American Board of Internal Medicine (ABIM) Certification Practice Exam

Which condition is associated with retroperitoneal fibrosis?

1. Rheumatoid arthritis

2. IgG4-related disease

3. Ankylosing spondylitis

4. Chronic pancreatitis

The correct answer is: IgG4-related disease

Retroperitoneal fibrosis is a condition characterized by the development of fibrous tissue in the retroperitoneal space, which can encase abdominal organs such as the ureters, potentially leading to obstruction and other complications. IgG4-related disease is a systemic condition that often features significant inflammation and fibrosis, and it specifically is known to cause retroperitoneal fibrosis in some patients. The underlying immunological mechanisms of IgG4-related disease involve elevated levels of IgG4 antibodies and a prominent plasma cell infiltrate, which can contribute to the fibrotic process in the retroperitoneum. In contrast, while rheumatoid arthritis and ankylosing spondylitis are autoimmune diseases, they are not typically associated with retroperitoneal fibrosis. Chronic pancreatitis can lead to fibrosis and changes in the retroperitoneal space, but it does not directly cause retroperitoneal fibrosis in the way that IgG4-related disease does. Therefore, the connection between IgG4-related disease and retroperitoneal fibrosis is significant, making it the most applicable answer to this question.