• A 31 year old man was diagnosed in 1986 as having histiocytosis X because of pulmonary fibrosis and radiological maxillary lytic lesions. (bmj.com)
  • Following intravenous administration of APOKYN, serious adverse reactions including thrombus formation and pulmonary embolism have occurred. (apokynhcp.com)
  • Before using this medication, tell your doctor or pharmacist your medical history, especially of: liver disease, high blood pressure hypertension, heart valve disease, abnormal scarring/thickening of the lining in the lungs/heart/behind the abdomen pulmonary/pericardial/retroperitoneal fibrosis. (gut-wasserwaid.de)
  • On histopathology, this fibroinflammatory disease is characterized with specific findings such as lymphoplasmacytic infiltration of IgG4-positive plasma cells, obliterative phlebitis, and storiform fibrosis, in most cases accompanied by increased levels of serum IgG4 [ 10 ]. (hindawi.com)
  • Immunoglobulin G4-related disease (IgG4-RD) is a chronic inflammatory disorder associated with fibrosis and abundant tissue lymphoplasmacytic infiltrations. (hindawi.com)
  • However, IgG4-RD can involve the vast majority of organs, including the thyroid and the orbit [ 2 , 8 , 9 ]. (hindawi.com)
  • Immunoglobulin G4-related disease (IgG4-RD) is a chronic immune-mediated fibroinflammatory disorder that often manifests with tumor-like masses and/or painless enlargement of multiple organs. (msdmanuals.com)
  • IgG4-RD has a wide range of manifestations that vary by which organs are involved and that are unified by their histopathologic findings and response to treatment. (msdmanuals.com)
  • The clinical manifestations of IgG4-RD are usually tumor-like masses or organ enlargement, which result from dense tissue infiltration by immune cells and expansion of the extra-cellular matrix. (msdmanuals.com)
  • IgG4-RD likely accounts for most cases of idiopathic retroperitoneal fibrosis. (msdmanuals.com)
  • Treatment of IgG4-RD includes glucocorticoids tapered over 6 months. (asn-online.org)
  • IgG4-related disease ( IgG4-RD ), formerly known as IgG4-related systemic disease , is a chronic inflammatory condition characterized by tissue infiltration with lymphocytes and IgG4 -secreting plasma cells , various degrees of fibrosis (scarring) and a usually prompt response to oral steroids . (mdwiki.org)
  • Pusztaszeri M, Triponez F, Pache JC, Bongiovanni M. Riedel's thyroiditis with increased IgG4 plasma cells: evidence for an underlying IgG4-related sclerosing disease? (medscape.com)
  • Histologic evaluation demonstrates IgG4+ plasmacyte infiltrate, storiform fibrosis, and obliterative phlebitis. (asn-online.org)
  • Common features of IgG4-related disorders include tumor-like swelling of involved organs, lymphoplasmacytic infiltration enriched in IgG4-positive plasma cells, and variable degree of storiform fibrosis. (basicmedicalkey.com)
  • Chronic Pancreatitis Chronic pancreatitis is persistent inflammation of the pancreas that results in permanent structural damage with fibrosis and ductal strictures, followed by a decline in exocrine and endocrine. (msdmanuals.com)
  • Sclerosing Cholangitis Sclerosing cholangitis refers to chronic cholestatic syndromes characterized by patchy inflammation, fibrosis, and strictures of the intrahepatic and extrahepatic bile ducts. (msdmanuals.com)
  • A 66 yo male with a history of chronic kidney disease (CKD) 3b presents with acute renal failure. (asn-online.org)
  • The most recent studies have shown that, after eliminating hepatotoxic drugs and fatty liver disease, the two main causes of liver disease in primary SS are chronic viral infections and autoimmune liver diseases. (xiahepublishing.com)
  • Retroperitoneal fibrosis (RPF) is a form of chronic periaortitis characterized by the development of extensive fibrosis throughout the retroperitoneum, typically centered over the anterior surface of the fourth and fifth lumbar vertebrae and resulting in entrapment and obstruction of retroperitoneal structures, notably the ureters. (medscape.com)
  • Patients with Chronic Liver Disease (CLD) frequently experience neurologic sequel, usually associated with extensive portosystemic shunt of blood, a liver bypass either by portosystemic anastomosis or as a result of the development of an extensive portosystemic collateral circulation. (openventio.org)
