Leflunomide plus glucocorticoids for IgG4-RD
نویسندگان
چکیده
منابع مشابه
Development of an IgG4-RD Responder Index
IgG4-related disease (IgG4-RD) is a multiorgan inflammatory disease in which diverse organ manifestations are linked by common histopathological and immunohistochemical features. Prospective studies of IgG4-RD patients are required to clarify the natural history, long-term prognosis, and treatment approaches in this recently recognized condition. Patients with IgG4-RD have different organ manif...
متن کاملImmunoglobulin G4-Related Disease (IgG4-RD) with Associated Sarcoidosis
Immunoglobulin G-4 related disease represents a collection of tumefactive entities associated with an abundance of infiltrating IgG4 positive plasma cells, which was described first in patients with chronic pancreatitis. Since then, IgG4 related disease has been described in multiple organ systems including the orbit. We describe here a patient with a history of sarcoidosis who was subsequently...
متن کاملA new clinical entity: IgG4-related disease (IgG4-RD) discovered in the 21st century.
IgG4-related disease (IgG4-RD) is a novel clinical disease entity characterized by an elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4-positive plasma cells (1), and may be present among a certain proportion of patients formerly diagnosed as autoimmune pancreatitis (2, 3), Mikulicz’s disease (4, 5), hypophysitis, Riedel thyroiditis, interstitial pneumonitis, inte...
متن کاملA Case of Type 1 Autoimmune Pancreatitis (AIP), a Form of IgG4-Related Disease (IgG4-RD)
BACKGROUND Type 1 autoimmune pancreatitis (AIP), also known as lymphoplasmacytic sclerosing pancreatitis (LPSP), is a rare cause of chronic pancreatitis, characterized by a fibro-inflammatory process. However, patients with AIP may have a good response to corticosteroid therapy. We describe a Tunisian patient with AIP that was confirmed to be an IgG4-related disease (IgG4-RD). CASE REPORT We de...
متن کاملIgG4 (IgG4-RD) related diseases, with a horizon not limited to Mikulicz's disease.
The IgG4-RD is characterized by pseudotumoral inflammatory lesions caused by lymphoplasmocytic infiltration of IgG4+ cells and elevated serum IgG4. For decades, Mikulicz’s disease and Sjögren’s syndrome were considered identical conditions.1–5 IgG1 immunoglobulin is most prevalent (>50%) and the IgG4 variety constitutes less than 5%. IgG4 has disulfide bonds linking the heavy chains unsteadily,...
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ژورنال
عنوان ژورنال: Nature Reviews Rheumatology
سال: 2020
ISSN: 1759-4790,1759-4804
DOI: 10.1038/s41584-020-0391-7