IgG4-Related Disease: A Constellation of Abdominal Manifestations

Abstract

IgG4-related disease is an autoimmune condition that results in inflammation and fibrosis throughout multiple organ systems. This disease is rare; however, it can mimic many more prevalent conditions clinically and thus should be included in their differential diagnoses. Although autoimmune pancreatitis is the most common abdominal manifestation, the disease can afflict the hepatobiliary, vascular and renal systems as well. We present a case of a 78-year-old male with symptoms of chronic fatigue and weight loss. Imaging was performed with the expectation of revealing a malignancy, although the features of IgG4-related disease involving multiple organs were detected instead. Serology confirmed the diagnosis. It is imperative to diagnose IgG4-related disease early as it has a different treatment and favorable prognosis compared to many malignancies.