  • This infection has been linked to various extra-hepatic manifestations, while chronic infections with a rapid development of liver failure have been described in heavily immunosuppressed patients undergoing solid organ transplantations (SOTs), in patients with hematological diseases or with immunodeficiency virus infection. (biomedcentral.com)
  • In patients with Marfan's syndrome , chronic management with propranolol may reduce the rate of aortic dilation and associated complications including aortic dissection and regurgitation. (pharmacology2000.com)
  • ETHNOPHARMACOLOGICAL RELEVANCE: More than 300 million people worldwide suffer from asthma, a chronic respiratory inflammatory disease. (bvsalud.org)
  • Liver cirrhosis is the endstage of different chronic liver diseases, and is often neglected until complications, such as variceal haemorrhage, spontaneous bacterial peritonitis, ascites, or hepatic encephalopathy occur. (web.app)
  • Typical signs of GO accompanied by increased concentration of IgG4 include younger age at diagnosis, and more severe course of the disease with a higher Clinical Activity Score (CAS). (hindawi.com)
  • We strongly recommend considering the diagnosis of GO with elevated IgG4 in patients with an established diagnosis of GD, elevated serum IgG4 levels, and clinical features of ophthalmic disease overlapping with those of IgG4-related orbital disease. (hindawi.com)
  • Fenaroli P, Maritati F, Vaglio A. Into Clinical Practice: Diagnosis and Therapy of Retroperitoneal Fibrosis. (medscape.com)
  • Patients with abdominal CT scans performed up to a year prior to cancer diagnosis were included. (ima.org.il)
  • In 1994, after three years of progressive neurological dysfunction, diagnosis of Erdheim-Chester disease was made by analysis of biopsies of the femur bones, showing infiltration with foamy histiocytes lacking Birbeck granules and S-100 protein, and with few lymphocytes. (bmj.com)
  • The differential diagnosis of liver disease in primary SS (viral vs autoimmune) is clinically important, since the two processes require different therapeutic approaches and have different prognoses. (xiahepublishing.com)
  • This chapter provides the latest information on the diagnosis of systemic autoimmune diseases. (basicmedicalkey.com)
  • The diagnosis of RPF requires a high degree of suspicion, and the evaluation of patients with presumed idiopathic RPF includes ruling out secondary RPF due to malignancy, infection, retroperitoneal injury, or drugs. (medscape.com)
  • Biopsy and histologic/immunohistochemical examination of the affected tissue can aid in the diagnosis and determination of the extent of the disease, which in turn affect treatment management strategies. (medscape.com)
  • Optimal care in patients with RPF requires an integrated approach of surgical and nonsurgical therapies, and depends on the stage of the disease at diagnosis. (medscape.com)
  • It is asymptomatic with a benign course and occurs most often in young adults, with a median age at diagnosis in the 3rd or 4th decade(3).HHV-8-associated Castleman disease is more aggressive, occurs predominantly in immunosuppressed / human immunodeficiency virus (HIV)-positive patients and manifests commonly with generalized lymphadenopathy, constitutional symptoms, and hematologic and/or immunologic abnormalities. (iriakerala.org)
  • This pictorial review provides an overview of the ultrasonographic anatomy of the scrotum followed by diagnosis of a variety of intra- and extratesticular lesions based on different clinical manifestations. (e-ultrasonography.org)
  • The center focuses on rheumatic diseases such as rheumatoid arthritis, systemic lupus erythematosus and Sjögren's syndrome, and has researched and developed more than 20 new specific markers and immune techniques in diagnosis, such as antibodies to the citrullinated HPVP, antibodies to citrullinated fibrinogen peptide, anti-mDNA, anti-nucleosome, anti- M3 receptor and anti-α-fodrin antibodies. (bdfs.org.cn)
  • Diagnosis of MAS can be difficult and it is hard to distinguish from flares of the underlying disease, and from sepsis-like syndromes, although it may also be associated with sepsis, especially in cases of intraphagocytic infections [ 6 ]. (biomedcentral.com)
