Systemic lupus erythematosus complicated by thrombotic microangiopathy with atypical HUS features: A case report

Systemic lupus erythematosus (SLE)-associated thrombotic microangiopathy with features of atypical hemolytic uremic syndrome (aHUS) is a rare but life-threatening complication. Early recognition is challenging because of its overlapping clinical manifestations with other thrombotic microangiopathies.

A 49-year-old man with SLE presented with anemia, thrombocytopenia, and multi-system involvement. Despite initial immunosuppressive therapy, his hematologic parameters progressively deteriorated, with hemoglobin decreasing to 89 g/L and the platelet count to 54 × 109/L.

Laboratory investigations demonstrated elevated lactate dehydrogenase (501 U/L) and markedly increased soluble complement membrane attack complex (sC5b-9, >1790 ng/mL). ADAMTS13 activity was normal (75.41%), excluding thrombotic thrombocytopenic purpura. Based on the clinical and laboratory findings, the patient was diagnosed with SLE-associated aHUS.

Following the diagnosis, the patient received eculizumab, a monoclonal antibody targeting complement component C5.

Within 6 days of eculizumab treatment, hemoglobin increased to 102 g/L, and the platelet count recovered to 108 × 109/L, indicating a rapid hematologic response.

This case highlights that aHUS can occur as a severe complication of SLE, even in male patients. Complement-mediated thrombotic microangiopathy may coexist with and be triggered by immune hemolysis in SLE. Early recognition and prompt complement inhibition with eculizumab can produce an ultra-rapid hematologic response and may be critical for improving patient outcomes.