What’s new in critically-ill patients with antiphospholipid syndrome?
Keywords:MANQUANTS, Antiphospholipid syndrome, Catastrophic antiphospholipid syndrome, Antiphospholipid antibodies, Lupus anticoagulant, Triple therapy
Antiphospholipid syndrome (APS) is a rare systemic autoimmune disease responsible for thrombotic events and obstetric morbidity in patients with persistent antiphospholipid autoantibodies (aPL). APS may be complicated by a “thrombotic storm” called the catastrophic antiphospholipid syndrome (CAPS), responsible for multiple organ failure and often requiring intensive care unit admission. CAPS was defined by an international consensus as the occurrence of: ≥ 3 organ/tissue/system involvement, developing in <7 days, with biopsy-proven small vessel occlusion in patients with persistent high aPL title. Thrombosis may develop in any organ, tissue or system. Small vessel occlusion is the hallmark of the classical presentation of the disease but large vessel thrombosis is also frequent. Thrombocytopenia is the most typical laboratory finding, occurring in >90% patients, occasionally below 20G/L, associated with anaemia although a true pattern of thrombotic microangiopathy is infrequent. Anticoagulation is the cornerstone treatment of CAPS, being the only one associated with better outcome in several cohorts. In case of life-threatening involvement, the treatment relies on a triple-therapy associating anticoagulation, high-dose corticosteroids and plasma exchange or intravenous immunoglobulins. One-year mortality of CAPS patients admitted to the intensive care unit is as high as 34%. Owing the rarity and the severity of the disease, the diagnosis and management of CAPS rely on a close cross-talk between intensivists and internal medicine physicians.