Abstract: TH-PO954
A Case of Poststreptococcal Glomerulonephritis with Concurrent ANCA-Associated Vasculitis and Aortitis: Challenges in Diagnosis and Treatment
Session Information
- Glomerular Trainee Case Reports
November 07, 2019 | Location: Exhibit Hall, Walter E. Washington Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Report
- 1202 Glomerular Diseases: Immunology and Inflammation
Authors
- Panda, Monisha, American University of the Caribbean School of Medicine, Chadds Ford, Pennsylvania, United States
- Abdul Ghaffar, Madeeha, Northwell Health/Staten Island University Hospital, Staten Island, New York, United States
- Manchandani, Umesh Kumar, Northwell Health/Staten Island University Hospital, Staten Island, New York, United States
Introduction
Aortitis is an uncommon condition with either infectious or rheumatologic etiology. Common rheumatologic causes of aortitis include the large vessel vasculitides, Takayasu’s arteritis and giant cell arteritis; however, other rheumatologic causes exist, such as ANCA-associated vasculitides (AAV). A few previous cases of aortitis associated with perinuclear antineutrophil cytoplasmic antibody (p-ANCA) and anti-myeloperoxidase (MPO) antibody have been reported, however, in medical literature, AAV is infrequently discussed in association with large vessel involvement. It is rare to find cases of post-streptococcal glomerulonephritis (PSGN) in which there is concurrent AAV affecting large vessels.
Case Description
We report a case of a 36-year old Hispanic woman with an initial presentation of right leg cellulitis with positive antistreptolysin-O titers. Her past medical history was significant for previous right leg skin lesion and right groin lymph node abscesses. She developed hematuria during her admission and severe epistaxis. Due to worsening kidney function and persistent hyperkalemia, hemodialysis was initiated. Kidney biopsy results supported PSGN. P-ANCA, MPO, and proteinase-3 (PR3) antibody assays were positive. Magnetic resonance angiography results supported aortitis. Due to treatment for active infection, immunotherapy was postponed. Repeat P-ANCA, PR3, and MPO were persistently positive. Subsequently, she developed a cough and hemoptysis, after bronchoscopy she was determined to have diffuse alveolar hemorrhage. Methylprednisolone and plasmapheresis were initiated, followed by induction of rituximab.
Discussion
This case highlights a diagnostic dilemma in vasculitis classification: determining whether the aortitis was due to ANCA-associated vasculitis, large vessel arteritis, or an infectious process. Renal biopsy and clinical picture supported an infectious etiology of glomerulonephritis, thus the positive p-ANCA and MPO antibody assay were initially thought to be induced by infection, however, our patient had persistently positive ANCA results and developed DAH. This case also highlights the challenge in starting immunosuppressive treatments during infection, and, finally, the response to Rituximab and plasmapheresis therapies.