Why is one arm stronger than two arms? IgG4 antibodies in IgG4-related autoimmune pancreatitis

In this issue, Shiokawa et al1 recapitulated the approach, previously established in pemphigus vulgaris,2 of passive transfer of IgG1 and IgG4 purified from patients with autoimmune pancreatitis (AIP) type 1 into neonatal Balb/c mice. These patients suffered from IgG4-related disease (IgG4-RD). IgG4-RD is a novel clinical entity that includes a growing number of medical conditions that have the following features in common: diffuse organ swelling or focal mass formation, sclerosing storiforme (whirl-like) fibrosis with a lymphoplasmacytic infiltrate rich in IgG4-bearing plasma cells and elevated levels of serum IgG4. The disease usually responds well to steroid treatment and is predominantly diagnosed in elderly men.3 4 IgG4-RD is poorly understood but is currently classified as an autoimmune disease because of its association with autoantibody formation. However, so far there has been no evidence that the autoantibodies reported in IgG4-RD contribute directly to the...

Source: Gut - July 7, 2016 Category: Gastroenterology Authors: Mayerle, J., Lerch, M. M., Greinacher, A. Tags: Commentary Source Type: research