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

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. 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. The prompt initiation of TPE, because of the suspicion of CAPS, increases the chances of a favourable evolution.

Bereanu AS ，Pisaltu T ，Bereanu R ，Vintila B ，Codru I ，Chicea L ，Crisan O ，Căinap C ，Cainap S ，Sava M ... -  《-》

[Catastrophic antiphospholipid syndrome].

The catastrophic antiphospholipid syndrome (CAPS) is a life-threatening condition resulting from rapidly progressive widespread thromboses mainly affecting the microvasculature in the presence of antiphospholipid antibodies. Within a few days, the patients develop multiorgan failure with pulmonary distress, renal failure with severe hypertension, cerebral, cardiac, digestive or cutaneous involvement. CAPS develops in less than 1% of patients with antiphospholipid syndrome, either primary or associated with systemic lupus erythematosus. CAPS reveals the antiphospholipid syndrome in about 50% of cases. CAPS may be precipitated by infectious diseases, surgical procedures or discontinuation of anticoagulation. CAPS overall mortality rate has decreased in the past decade and is now around 30%. Within our hospital, it has been reduced to 10%. The main differential diagnoses are other thrombotic microangiopathies, and heparin-induced thrombocytopenia. The treatment of CAPS consists of the empirical association of anticoagulation and corticosteroids, plus plasma exchange or intravenous immunoglobulins. Cyclophosphamide is added in patients with systemic lupus erythematosus. The prevention of CAPS is based upon the adequate management of the perioperative period when surgery cannot be avoided, the prompt treatment of infections and the education of patients with antiphospholipid syndrome.

Costedoat-Chalumeau N ，Arnaud L ，Saadoun D ，Chastre J ，Leroux G ，Cacoub P ，Amoura Z ，Piette JC ... -  《-》

Recovery from catastrophic antiphospholipid syndrome by a plasma exchange procedure: report of four cases and review of the literature.

Bortolati M ，Marson P ，Fabris F ，Pengo V ，Facchinetti M ，De Silvestro G ，Ruffatti A ... -  《-》

Aortitis in the setting of catastrophic antiphospholipid syndrome in a patient with systemic lupus erythematosus.

González-García A ，López-Rodríguez M ，Redondo S ，Patier JL ，Masso P ，Tenorio M ，Fajardo GS ，Sarhane Y ，Lacalzada MM ，Manzano L ... -  《-》

Catastrophic antiphospholipid syndrome; a "CATASTROPHIC" case of systemic lupus erythematosus.

Roberts JR ，Finger DR 《-》