Concomitant Atypical Hemolytic Uremic Syndrome and Lupus Nephritis in a Pregnant Woman
- 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
- Bangaru, Mohan krishna, Wayne State University School of Medicine, Detroit, Michigan, United States
- Barkho, Sulaiman Jamil, Wayne State University School of Medicine, Detroit, Michigan, United States
- Rossi, Noreen F., Wayne State University School of Medicine, Detroit, Michigan, United States
- Song, Yeohan, Wayne State University/Detroit Medical Center, Detroit, Michigan, United States
Atypical hemolytic uremic syndrome (aHUS) is a rare but devastating condition. It involves uncontrolled activation of alternative complement pathway either due to genetic mutation or autoantibodies leading to dysfunction of regulatory proteins. Various triggering factors have been associated with aHUS including infections, pregnancy, drugs, and systemic lupus erythematosus (SLE). In pregnancy with known SLE, diagnosing aHUS can be delayed as the presentation may mimic a flare of lupus nephritis.
A 20-year female diagnosed with lupus nephritis (Class III) diagnosed at the age of 13 was on maintenance therapy with prednisone and mycophenolate mofetil with preserved renal function for 6 years, creatinine 0.6 mg/ml. She stopped taking immunosuppressive medications on her own initiative. Two months later she became pregnant. At 10 weeks gestation, creatinine had increased to 3.3 mg/dl accompanied by severe anemia, thrombocytopenia, high anti-ds DNA titers, and severe hypocomplementemia. Her pregnancy was terminated, and she was placed on hemodialysis and was discharged after being treated as a lupus nephritis flare with prednisone and cyclophosphamide. She was readmitted 2 weeks later with shortness of breath worsening anemia and thrombocytopenia with numerous schistocytes. ADAMTS13 activity level was 100% prompting initiation of plasmapheresis, but without improvement. aHUS panel showed low complement factor H levels with normal complement factors I and B. Renal biopsy showed class V lupus nephritis and thrombotic microangiopathy with focal interstitial fibrosis consistent with aHUS. She was initiated on eculizumab as well as prednisone and mycophenolate mofetil and continues on hemodialysis and this pharmacologic regimen.
Diagnosis of aHUS can be difficult and is often delayed in patients as the clinical presentation may mimic lupus nephritis particularly in pregnancy or may be present together with lupus nephritis. Assessment of the alternative complement pathway, early diagnosis and treatment with eculizumab is important in these setting.