• Granulomatosis with polyangiitis (Wegener's granulomatosis) is a type of vasculitis which can be categorized as a sub-branch of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides. (rheumres.org)
  • This study examined the demographic, clinical, radiographic and laboratory characteristics of patients with granulomatosis with polyangiitis (GPA) in Imam Reza Hospital, Mahshad, Iran. (rheumres.org)
  • The medical records of patients admitted to the Department and Clinic of Rheumatology who were diagnosed with granulomatosis with polyangiitis between January 1, 2006 and December 31, 2016 were retrospectively studied. (rheumres.org)
  • Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis, is a type of vasculitis which can be categorized as a sub-branch of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides [1]. (rheumres.org)
  • In January 2011, Wegener granulomatosis was renamed granulomatosis with polyangiitis (GPA) because of Dr. Wegener's connection with the Nazi regime [3, 4]. (rheumres.org)
  • Granulomatosis with polyangiitis is the third most common type of vasculitis in Iran after Behcet's disease and cutaneous leukocytoclastic angiitis [6]. (rheumres.org)
  • Granulomatosis with polyangiitis (formerly known as Wegner's granulomatosis). (clevelandclinic.org)
  • Eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss vasculitis). (clevelandclinic.org)
  • The diagnosis of idiopathic hypereosinophilic syndrome poses a dilemma because clinical manifestation and serum biomarkers are similar to those of eosinophilic granulomatosis with polyangiitis. (biomedcentral.com)
  • Only a few cases have been reported where coronavirus disease 2019 may have caused the new onset or exacerbation of eosinophilic granulomatosis with polyangiitis or idiopathic hypereosinophilic syndrome. (biomedcentral.com)
  • Although the patient tested negative for anti-neutrophilic cytoplasmic antibodies and skin biopsy was negative for vasculitis, eosinophilic granulomatosis with polyangiitis could not be excluded. (biomedcentral.com)
  • Since glucocorticoids are a standard therapy for both idiopathic hypereosinophilic syndrome and eosinophilic granulomatosis with polyangiitis, we initiated glucocorticoids following a multidisciplinary discussion. (biomedcentral.com)
  • Polyarteritis nodosa, granulomatosis with polyangiitis, Henoch-Schönlein purpura, scleroderma, and otherwise nonspecified vasculitides also were reported to have caused ESRD during this period. (medscape.com)
  • Granulomatosis with polyangiitis (GPA), formerly known as Wegener granulomatosis, is a rare multisystem autoimmune disease of unknown etiology. (medscape.com)
  • Necrotic, purpuric, and blistering plaque on the wrist in a patient with granulomatosis with polyangiitis. (medscape.com)
  • Granulomatosis with polyangiitis (GPA) is a chronic and systematic autoimmune condition characterized by granuloma formation and necrotizing vasculitis of small to medium-sized vessels. (ejao.org)
  • Granulomatosis with polyangiitis (GPA, previously called Wegener's granulomatosis) is a rare, systemic, antineutrophil cytoplasmic antibody (ANCA)-associated form of vasculitis whose lesions usually affect the respiratory tract and kidneys [ 1 ]. (ejao.org)
  • Granulomatosis with Polyangiitis (GPA) Granulomatosis with polyangiitis is characterized by necrotizing granulomatous inflammation, small- and medium-sized vessel vasculitis, and focal necrotizing glomerulonephritis, often with crescent. (merckmanuals.com)
  • Its pathogenic mechanisms and clinical manifestations are varied. (bvsalud.org)
  • Approximately 50% of the patients had a significant drug history, 10% were attributed to infection and 10% had positive collagen workup without any overt manifestations, while 2% each had Wegener granulomatosis and cryoglobulinemia. (bvsalud.org)
  • Follow up of these patients is very essential as cutaneous manifestations may be the forme fruste of serious systemic involvement. (bvsalud.org)
  • With systemic and congenital disorders, extraocular manifestations are observed. (lecturio.com)
