Mixed connective tissue disease and idiopathic retroperitoneal fibrosis: A rare but important association

Abstract

Idiopathic Retroperitoneal fibrosis (RPF) is a fibro-inflammatory disease. In patients with known mixed connective tissue disease (MCTD) it has rarely been described. Our case illustrates a unique presentation of RPF in a patient with MCTD. We emphasise possible links between the two disease processes and the high level of clinical suspicion required to make a diagnosis.

Keywords

• Retroperitoneal fibrosis
• Mixed connective tissue disease
• Vasculitis