Poster Abstracts Part B

Abstract

Tey S.¹; Ahmad A.¹; Samat S.¹; Mohd Perdaus A.²

¹KPJ Rawang Specialist Hospital; ²KPJ Damansara Specialist Hospital

Background: Retroperitoneal Fibrosis (RPF) is an interesting yet daunting rare clinical encounter, especially one that is associated with SLE. It was first described by Albarran in 1905, subsequently by Ormond in 1948, which involves the formation of fibrous bands around the abdominal aorta and its surrounding retroperitoneal organs. SLE coronary vasculitis is also another underappreciated entity, one that is occasionally encountered among young individuals in clinical practice, but not widely discussed in the literature till date.

Case Presentation: We describe a young 38-year-old Malaysian female who was diagnosed with SLE and RPF after she presented with a 6-month history of a left lower quadrant abdominal pain and a worsening stable angina. She had undergone a recent coronary arterial bypass grafting (CABG) for a triple coronary arterial disease 1 year prior to this presentation. Her Computed Tomography Scan with Angiogram demonstrated periaortitis, RPF and a mural thrombus extending from the renal hilar region down to the common iliac arteries. Her CT coronary angiogram demonstrated microaneurysms and beadings. Her IgG4 level, cANCA and pANCA were negative. There were clinical and radiological improvements with intravenous (IV) Cyclophosphamide and high dose corticosteroids.

Conclusion: Corticosteroids and immunosuppressants remain the preferred treatment modalities for RPF across case studies, with an optimistic prognosis. Relapses have been reported, especially ones that are associated with a positive anti-nuclear antigen (ANA) level. Therefore, patients should be educated and followed up closely.

Key Words: SLE, Retroperitoneal Fibrosis, Coronary Vasculitis