The Clinical and Pathological Features of IgG(4)-Related Disease.

Published on Oct 1, 2011in Current Rheumatology Reports3.873
· DOI :10.1007/S11926-011-0213-7
Arezou Khosroshahi30
Estimated H-index: 30
(Harvard University),
Vikram Deshpande100
Estimated H-index: 100
(Harvard University),
John H. Stone76
Estimated H-index: 76
(Harvard University)
The rapidly emerging disorder now known as IgG4-related disease (IgG4-RD) includes a variety of clinical entities once regarded as being entirely separate diseases. Manifestations of IgG4-RD have now been reported in essentially all organ systems. Regardless of which organ is involved, tissue biopsies reveal striking histopathological similarities. The hallmark pathology findings are diffuse lymphoplasmacytic infiltrates, abundant IgG4-positive plasma cells, modest tissue eosinophilia, and extensive fibrosis. Tumorous swelling and obliterative phlebitis are other frequently observed features. Polyclonal elevations of serum IgG4 are found in approximately 70% of patients. Many questions pertaining to the etiology, pathophysiology, epidemiology, clinical features, therapy, disease monitoring, and long-term outcomes remain to be addressed. This paper focuses on the clinical and pathological features of IgG4-RD. Keywords IgG4 .IgG4-related disease.Autoimmune pancreatitis.Chronic sclerosing sialadenitis.Riedel's thyroiditis.Sclerosing cholangitis.Lymphoplasmacytic aortitis.Retroperitoneal fibrosis.Tubulointerstitial nephritis.Orbital pseudotumor.Dacryoadenitis. Dacrocystitis.Lacrimal gland.Mikulicz's syndrome. Lymphadenopathy.Pachymeningitis.Storiform fibrosis. Obliterative phlebitis.Eosinophilia.Hypophysitis.
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