  • RPF must be differentiated from other conditions that can cause ureteric obstruction and renal failure, such as retroperitoneal abscess, infections, inflammation, pelvic surgery, and radiation therapy. (medscape.com)
  • Histiocytosis is a group of rare diseases characterized by inflammation and accumulation of cells derived from monocytes and macrophages in different tissues. (bvsalud.org)
  • Sjögren syndrome (SS) is a systemic autoimmune disease in which immune-mediated inflammation causes secretory gland dysfunction, leading to dryness of the main mucosal surfaces. (xiahepublishing.com)
  • Intracranial manifestations are rare and the usual localisations are the retro-orbital space and hypothalamic involvement with neurogenic diabetes insipidus. (bmj.com)
  • Retroperitoneal fibrosis can also be associated with Crohn disease, ulcerative colitis, and sclerosing cholangitis. (medscape.com)
  • Finally, IgG4-related disease must be investigated in patients with SS presenting with sclerosing cholangitis, especially when autoimmune pancreatitis or retroperitoneal fibrosis are also present. (xiahepublishing.com)
  • IgG4-related sclerosing cholangitis (IgG4-SC) is characterized by elevation of serum IgG4 levels, and dense infiltration of IgG4-positive plasma cells with extensive fibrosis in the bile duct wall. (biomedcentral.com)
  • Retroperitoneal fibrosis (RPF) is characterized by the development of extensive fibrosis throughout the retroperitoneum, typically centered over the anterior surface of the fourth and fifth lumbar vertebrae, and results in entrapment and obstruction of retroperitoneal structures (notably the ureters). (medscape.com)
  • Liver involvement was one of the first extraglandular manifestations to be reported in patients with primary Sjögren syndrome (SS). (xiahepublishing.com)
  • In the 1990s, a study of liver involvement in patients with primary SS integrated the evaluation of clinical signs of liver disease, liver function and a complete panel of autoantibodies. (xiahepublishing.com)
  • Recent developments in the field of hepatic and viral diseases have significantly changed the diagnostic approach to liver involvement in SS. (xiahepublishing.com)
  • After eliminating viral hepatitis, primary biliary cirrhosis (PBC) should be considered the main cause of liver disease in primary SS. (xiahepublishing.com)
  • PBC-related SS patients may have a broad spectrum of abnormalities of the liver, including having no clinical or analytical data suggestive of liver disease. (xiahepublishing.com)
  • Autoimmune hepatitis (AIH) is the second most frequently found autoimmune liver disease to be associated with SS (all reported cases are type I), and nearly 10% of these patients have an AIH-PBC overlap. (xiahepublishing.com)
  • Previous studies have reported the therapeutic properties of A. iwayomogi on obesity, hyperlipidemia, and liver fibrosis [ 7 , 8 ]. (hindawi.com)
  • Therefore, we evaluated the effect of ACE on major manifestations of metabolic syndrome including obesity, hyperlipidemia, and fatty liver using a high-fat diet mouse model. (hindawi.com)
  • The scarcity of information concerning such a critical organ presents an interesting conundrum for the scientific and biomedical communities alike, especially given the prevalence of liver degeneracy and disease in the modern clinical setting. (bepress.com)
  • Diseases that can cause severe liver damage can lead to ascites. (web.app)
  • The american association for the study of liver diseases. (web.app)
  • Extracardiac manifestations, such as massive ascites and liver cirrhosis. (web.app)
  • With each injury, the liver forms scar tissue fibrosis, initially without losing its. (web.app)
  • Ascites in hepatic cirrhosis is associated with advanced liver disease and with poor hepatic function and portal hypertension, and consequently it is also associated with a poor prognosis. (web.app)
  • In patients with advanced liver disease, portal hypertension is essential for the formation of ascites. (web.app)
  • The symptoms and signs associated with retroperitoneal fibrosis are nonspecific. (medscape.com)
  • Although neurological manifestations and changes in brain volumes have been described in Erdheim-Chester disease (ECD), it remains unknown whether ECD may be associated with psychiatric symptoms and cognitive dysfunctions. (bvsalud.org)
