A Rare Case of Anti-GBM Disease Complicated by Thrombotic Microangiopathy

Introduction: Anti-glomerular basement membrane (GBM) disease is a small vessel vasculitis marked by antibodies targeting type IV collagen in the glomerular and alveolar basement membranes. This leads to rapidly progressive glomerulonephritis and/or alveolar hemorrhage. Pulmonary involvement often occurs in patients with factors like smoking, infection, or hydrocarbon exposure. Kidney manifestations typically present as acute injury with proteinuria, nephritic sediment, and hematuria. Thrombotic microangiopathy (TMA) involves thrombocytopenia, microangiopathic hemolytic anemia (MAHA), and organ damage. We present a case of anti-GBM disease complicated by TMA in a patient with hydrocarbon exposure. Case Description: A 59-year-old female with chronic obstructive pulmonary disease, hypertension, and cystic kidney disease presented with a two-week history of hematuria, diarrhea, and fatigue. Labs revealed a creatinine level of 10.36, nephrotic range proteinuria, and elevated anti-GBM IgG antibodies. Kidney biopsy showed crescentic glomerulonephritis. The patient developed TMA, requiring multiple blood and platelet transfusions, managed with steroids and plasma exchange. Respiratory distress and hemoptysis necessitated intubation, and bronchoscopy confirmed alveolar hemorrhage. She began immunotherapy with rituximab. Post-extubation, outpatient treatment included plasma exchange, hemodialysis, and steroid tapering. Discussion: This case highlights the rare occurrence of anti-GBM disease complicated by TMA in a patient with hydrocarbon exposure. Literature indicates TMA often arises during anti-GBM treatment, with poor renal outcomes despite ADAMTS-13 activity above 10% in most cases [1]. Initially presenting with diarrhea, Shiga toxin-induced hemolytic uremic syndrome was unlikely. Clinical improvement with rituximab, steroids, and plasma exchange suggests TMA was a complication of anti-GBM disease, emphasizing the disease's complex immune dysregulation. Works Cited: 1. Nakamura, Yoshihiro, et al. “Clinical Characteristics of Anti-GBM Disease with Thrombotic Microangiopathy: A Case Report and Literature Review.” CEN Case Reports, vol. 13, no. 1, 22 May 2023, pp. 37–44, www.ncbi.nlm.nih.gov/pmc/articles/PMC10201029/, https://doi.org/10.1007/s13730-023-00797-4. Accessed 8 May 2024.