Multisystem Fibro Inflammatory Disease: A Rare Combination

Abstract

Idiopathic Retroperitoneal Fibrosis (RPF) is a disease of unknown aetiology, characterised by extensive periaortic sclerosis. A subset of idiopathic RPF is found to be associated with Immunoglobulin G4-Related Disease (IgG4-RD) which is also designated as Multisystem Fibro Inflammatory Disease (MFID). IgG4-RD is an emerging immune mediated disorder of unknown aetiology being increasingly appreciated in recent times. This disorder encompasses a spectrum of disorders involving more than one organ system and shares a common pool of clinical, serological and pathological features. There are few clinical, radiological, pathological and treatment response differences found between the subset of idiopathic RPF that is associated with IgG4-RD and the rest without IgG4-RD association. Here we present a case of MFID with a rare combination of idiopathic RPF causing bilateral ureteral encasement, large bowel obstruction and gross pericardial effusion.

Keywords

• benign
• ceroid
• malignant
• retroperitoneal fibrosis