  • 3 4 We report on two patients with Erdheim-Chester disease with slowly progressive cerebellar dysfunction, associated with pyramidal symptoms. (bmj.com)
  • Parlodel is sometimes used with surgery or radiation to treat acromegaly , and it is also used to treat symptoms of Parkinson's disease , such as stiffness, tremors, muscle spasms, and poor muscle control. (rxlist.com)
  • 1 Although xerophthalmia and xerostomia are the most frequent sicca symptoms, nearly 30% of patients present with extraglandular manifestations, and 5% may develop a hematological neoplasia. (xiahepublishing.com)
  • Negative symptoms include anhedonia (loss of ability to experience pleasurable emotions from typically pleasurable life experiences), reduced emotional expression, decreased social engagement, and concentration dysfunction. (pharmacology2000.com)
  • it can lead to local inflammatory symptoms, and sometimes to systemic symptoms and disease. (biomedcentral.com)
  • Postoperative regression of retroperitoneal fibrosis in patients with inflammatory abdominal aortic aneurysms: evaluation with spiral computed tomography. (medscape.com)
  • For this procedure, outcomes have been improved by intraoperative application of 5-FU (5-fluorouracil) or mitomycin, agents that reduce postoperative fibrosis which may result in channel closure. (pharmacology2000.com)
  • Autoimmune disease is the pathologic result of autoimmunity, whereby the immune system attacks the person's healthy body tissues. (basicmedicalkey.com)
  • Graves disease, myasthenia gravis) or cause tissue damage, either directly or by forming immune complexes that are deposited in tissues or blood vessels. (basicmedicalkey.com)
  • These disorders can be classified as systemic, affecting multiple organs or tissues (e.g., connective tissue autoimmune diseases such as systemic lupus erythematosus [ SLE ], Sjögren syndrome [ SjS ], or scleroderma), or organ specific, targeting one particular organ. (basicmedicalkey.com)
  • This contrasts with previous reports, which describe rare neurological manifestations and the failure of different therapeutic approaches. (bmj.com)
  • Laparoscopic biopsy is safe, minimally invasive, cost-effective, and useful in making therapeutic decisions for retroperitoneal masses. (medscape.com)
  • The purpose of this review article is to describe rheumatic manifestations related to HEV infection and their implications for rheumatologists in the daily clinical practice. (biomedcentral.com)
  • Despite recent accumulating literature in this field, little is known about the course of the infection in patients with rheumatic diseases (RDs) and about the impact of immunosuppressive drugs. (biomedcentral.com)
  • Moreover, HEV infection can mimic RDs' manifestations or drugs toxicity. (biomedcentral.com)
  • Flank pain has many possible causes, including a kidney stone, a urinary tract infection, or back strain. (com.ng)
  • The aim of this paper is to describe the pathophysiological, histopathological, and clinical features of Graves' Disease (GD) and Graves' Orbitopathy (GO) with elevated IgG4 levels. (hindawi.com)
  • It is a relapsing-remitting disease associated with a tendency to mass forming, tissue-destructive lesions in multiple sites, with a characteristic histopathological appearance in whichever site is involved. (mdwiki.org)
  • On histopathological examination, lymphnodal mass consistent with Castleman's disease( hyaline vascular type ), was diagnosed. (iriakerala.org)
  • IgG4-related disease (RD) is a heterogenous condition with renal manifestations. (asn-online.org)
  • Renal involvement can be intrinsic or secondary to retroperitoneal fibrosis. (asn-online.org)
  • The diagnostic and prognostic information available from captopril renography and the increasing availability of magnetic resonance angiography (MRA) have reduced the use of renal arteriography as a diagnostic tool, except in evaluating kidneys with intrarenal branch-artery stenoses and those with complex vascular anatomy, including multiple accessory arteries. (medscape.com)
  • The clinical manifestations reflect the arteries involved and most commonly include hypertension caused by renal-artery stenosis (RAS) or strokes from carotid artery disease. (medscape.com)
  • Recent studies have suggested that IgG4 plays a role in the pathophysiology of autoimmune thyroid diseases. (hindawi.com)