  • Sarcoidosis is a non-caseating granulomatous multisystem disease with a wide range of clinical and radiographic manifestations. (radiopaedia.org)
  • BD is a rare multisystem vasculitis characterized by recurrent oral and genital aphtosis, uveitis, skin lesions, neurological and vascular manifestations. (biomedcentral.com)
  • These systemic disorders and the associated renal diseases and cutaneous manifestations are tabulated in Table 1, below. (medscape.com)
  • 2. Autoimmune disorders (14.00D) . Autoimmune disorders are caused by dysfunctional immune responses directed against the body's own tissues, resulting in chronic, multisystem impairments that differ in clinical manifestations, course, and outcome. (ssa.gov)
  • Primary immunodeficiencies (PID) are a heterogeneous group of disorders with a variable clinical spectrum of manifestations. (ac.ir)
  • Clinical manifestations of these conditions, as well as allergy, show significant overlap. (medscape.com)
  • Clinical manifestations of specific vasculitic disorders are diverse and depend on the size and location of the involved vessels, the extent of the organ involvement, and the degree and pattern of extravascular inflammation. (merckmanuals.com)
  • Patients were classified according to the American College of Rheumatology (ACR) 1990 classification criteria for Wegener's granulomatosis. (rheumres.org)
  • Patients were classified according to the American College of Rheumatology (ACR) 1990 classification criteria for Wegener's granulomatosis [8] and 2012 Revised International Chapel Hill Consensus Conference Nomenclature [1]. (rheumres.org)
  • All inpatients and outpatients who met the ACR 1990 criteria for the classification of Wegener's granulomatosis were included in the study. (rheumres.org)
  • Our facility may offer patients the opportunity to participate in state-of-the-art clinical trials for juvenile arthritis and vasculitis . (clevelandclinic.org)
  • BACKGROUND: Cutaneous vasculitis presents as a mosaic of clinical and histological findings. (bvsalud.org)
  • Based on the clinical presentation, vasculitis was classified according to modified Gilliam's classification. (bvsalud.org)
  • The workup of patients with cutaneous vasculitis includes detailed history, clinical examination and investigations to rule out multisystem involvement followed by skin biopsy and DIF at appropriate stage of evolution of lesions. (bvsalud.org)
  • In addition the patients with poor functional outcomes had significantly more systemic organ damage caused by vasculitis (P (lookformedical.com)
  • This increased clinical trial activity in vasculitis has been accompanied by the development and validation of new outcome measures - a challenging process for these complex, multiorgan system diseases. (jrheum.org)
  • The international OMERACT Vasculitis Working Group has developed and implemented an iterative research agenda that has utilized accumulated experience and datasets from several multicenter clinical trials and large cohort studies. (jrheum.org)
  • Determining disease activity in vasculitis is complex because there is no single biomarker to evaluate the heterogeneous, multisystem nature of vasculitis. (jrheum.org)
  • See Vasculitis: Case Presentations , a Critical Images slideshow, for more information on clinical, histologic, and radiographic imaging findings in various forms of vasculitis. (medscape.com)
  • There are multiple distinct forms of systemic vasculitis. (cohencenters.com)
  • These disorders include a chronic inflammatory disease called Aicardi-Goutieres syndrome, as well as systemic lupus erythematosus (SLE), including a rare form of SLE called chilblain lupus that mainly affects the skin. (wikipedia.org)
  • Systemic lupus erythematosus (SLE). (clevelandclinic.org)
  • Systemic lupus erythematosus (SLE) has been the most commonly reported rheumatologic cause of ESRD. (medscape.com)
  • Systemic Lupus Erythematosus (SLE) Systemic lupus erythematosus is a chronic, multisystem, inflammatory disorder of autoimmune etiology, occurring predominantly in young women. (msdmanuals.com)
