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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.
IgG4-related autoimmune diseases are characterized by excessive fibrosis. In case of Riedel's thyroiditis, fibrosis extends beyond the capsule and involves contiguous neck structures, clinically simulating thyroid carcinoma. There is a rapid thyroid enlargement. Compression of trachea, dysphagia are probable outcomes.
The association of idiopathic retroperitoneal fibrosis with various immune-related conditions and response to immunosuppression led to a search for an autoimmune cause of idiopathic RPF. [5] [6] Many of these previously idiopathic cases can now be attributed to IgG4-related disease, an autoimmune disorder proposed in 2003.
Type 1 AIP is now regarded as a manifestation of IgG4-related disease, [2] and those affected have tended to be older and to have a high relapse rate. Type 1 pancreatitis, is as such as manifestation of IgG4 disease, which may also affect bile ducts in the liver, salivary glands, kidneys and lymph nodes.
Idiopathic orbital inflammatory disease; Idiopathic sclerosing mesenteritis; IgG4-related ophthalmic disease; IgG4-related prostatitis; IgG4-related skin disease; Inflammatory myofibroblastic tumour; Interstitial nephritis
Although a clear understanding of the various skin lesions in IgG4-related disease is a work in progress, skin lesions have been classified into subtypes based on documented cases: [2] Angiolymphoid hyperplasia with eosinophilia (or lesions that mimic it) [3] and cutaneous pseudolymphoma; Cutaneous plasmacytosis [Note 1]
Benign lymphoepithelial lesion or Mikulicz' disease is a type of benign enlargement of the parotid and/or lacrimal glands. This pathologic state is sometimes, but not always, associated with Sjögren's syndrome .
IgG4-related ophthalmic disease (IgG4-ROD) is the recommended term to describe orbital (eye socket) manifestations of the systemic condition IgG4-related disease, [2] which is characterised by infiltration of lymphocytes and plasma cells and subsequent fibrosis in involved structures. It can involve one or more of the orbital structures.