IgG 4 related systemic disease

Here is a case description and brief discussion in the green journal.

Originally described as a cause of autoimmune pancreatitis, IgG 4 disease has emerged as a multi organ condition.

Elevated IgG 4 can be helpful in making the diagnosis but is present in only 60% of cases. Histopathologic criteria are the most important features in diagnosis.

The condition can affect virtually every organ system and has features of both an autoimmune and allergic (atopic) disorder.

Renal involvement, particularly interstitial nephritis, has been recognized.