Abstract: SA-PO245
Gall Bladder Arteritis in a 35-Year-Old Lady with Mixed Cryoglobulinemia Syndrome
Session Information
- Trainee Case Reports - V
October 27, 2018 | Location: Exhibit Hall, San Diego Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Reports
- 1202 Glomerular Diseases: Immunology and Inflammation
Authors
- Kiliaris, Maria Ioanna, Milton Keynes University Hospital NHS Foundation Trust, Milton Keynes, United Kingdom
- Abuleis, Husham Rasheed, Oxford University Hospitals NHS Foundation Trust, Oxford, United Kingdom
- Shenbagaraman, Packiam, Oxford University Hospitals NHS Foundation Trust, Oxford, United Kingdom
Introduction
Mixed Cryoglobulinemia Syndrome is a systemic disease caused by the deposition of circulating immune complexes (cryoglobulins) in small and medium –sized arteries. Type II cryoglobulinemia is most often due to Chronic Hepatitis C infection and it is also seen in autoimmune disorders such as SLE and Sjogren’s syndrome. Whilst skin and renal involvement are common clinical features, gall bladder involvement is very rare and we report a case of cholecystitis due to cryoglobulinemic vasculitis.
Case Description
A 35-year-old lady with Sjogren’s syndrome presented in August 2016 with systemic symptoms, acute kidney injury(AKI), hypertension, significant hemoproteinuria, low C3 and C4 and positive cryoglobulins (type II). ANCA and anti GBM antibodies were negative. PET CT scan and a bone marrow trephine were done to exclude lymphoma. She underwent a kidney biopsy which confirmed cryoglobulinaemic glomerulonephritis and was managed with plasmapheresis, Rituximab and Methylprednisolone followed by oral prednisolone. Her systemic symptoms, kidney function and proteinuria improved. In December 2016 her condition relapsed with systemic symptoms, skin rash, worsening renal function and positive cryoglobulins (cryocrit 8%). She responded to another course of plasmapheresis, Rituximab and prednisolone. In May 2017 she was admitted under Gastroenterology for right upper quadrant pain, raised CRP and deranged liver function tests. She was diagnosed with acute cholecystitis. At this time she developed another cryoglobulinemic relapse with oliguric AKI. She was managed again with plasmapheresis, Rituximab and prednisolone. She also needed hemodialysis briefly. She had an interval cholecystectomy. The gall bladder histology was reported to show florid arteritis in the medium sized arteries related to cryoglobulinemia (images will be provided in the poster). Her kidney biopsy was repeated and it also showed arteritis with severe chronic damage. Cyclophosphamide was added to the treatment regimen to prevent further relapses and because of the extra-renal disease.
Discussion
To our knowledge this is the first case report of cryoglobulinemic vasculitis that manifested in the gall bladder and mimicked acute cholecystitis during a relapse. The diagnosis can be missed or delayed and therefore careful examination and correlation with the clinical picture is essential.