  • lupus anticoagulants, recurrent venous thrombosis, stroke, spontaneous abortions, livedo reticularis) also can lead to sterile endocardial vegetations and systemic emboli. (msdmanuals.com)
  • This chapter emphasizes congenital and genetic disorders, but we also discuss the pathophysiology and manifestation of various acquired neurocutaneous disorders with an emphasis Behcet's disease, dermatomyositis, Sjögren's syndrome, systemic lupus erythematosus, scleroderma, Parry-Romberg syndrome and Degos disease. (degosdisease.org)
  • This article does not discuss systemic disorders, because most of them are discussed in other articles in Medscape Reference . (medscape.com)
  • The same favourable effect in clinical practice comes from the accumulated knowledge of the complex clinical picture of various causes of RPD, associated specific neurological features (pyramidal signs, ataxia, myoclonus) and systematic features (weight loss, hyponatraemia, hepatic disorders) and their mode of progression. (touchneurology.com)
  • Various conditions involving the central nervous system (CNS) can emerge as RPD, including Creutzfeld-Jakob disease (CJD) and other spongiform encephalopathies, vascular disorders, autoimmune and paraneoplastic encephalopathies, subacute infections, metabolic and toxic disorders and systemic diseases (see Table 1 ). (touchneurology.com)
  • Systemic diseases such as sarcoidosis Sarcoidosis Sarcoidosis is a multisystem inflammatory disease that causes noncaseating granulomas. (lecturio.com)
  • Sarcoidosis is a multisystem disorder of unknown etiology characterized by the formation of inflammatory non-caseating granulomas within affected tissues. (radiopaedia.org)
  • Sarcoidosis is an idiopathic multisystem granulomatous disorder of unknown cause. (bmj.com)
  • Asthma is a clinical diagnosis based on symptoms, detailed history and pulmonary function testing [ 1 ]. (ersjournals.com)
  • Autoimmune sensorineural hearing loss, also known as autoimmune inner ear disease (AIED) is a rare clinical entity characterized by progressive and bilateral sensorineural hearing loss often accompanied by vestibular symptoms. (biomedcentral.com)
  • Symptoms are those of systemic arterial embolism. (msdmanuals.com)
  • After discontinuation of montelukast therapy and administration of systemic corticosteroids the patient's symptoms reversed rapidly and there was prompt resolution of the pulmonary infiltrates. (lookformedical.com)
  • Drug reaction with eosinophilia and systemic symptoms (DRESS) is a severe hypersensitivity drug reaction involving the skin and multiple internal organ systems. (biomedcentral.com)
  • Although uncommon, systemic GPA may cause initial otologic symptoms and should not be dismissed as a possible cause of an otologic disease resistant to standard therapy. (ejao.org)
  • Patchy infiltrates in a patient with clinical pneumonia symptoms who otherwise young, healthy, and walking around…think mycoplasma. (benwhite.com)
  • Oral corticosteroids reduce inflammation and are particularly useful for shrinking nasal polyps, though they also may result in the multisystem improvement of symptoms. (bkallergy.com)
  • The precise meaning will depend on the specific immune system disorder, the usual course of the disorder, and the other circumstances of your clinical course. (ssa.gov)
  • Systemic juvenile idiopathic arthritis (sJIA), adult-onset Still's disease (AOSD), gout, and pseudogout: rheumatologist. (aetna.com)
  • Neonatal-onset multisystem inflammatory disease (NOMID) or Chronic Inflammatory Neonatal Arthritis (CINCA). (clevelandclinic.org)
  • Clinical presentation is variable and diagnosis is usually made on the combination of clinical and radiological features. (radiopaedia.org)
  • Clinical presentation is variable thus making the diagnosis difficult in many instances. (biomedcentral.com)
  • Clinical diagnosis is based on a constellation of clinical findings rather than a single definitive test result. (msdmanuals.com)
  • The results of a multiorgan workup performed at the time of diagnosis were predictive of which patients in this cohort manifested multisystem disease. (thedoctorsdoctor.com)
