What is IgG4-Related Disease?

IgG4-Related Disease
Gross and histopathological features of IgG4-related disease of rectum. (A) Photograph of biopsy specimen; (B) tissue specimen from patient with IgG4-related disease shows rectum (H&E, ×100); (C) plasma cells in specimens (immunoperoxidase staining, ×100).IgG4-related disease of the rectum. Choi SB, Lim CH, Cha MG, Kang WK - Annals of surgical treatment and research (2016). Not Altered. CC.

IgG4-related disease is a new rare syndrome that is associated with many disease processes.

The pathology of IgG4-related disease is:

-Etiology: The cause of IgG4-related disease is unknown, but may be attributed to an autoimmune and allergic disorder.

-Pathogenesis: The sequence of events that lead to IgG4-related disease starts with autoantigens that trigger the condition, with IgG4 antibodies proven to be not pathogenic themselves and are released due to immunologic response to infection.

-Morphologic changes: The morphologic changes involved with IgG4-related disease are inflammatory pseudotumors, lymphoplasmacytic infiltration, and storiform fibrosis.

IgG4-related disease typically affects male in the head and neck region and present at age range of 50-55 years old. The symptoms, features, and clinical findings associated with IgG4-related disease include swelling of involved organ, pancreatitis, lymphadenopathy, hypothyroidism, bile duct lesion, salivary and lacrimal gland involvement, allergies and plaques, papules and nodules that are pruritic and most commonly located in the face or forearm.

IgG4-related disease is diagnosed through the combination of clincial, radiology, serologic, and endoscopic examination. Definite diagnosis requires biopsy. 

IgG4-related disease is treated with systemic steroids, azathioprine, mycophenolate mofetil, and rituximab.

The prognosis of IgG4-related disease is dependent on its manifestation, that can either result to spontaneous resolution or progress to persistent and relapsing symptoms.

Tags:
Last updated on May 27, 2022