Abstract: TH-PO944
A Rare Case Report of Systemic Lupus Erythematosus (SLE) with Acute Nephritis, Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis, and Thrombotic Thrombocytopenic Purpura (TTP)
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
- Savedchuk, Solomiia, Beaumont Health, Royal Oak, Michigan, United States
- Farshad, Sohail, Beaumont Health System, Royal Oak, Michigan, United States
- Karmo, Dillon, Beaumont Health, Royal Oak, Michigan, United States
- Halalau, Alexandra, Beaumont Hospital, Troy, Michigan, United States
- Swami, Abhishek, Southeast Michigan Kidney Center, Troy, Michigan, United States
Introduction
Thrombotic thrombocytopenic purpura (TTP) is a potentially fatal disorder that requires urgent identification and treatment. The association of TTP with systemic lupus erythematosus (SLE) and vasculitis has been reported, however, never simultaneously.
Case Description
A 33-year-old woman with history of SLE presented with acute abdominal pain, fever, arthralgias, and skin rash. She had acute severe hypertension, diffuse abdominal tenderness, and petechial rash. Diagnostic work-up revealed active urine sediment with proteinuria and hematuria, elevated creatinine, anemia, and thrombocytopenia. She was diagnosed with acute lupus nephritis and early microangiopathic hemolytic anemia in the setting of hypertensive urgency and started on intravenous methylprednisolone 500 mg once a day. Within 48 hours she developed shock with multiorgan dysfunction and succumbed to her illness. Laboratory tests later showed ADAMTS13 activity less than 10% consistent with TTP and p-antineutrophil cytoplasmic antibody (ANCA) positivity. Autopsy revealed small vessel vasculitis of the visceral organs. Kidney biopsy demonstrated diffuse proliferative glomerulonephritis.
Discussion
This case illustrates the occurrence of SLE nephritis, p-ANCA vasculitis, and severe TTP with rapidly fatal course, and the importance of having a low threshold for initiating plasma exchange therapy. Based on our literature search, this is the first case to report on these three afflictions occurring at the same time. The nonspecific signs and symptoms of TTP may hamper a physician’s ability to suspect it on clinical grounds alone, especially in patients with underlying autoimmune conditions. Therefore, when patients with SLE present with thrombocytopenia and features of MAHA, the possibility of TTP should be considered with prompt initiation of empiric plasmapheresis while awaiting test results for confirmation.