IgG4-related systemic disease of the pancreas with involvement of the lung: a case report and literature review

Abstract

IgG-related systemic disease (ISD) remains exceedingly rare and unfamiliar, particularly extrapancreatic disease. We report a patient with separate presentations of IgG4 pulmonary disease and recurring IgG4 related biliary sclerosis and pancreatitis. Because of the intricate and perplexing pathogenesis, overlapping organ systems and wide variation in disease presentation, ISD in its entirety remains undefined. Accurate identification of ISD is critical to avoid permanent organ damage especially since treatment is nearly always successful with corticosteroids. As recognition and awareness of this disease grows, development of standard diagnostic criteria and treatment plans are needed.

Keywords

• IgG4-related systemic disease
• pancreas
• biliary sclerosis
• pancreatitis
• hypergammaglobulinemia
• corticosteroid
• interstitial lung disease
• IgG4