Multi-organ Involvement in Catastrophic Antiphospholipid Syndrome: A Challenging Case with a Fatal Outcome

Abstract
Catastrophic antiphospholipid syndrome (CAPS) is a rare, life-threatening variant of antiphospholipid syndrome (APS) characterized by extensive thrombosis, multi-organ involvement, and high mortality. Despite advances in the understanding and management of APS, CAPS remains a challenge because of its rapidly progressive and potentially lethal course. We report the case of a 33-year-old man with a history of chronic venous insufficiency and recent surgery for chronic thromboembolic pulmonary hypertension (CTEPH) who presented with abdominal pain, exertional dyspnoea, and rapidly worsening clinical status leading to multi-organ dysfunction. Despite aggressive treatment, including anticoagulation, corticosteroids, intravenous immunoglobulin, and immunosuppression, the patient succumbed, highlighting the aggressive nature of CAPS. Initial treatment consisted of anticoagulation with low-molecular-weight heparin and warfarin, supplemented by immunosuppressive therapy including hydroxychloroquine, corticosteroids, and cyclophosphamide. The complexity of management escalated with the development of diffuse alveolar hemorrhage, which required broadspectrum antibiotics and hemodiafiltration for acute renal failure. Despite multidisciplinary efforts and adherence to recommended CAPS protocols, the patient’s condition progressively deteriorated, culminating in fatal multiorgan failure. The unpredictable and aggressive nature of CAPS and the limitations of current management strategies are highlighted in this case. This case highlights the need for increased awareness and early aggressive treatment of CAPS patients. It also highlights the importance of ongoing research into more effective treatment modalities and the potential benefits of a multidisciplinary approach in the management of such complex cases. Early recognition and intervention remain critical to the improvement of the prognosis and outcome of patients with CAPS.

Cite this article as: Özlem Çelikdelen S, Bilici R. Multi-organ involvement in catastrophic antiphospholipid syndrome: A challenging case with a fatal outcome. Eur J Rheumatol. 2025; 12, 0025, doi: 10.5152/eurjrheum.2025.24025.