[Differential diagnosis of IgG4-related disease].

Abstract

IgG4-related disease is a chronic autoimmune fibro-inflammatory disease characterized by the presence of lymphoplasmacytic infiltrate, storiform fibrosis, obliterating phlebitis, increased number of IgG4+ cells in tissue, and, in most cases, an elevated serum IgG4 level. This disease often affects the pancreas, salivary glands and lymph nodes, but can involve almost any tissue. Its etiology is still unclear, the central role in the pathogenesis belongs to B-lymphocytes, T2-helpers, interleukins 1-β, 4, 5, 10, 13 and tumor growth factor 1-β. The ambiguous clinical picture and frequent simultaneous involvement of several organs make it difficult to diagnose, so biopsy plays a leading role in making a diagnosis. The characteristic microscopic picture, the presence of certain populations of lymphocytes are key criteria in establishing the correct diagnosis.