The American journal of the medical sciences · 2025
Etiology-based outcomes of biopsy-proven kidney thrombotic microangiopathy: A retrospective analysis of genetic, secondary, and infectious causes.
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Abid Sidrah, Pal Aman, Audil Aliyah, Humayun Noor, Ali Omar, Beers Kelly, Feustel Paul J, Mehta Swati
Аннотация
BACKGROUND: Thrombotic microangiopathy (TMA) is a pathological diagnosis characterized by microvascular thrombosis, hemolytic anemia, thrombocytopenia, and ischemic organ dysfunction. The evolving understanding of TMA's heterogeneous etiology, particularly in relation to genetic and complement dysregulation, complicates its diagnosis and management.
METHODS: This retrospective study at Albany Medical Center (AMC) aimed to explore clinical outcomes and management strategies among 33 patients diagnosed with biopsy-confirmed TMA between 2018 and 2024.
RESULTS: The cohort consisted of primary hereditary TMA (15.2 %), secondary TMA (69.7 %) and mixed etiologies (15.1 %). Gene panels were performed in 11 patients, revealing complement-related mutations in 9 cases. Of these, 5 had hereditary TMA, while 4 had mixed etiologies involving hereditary in addition to secondary or infectious triggers. Notably, 10 patients received complement inhibition, with 60 % showing improvement in kidney function at 6 months.
DISCUSSION: This highlights the potential benefit of complement-targeted therapies, even in cases where secondary or mixed etiologies are suspected. The results raise the possibility that many secondary or infection-associated TMAs may harbor unrecognized complement-related abnormalities, emphasizing the importance of genetic testing and early complement inhibition in these cases.
CONCLUSIONS: Future studies with larger cohorts are essential to further investigate these findings and refine diagnostic and therapeutic strategies for TMA, particularly in cases with complex or overlapping etiologies.
METHODS: This retrospective study at Albany Medical Center (AMC) aimed to explore clinical outcomes and management strategies among 33 patients diagnosed with biopsy-confirmed TMA between 2018 and 2024.
RESULTS: The cohort consisted of primary hereditary TMA (15.2 %), secondary TMA (69.7 %) and mixed etiologies (15.1 %). Gene panels were performed in 11 patients, revealing complement-related mutations in 9 cases. Of these, 5 had hereditary TMA, while 4 had mixed etiologies involving hereditary in addition to secondary or infectious triggers. Notably, 10 patients received complement inhibition, with 60 % showing improvement in kidney function at 6 months.
DISCUSSION: This highlights the potential benefit of complement-targeted therapies, even in cases where secondary or mixed etiologies are suspected. The results raise the possibility that many secondary or infection-associated TMAs may harbor unrecognized complement-related abnormalities, emphasizing the importance of genetic testing and early complement inhibition in these cases.
CONCLUSIONS: Future studies with larger cohorts are essential to further investigate these findings and refine diagnostic and therapeutic strategies for TMA, particularly in cases with complex or overlapping etiologies.