IgG4-related disease: a systematic review of this unrecognized disease in pediatrics

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IgG4-related disease: a systematic review of this unrecognized disease in pediatrics

BACKGROUND Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibro-inflammatory condition with an unclear pathophysiological mechanism affecting different parts of the body. If untreated, the disease can lead to fibrosis and irreversible organ damage. IgG4-RD mostly has been described in adults, hence it is generally unknown among pediatricians. This systematic search of the literature ...

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Ormond's Disease--IgG4-related Disease.

Ormond's disease is a relatively rare disease with unclear etiology, characterized by chronic periaortitis and retroperitoneal fibrosis. The inflammatory process affects the infrarenal part of the abdominal aorta and the iliac arteries, and the presence of infiltrates encasing the ureters and inferior vena cava. This disease is currently classed as an IgG4-related disease. In our review we anal...

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IgG4-Related Perineural Disease

Aims. To elucidate characteristics of IgG4-related disease involving the peripheral nervous system. Methods. Retrospective review of 106 patients with IgG4-related disease identified 21 peripheral nerve lesions in 7 patients. Clinicopathological and radiological features were examined. Results. Peripheral nerve lesions were commonly identified in orbital or paravertebral area, involving orbital...

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IgG4-related sclerosing disease.

Based on histological and immunohistochemical examination of various organs of patients with autoimmune pancreatitis (AIP), a novel clinicopathological entity of IgG4-related sclerosing disease has been proposed. This is a systemic disease that is characterized by extensive IgG4-positive plasma cells and T-lymphocyte infiltration of various organs. Clinical manifestations are apparent in the pa...

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[IgG4-related disease].

IgG4-related disease is a recently recognized systemic syndrome characterized by mass-forming lesions, mainly in exocrine tissue, that consist of lymphoplasmacytic infiltrates and sclerosis. There are numerous IgG4+ plasma cells in the affected tissues, and the serum IgG4 level is elevated in these patients. Ocular adnexal IgG4-related disease frequently involves bilateral lacrimal glands, and ...

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ژورنال

عنوان ژورنال: Pediatric Rheumatology

سال: 2016

ISSN: 1546-0096

DOI: 10.1186/s12969-016-0079-3