Multisystem Fibro Inflammatory Disease: A Rare Combination
Published: February 1, 2018 | DOI: https://doi.org/10.7860/JCDR/2018/31871.11193
Sivakami Pradheepkumar, Ramesh Kumar Rudrappa
1. Consultant Radiologist, Department of Radiodiagnosis, Diwan Health Complex, Salalah, Oman.
2. Professor and Head, Department of Radiodiagnosis, Sri Manakula Vinayagar Medical College and Hospital, Madagadipet, Puducherry, India.
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.
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