Multisystem Fibro Inflammatory Disease: A Rare Combination
TD06-TD08
Correspondence
Dr. Sivakami Pradheepkumar,
Consultant Radiologist, Department of Radiodiagnosis, Diwan Health Complex,
Post Box No-977, Postal Code-211, Salalah, Oman.
E-mail: drsivakamijj@gmail.com
Idiopathic Retroperitoneal Fibrosis (RPF) is a disease of unknown aetiology, characterised by extensive peri aortic 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.