Abstract: PUB065
Demystifying the Atypical Presentation of Complement-Mediated Thrombotic Microangiopathy
Session Information
Category: Acute Kidney Injury
- 102 AKI: Clinical, Outcomes, and Trials
Authors
- Yasuda, Allysha, Banner - University Medical Center Tucson, Tucson, Arizona, United States
- Takamatsu, Chelsea, The University of Arizona, Tucson, Arizona, United States
- Nocera, Meleesa, The University of Arizona College of Medicine Tucson, Tucson, Arizona, United States
Introduction
Complement-mediated thrombotic microangiopathy (CM-TMA), also known as atypical hemolytic uremic syndrome (aHUS), is a rare disease characterized by diffuse endothelial blood vessel damage secondary to overactivation of the complement immune system. The classic presentation includes the triad of hemolytic anemia, thrombocytopenia, and acute kidney injury. CM-TMA occurs more commonly in children, but when it occurs in adults, identifying both the diagnosis and underlying etiology requires extensive workup via multi-disciplinary collaboration.
Case Description
We present a male veteran with a complex medical history notable for Crohn’s disease treated with increasing doses of his biologic agent, presenting with new-onset liver cirrhosis, splenomegaly, pulmonary hypertension, and the triad of CM-TMA. Our patient’s presentation of CM-TMA posed a diagnostic dilemma with overlapping multiorgan dysfunction. However, while awaiting definitive diagnostic confirmation for CM-TMA with genetic complement screening results pending for weeks, ruling out other infectious, hematologic, rheumatologic, autoimmune, and iatrogenic explanations for our patient’s condition led us to diagnose CM-TMA and begin urgent treatment. Our patient received infusions of a novel monoclonal complement C5 inhibitor, Ravulizumab, prior to esoteric tests resulting to prevent further, irreversible organ damage.
Discussion
In highlighting the diagnostic workup for and the evolving management of this rare disease, we intend to guide clinicians managing similar patients with confounding pathologies in a clear algorithmic approach to minimize the devastation of CM-TMA.