  • A multiorgan evaluation at the time of diagnosis may be predictive of the probability of multisystem involvement with LCH. (thedoctorsdoctor.com)
  • We believe that cysteinyl leukotriene type 1 receptor antagonists are safe and effective drugs for most patients with asthma but caution is needed for those with more severe disease who require systemic corticosteroids, especially if they show characteristics of the atypical allergic diathesis seen in the prodromal phase of Churg-Strauss syndrome. (lookformedical.com)
  • Her clinical course was complicated by intracerebral haemorrhage following intravenous corticosteroids for neurological relapse. (bmj.com)
  • It should be pointed out that oral corticosteroids carry a risk for significant systemic side effects such as weight gain, peptic ulcers, cataract formation, thinning of one's bones, depression, and/or endocrine dysfunction. (bkallergy.com)
  • The following data was collected from the patients' files: age at onset of disease, gender, disease duration, clinical and laboratory features, and types of treatment. (rheumres.org)
  • Nephrology: nephritis, nephritic proteinuria associated with systemic autoimmune disease. (clevelandclinic.org)
  • Guillain-Barré syndrome and multisystem inflammatory syndrome in children (MIS-C), which are similar to Kawasaki disease, are well-known complications. (biomedcentral.com)
  • The purpose of this article is to integrate renal and cutaneous aspects of disease as well as highlight some important, although frequently underappreciated, clinical or laboratory findings that ally renal and skin diseases. (medscape.com)
  • Disease activity encompasses remission (complete absence of disease activity), response (quantifiable improvement in disease activity), and relapse (increase in disease activity from a previous low or absent state), which are used as outcomes in clinical trials 1 , 2 , 3 , 4 , 5 . (jrheum.org)
  • OBJECTIVES: To describe the morphologic characteristics of skin lesions, extent of extracutaneous disease, and outcomes in patients with neonatal presentation of Langerhans cell histiocytosis (LCH), and to examine clinical predictors of disease prognosis. (thedoctorsdoctor.com)
  • Twelve of the 19 patients had multisystem disease, and 2 died of disease. (thedoctorsdoctor.com)
  • Most patients undergo spontaneous resolution or present a good response to systemic treatment, but a small percentage of patients develop a chronic, progressive disease, which can be refractory to multiple lines of treatment [ 1 ]. (biomedcentral.com)
  • Provided that steroids represent the gold standard as first line treatment, immunosuppressive drugs and antimalarial therapies are currently used in the clinical management of the disease, when steroid-dependence or resistance develop. (biomedcentral.com)
  • Diagnosing neurosarcoidosis in people with previously confirmed systemic disease may be relatively straightforward, but diagnosing primary neurosarcoidosis is challenging. (bmj.com)
  • However, even in the more common and usually slowly progressive dementias such as Alzheimer's disease, frontotemporal lobar degeneration, dementia with Lewy bodies and other degenerative dementias, as well as vascular dementia, establishment and progression of the disease is occasionally surprisingly accelerated, leading to a clinical presentation of RPD. (touchneurology.com)
  • The initial clinical course and the extent of systemic vasculitic lesions may influence the long-term functional prognosis. (lookformedical.com)
  • Clinical examination findings may correlate with the severity of clubbing. (medscape.com)
  • The clinical features, laboratory data, and radiographic findings of the studied patients are summarized in Table 1. (rheumres.org)
  • Patients with the common cold may have a paucity of clinical findings despite notable subjective discomfort. (medscape.com)
  • The range of clinical findings varies, even within the same family. (nih.gov)
  • In general, two different forms of GPA have been described: the localized form, usually limited to the upper airway, and the systemic form, with predominant renal and pulmonary involvement [ 1 ]. (ejao.org)