  • The genetic background of our patient with silicone breast implants might have predisposed her to three rare and difficult to diagnose syndromes/diseases: macrophage activation syndrome/hemophagocytic lymphohistiocytosis, autoimmune/inflammatory syndrome induced by adjuvants, and intravascular large B-cell lymphoma. (biomedcentral.com)
  • proposed the term "IgG4-related autoimmune disease" as a systemic disease with multiorgan involvement [ 3 ]. (hindawi.com)
  • The clinical picture ranges from focal bone lesions to systemic disease with life threatening visceral involvement. (bmj.com)
  • PTLD has a broad range of manifestations with extranodal involvement more common in the abdo- men than nodal involvement. (iriakerala.org)
  • This ultimately led to the establishment of new clinical entities called IgG4-related thyroid disease and thyroid disease with an elevation of IgG4. (hindawi.com)
  • however, findings from these examinations can be normal in advanced disease. (medscape.com)
  • Laboratory findings of TIN include pyuria, variable proteinuria, hypocomplementemia and elevated IgG4 subclass. (asn-online.org)
  • Each entry is organized with a brief definition of the disease, information regarding clinical presentation, and laboratory findings. (basicmedicalkey.com)
  • The first description about the constellation of pathological findings of the disease was by Dr. Benjamin Castleman in the 1950s (1). (iriakerala.org)
  • CT signs of missed cancers included small hypodense lesions, peri-pancreatic fat stranding, and dilated pancreatic duct with a cut-off sign. (ima.org.il)
  • Frequent causes include pancreatic cancer and retroperitoneal lymphadenopathy. (nih.gov)
  • Plain abdominal radiograph of a patient with biopsy-proven retroperitoneal fibrosis shows a calcified mass in the retroperitoneum (to the right of the mid lumbar spine). (medscape.com)
  • of either the thoracic or abdominal aorta, which is distinguished from retroperitoneal fibrosis by the presence of circumferential mural aortic wall thickening or enhancement on imaging studies. (msdmanuals.com)
  • However, it might also involve multiple other organs, including the orbit and the thyroid. (hindawi.com)
  • Characteristic features of GD with elevated IgG4 levels include lower echogenicity of the thyroid gland on ultrasound examination, peripheral blood eosinophilia, higher prevalence of orbitopathy, and better response to antithyroid drugs with a tendency to develop hypothyroidism when compared to patients with GD and normal levels of IgG4. (hindawi.com)
  • The authors used various nomenclatures to describe conditions related to the presence of IgG4 until 2010 in Kanazawa, where it was unified as an "IgG4-related disease" with the consensus that it can affect various organs, including the thyroid and the eye with its adnexa and extraocular muscles [ 4 ]. (hindawi.com)
  • Riedel thyroiditis (RT) is characterized by the replacement of normal thyroid parenchyma with dense fibrotic tissue and by the extension of this fibrosis to adjacent structures of the neck. (medscape.com)
  • When sicca features are found in association with another systemic autoimmune disease, most commonly rheumatoid arthritis (RA), systemic sclerosis (SSc) or systemic lupus erythematosus (SLE), it is classified as associated Sjögren syndrome. (xiahepublishing.com)
  • Riedel's thyroiditis and multifocal fibrosclerosis are part of the IgG4-related systemic disease spectrum. (medscape.com)
  • The most frequent systemic manifestations are bone lesions. (bmj.com)
  • Also, families that appear 'schizophrenia-prone' exhibit higher risk for certain other psychiatric abnormalities which include schizoaffective disorder and schizotypal and schizoid personality disorders. (pharmacology2000.com)
  • IgG4-related disease is a multiorgan immune-mediated condition composed of multiple disorders that share particular pathologic, serologic, and clinical features. (basicmedicalkey.com)
  • Posttransplant lymphoproliferative disorders (PTLDs) are a group of diseases that range from benign polyclonal to malignant monoclonal lymphoid proliferations. (iriakerala.org)
