through sixties. (ipl.org)
  • Dermatomyositis affects as many as 80,000 people in the US, EU and Japan. (corbuspharma.com)
  • Fast Five Quiz: Dermatomyositis - Medscape - Sep 08, 2023. (medscape.com)
  • Cutaneous improvement in refractory adult and juvenile dermatomyositis after treatment with rituximab. (medscape.com)
  • Dermatomyositis is a rare and serious systemic autoimmune condition characterized by skin and muscle involvement. (corbuspharma.com)
  • Associations of tumor necrosis factor alpha and HLA polymorphisms with adult dermatomyositis: implications for a unique pathogenesis. (medscape.com)
  • Pathogenesis of dermatomyositis: role of cytokines and interferon. (jamanetwork.com)
  • The exact cause of dermatomyositis is unknown, but it is believed to be an autoimmune disease, a type of disease in which the body mistakenly attacks its own tissues. (nih.gov)
  • Learn more about the etiology of dermatomyositis. (medscape.com)
  • Children and adults with dermatomyositis may develop calcium deposits, which appear as hard bumps under the skin or in the muscle (calcinosis). (nih.gov)
  • Dermatomyositis is a type of autoimmuneconnective tissue disease. (pinetreeacu.com)
  • She presented amyophatic dermatomyositis one year before, with disease well controlled with 10 mg/day oral prednisolone, 12,5 mg weekly oral methotrexate and 400 mg/day hydroxychloroquine. (biomedcentral.com)
  • Many people with a rare muscle disease called dermatomyositis carry antibodies to a protein called T1F1γ, but the protein's role in normal and diseased muscle has been elusive. (nih.gov)
  • To understand dermatomyositis at a fundamental level, we need to identify and characterize the molecules that the immune system goes after," said Andrew L. Mammen, M.D., Ph.D. , senior author of the study and head of the Muscle Disease Unit of the NIAMS Intramural Research Program. (nih.gov)
  • Finally, fully six years after her symptoms began to take over her life, Kellie Jo went back to her primary care doctor in 2017 with a one-word note, "dermatomyositis", which she handed over. (octapharma.com)
  • There are further complications possible with dermatomyositis. (wikipedia.org)
  • Treatment plans vary depending on the symptoms and complications dermatomyositis causes in each child. (gillettechildrens.org)
  • Rapid improvement of calcinosis in juvenile dermatomyositis with alendronate therapy. (jrheum.org)
  • A 6-year-old boy with improving juvenile dermatomyositis (JDM) developed severe and debilitating calcinosis, unresponsive to diltiazem and probenecid. (jrheum.org)

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