  • Clinical trials are underway, as are efforts to develop personalized medicines for patients with RVCL. (wikipedia.org)
  • It has long been appreciated that asthma is an umbrella term for patients meeting certain clinical and physiological criteria, but who differ on the basis of specific clinical, biological and/or therapeutic characteristics [ 2 ]. (ersjournals.com)
  • In other patient populations, erythrocyte transfusion has been associated with increased risk of adverse outcomes including sepsis, multisystem organ dysfunction, and death. (asahq.org)
  • The few published case series of RPD have shown that the relative frequency of underlying diseases depends mainly on the clinical setting. (touchneurology.com)
  • Progressive neurologic deterioration unresponsive to systemic immunosuppression including corticosteroid therapy and chemotherapeutic agents. (wikipedia.org)
  • We present a clinical case of systemic GPA initially presenting as nocturnal otalgia, unilateral peripheral facial palsy (PFP), and severe mixed hearing loss (MHL). (ejao.org)
  • Clinical spectrum of LIG4 deficiency is broadened with severe dysmaturity, primordial dwarfism, and neurological abnormalities. (ac.ir)
  • GPA has a spectrum of clinical presentations that includes recurrent respiratory infection in adults and upper and lower respiratory tract problems in children. (medscape.com)
  • The current study purposed to examine the demographic, clinical, radiographic, and laboratory characteristics of the GPA in Mahshad, Iran and compare them with those of other studies. (rheumres.org)
  • The research has utilized accumulated experience and datasets from several multicenter clinical trials and large cohort studies. (jrheum.org)
  • cardiovascular LeukoplakiaHairy portion is a proper tuberculosis, medical granulomatosis consumed on the neoplasm of the logic. (lakesinclair.org)
  • If the megakaryocytes gain access to the systemic circulation, then lodge in the microvasculature of the digits, platelet-derived growth factor and/or vascular endothelial growth factor are released. (medscape.com)
  • Cyclophosphamide and methylprednisolone relieved the patient's ear pain and partially improved his hearing, facial palsy, and overall clinical condition. (ejao.org)
  • This book is about the phenotypic and functional features of epidermal Langerhans cells in experimental models and in clinical medicine. (netlify.app)
  • Langerhans Cell-Histiocytos Langerhans Cell-Histiocytos (LCH) är en ovanlig och gåtfull sjukdom som i huvudsak drabbar barn och som tidigare varit mest känd under beteckningen Histiocytosis X. Förekomst Enligt vad vi vet idag drabbas 5-10 barn i Sverige av sjukdomen varje år. (netlify.app)
  • Samarghitean C, Ortutay C, Vihinen M. Systematic classification of primary immunodeficiencies based on clinical, pathological, and laboratory parameters. (ac.ir)
  • Cases were categorized as multisystem LCH (MS-LCH) (n = 208) and single-system LCH (SS-LCH) (n = 198). (thedoctorsdoctor.com)
  • In clinical practice, for the cases presenting with RPD, the diagnostic procedure must be exhaustive, starting with a detailed clinical evaluation and proceeding to a complete laboratory work-up and sophisticated neuroimaging studies. (touchneurology.com)
  • The clinical presentation and complications depend on the host's immune status. (lecturio.com)
  • This work has led to the development, evaluation, validation, and endorsement, through the OMERACT consensus and validation processes, of a "core set" of outcome measurements for use in clinical trials of AAV. (jrheum.org)
  • Core sets of outcome measures include the domains of the illness under study considered crucial to assess in clinical trials. (jrheum.org)
  • Transfusing blood has been shown to increase systemic oxygen delivery, but not have any effect on systemic oxygen consumption. (asahq.org)
  • 1- Introduction: from the clinical picture given the patient is suffering from congenital glaucoma with failed trabeculectomy and I will consider another trabeculectomy augmented with antimetabolite. (mrcophth.com)
  • Journal of Allergy and Clinical Immunology. (ac.ir)