Fulminant Lupus Myopericarditis With Class III Lupus Nephritis and Catastrophic Antiphospholipid Syndrome-Like Thrombosis: A Case Report
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ID: 322463
2026
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Abstract
Abstract Background Lupus myocarditis occurs in 1–3% of SLE cases and rarely coexists with proliferative nephritis at presentation. We describe simultaneous lupus myopericarditis, class III nephritis, MPO-ANCA positivity, and a CAPS-like thrombotic syndrome, highlighting the vulnerability of survivors after fulminant SLE. Case summary A previously healthy 29-year-old man presented with hypertensive pulmonary oedema, acute hypoxaemic respiratory failure requiring intubation, LVEF 36%, and dialysis-requiring acute kidney injury. Workup showed ANA positivity, high-titre anti-dsDNA antibodies, hypocomplementaemia, and triple-positive antiphospholipid antibodies. High-dose corticosteroids and immunosuppression were initiated, and rituximab was selected as adjunctive B-cell-depleting therapy in the setting of severe cardiac and renal SLE, with MPO-ANCA positivity raising concern for a higher-risk renal phenotype. Kidney biopsy confirmed class III proliferative lupus nephritis, and cardiac MRI supported myopericarditis. Cerebral and splenic infarctions prompted anticoagulation, IVIG, and plasma exchange. The course was complicated by CMV peritonitis requiring hemicolectomy. After discharge, he was readmitted with influenza A–associated septic and cardiogenic shock, LVEF 17%, recurrent multifocal cerebral infarctions, MSSA bacteraemia, and invasive aspergillosis. Treatment required plasma exchange, IVIG, tracheostomy, and CRRT. He survived to rehabilitation after 123 cumulative hospital days. Discussion This case illustrates convergent life-threatening autoimmune, thrombotic, and infectious complications in SLE. Cardiac MRI and kidney biopsy were essential for diagnosis. Infection-associated recurrent CAPS-like thrombosis during multiorgan failure underscores the need for infection prevention and close surveillance after fulminant lupus.
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| Authors | ALFREDO PEREZ TAGLE TEJEDA, Issa Jundi, Dahlia Ann Blake, Charles Bonanno, Walif Aji |
| Journal | European Heart Journal - Case Reports |
| Year | 2026 |
| DOI |
10.1093/ehjcr/ytag556
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| URL | |
| Keywords | Keywords not found |
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