Therapeutic Plasma Exchange in Catastrophic Antiphospholipid Syndrome (CAPS): A Rare Case Report and Literature Review

Background/Aim: Catastrophic antiphospholipid syndrome (CAPS) may be the first manifestation (de novo) of antiphospholipid syndrome (APS) or a complication in the clinical course of patients known to have this syndrome. Approximately 40% of patients had an associated autoimmune disease, mainly, systemic lupus erythematosus (SLE). The trigger can be one of the following: infections, surgical interventions, neoplasms, pregnancy, discontinuation of anticoagulant treatment, and others. CAPS is a medical emergency in which early identification and prompt initiation of aggressive therapy is extremely important. According to the Guidelines for the use of Therapeutic Apheresis in Clinical Practice developed by the American Society for Apheresis (ASFA), last updated in April 2023, in CAPS, the indication for therapeutic plasma exchange (TPE) is category I, grade 2C. Case Report: We present a case of probable CAPS secondary to systemic lupus erythematosus (SLE) in an elderly patient in whom clinical and biological improvement was achieved through a multidisciplinary approach and prompt initiation of TPE. Because TPE is considered first-line therapy in CAPS, we initiated the procedure as soon as the attending rheumatologist raised this suspicion. Four plasmapheresis sessions were performed in the Intensive Care Unit. We used TPE by membrane filtration. Following the therapeutic intervention with TPE, corticotherapy (Solumedrol in puls-therapy), cyclophosphamide and anticoagulant treatment, the evolution was favourable, with clinical and biological improvement.Conclusion: The prompt initiation of TPE, because of the suspicion of CAPS, increases the chances of a favourable evolution.

Automated summary

Prompt initiation of therapeutic plasma exchange (TPE) is crucial for a favorable outcome in patients with catastrophic antiphospholipid syndrome (CAPS) secondary to systemic lupus erythematosus (SLE). This case report highlights the successful multidisciplinary approach and the importance of early TPE in achieving clinical and biological improvement in a CAPS patient.