  • The classification now includes criteria for subtyping achalasia, EGJ outflow obstruction, motility disorders not observed in normal subjects (Distal esophageal spasm, Hypercontractile esophagus, and Absent peristalsis), and statistically defined peristaltic abnormalities (Weak peristalsis, Frequent failed peristalsis, Rapid contractions with normal latency, and Hypertensive peristalsis). (doczz.es)
  • Approximately one third of patients with RT have an associated extracervical manifestation of multifocal fibrosclerosis. (medscape.com)
  • All included patients were followed for 3 years with no evidence of prostate cancer. (ima.org.il)
  • Overall, 92 patients were included: 38 in the younger age group (40-49 years) and 54 in the older age group (50-69 years). (ima.org.il)
  • Two patients with Erdheim-Chester disease with progressive cerebellar dysfunction and pyramidal signs are reported on. (bmj.com)
  • INDICATION APOKYN is indicated for the acute, intermittent treatment of hypomobility, "of f " episodes ("end-of-dose wearing-of f " and unpredictable "on-of f " episodes) in patients with advanced Parkinson's disease (PD). (apokynhcp.com)
  • The disease may occur at all ages, but typically has its onset in the fourth to sixth decades of life, although some cases are detected in younger female patients, especially in mothers of babies with congenital heart block. (xiahepublishing.com)
  • Patients with paranoid-subtype tend to exhibit preoccupation with a specific delusional framework and do not qualify as having 'disorganized-type' disease which is characterized by disorganized speech/behavior associated with superficial/silly affect. (pharmacology2000.com)
  • Open angle glaucoma is painless and patients may be unaware of damage until relatively late in disease progression. (pharmacology2000.com)
  • To date, an association between IgG4 and various other diseases like allergies, cancer, or rheumatoid arthritis has been revealed [ 11 ]. (hindawi.com)
  • Retroperitoneal Causes of Genitourinary Pain Syndromes: Systematic Approach to Evaluation and Management. (nih.gov)
  • The simultaneous manifestation of all three syndromes suggests causal interrelationships. (biomedcentral.com)
  • To create a world without kidney diseases, the ASN Alliance for Kidney Health elevates care by educating and informing, driving breakthroughs and innovation, and advocating for policies that create transformative changes in kidney medicine throughout the world. (asn-online.org)
  • Kidney (e.g., anti-glomerular basement membrane antibody disease). (basicmedicalkey.com)
  • Anabolic steroids were used for the correction of anemia associated with kidney disease prior to the introduction of erythropoietin. (gut-wasserwaid.de)
  • Thoracic manifestations of IgG4-related disease. (nih.gov)
  • Secondary assessments included global impression scales. (apokynhcp.com)
  • The table below shows human diseases predicted to be associated to Nt5dc2 by phenotypic similarity . (mousephenotype.org)
  • Metabolic syndrome is defined as a cluster of metabolic abnormalities including obesity, hyperlipidemia, hyperglycemia, insulin resistance, and hypertension [ 3 ]. (hindawi.com)
  • However, fatal neurological disease can occur, particularly in immunosuppressed individuals. (frontiersin.org)
  • AIDS-like syndrome: AIDS-like disease (illness) (syndrome) ARC AIDS-related complex Pre-AIDS AIDS-related conditions Prodromal-AIDS 3. (cdc.gov)
  • Lifestyle modifications, including exercise and diet control, are recommended as a first-line treatment for managing metabolic syndrome [ 3 ]. (hindawi.com)
  • The center edited, translated or participated in the editing of over 30 books in rheumatology including Rheumatology, Kelley's Textbook of Rheumatology, Sjögren's syndrome, etc. (bdfs.org.cn)
  • Neurological manifestations are less frequent. (bmj.com)
  • Hepatitis E Virus and rheumatic diseases: what do rheumatologists need to know? (biomedcentral.com)
  • The disease may be more frequent than was previously thought, affecting an estimated 2-4 million people in the United States, 2 and with a prevalence of 0.1-3.3% in European countries. (xiahepublishing.com)
  • Initial characteristics might include social withdrawal and abnormalities in perception but evolve to include frequent delusions and hallucinations. (pharmacology2000.com)
  • IgG4-SC is classified into four types based on the site of strictures due to cholangiography and diseases to be distinguished [ 3 ]. (biomedcentral.com)