Catastrophic antiphospholipid syndrome: Clues to the pathogenesis from a series of 80 patients

We analyzed the clinical and laboratory characteristics of 80 patients with catastrophic antiphospholipid syndrome (APS) (30 new cases and 50 from a MEDLINE computer-assisted review of the literature from 1996 through 2000). Thirty-seven suffered from defined systemic lupus erythematosus (SLE), 33 from primary APS, 5 from "lupus-like" syndrome, 2 from systemic sclerosis, and 1 each had ulcerative colitis, rheumatoid arthritis and polychondritis. Mean age of patients in this series was 37 years (range, 7-67 yr). The most frequent precipitating factors were infections (35%), trauma and invasive procedures (13%), tumors (8%), and withdrawal of anticoagulants or low international normalized ratio (8%). The presentation of the acute multiorgan failure was usually complex, involving multiple organs simultaneously or in a very short time. The majority of patients manifested microangiopathy - that is, occlusive vascular disease affecting predominantly small vessels or organs, particularly kidney, lungs, brain, heart, and liver - with a minority of patients experiencing large vessel occlusions. Thrombocytopenia was reported in 48 (60%) patients, hemolytic anemia in 31 (39%), disseminated intravascular coagulation in 15 (19%), and schistocytes in 7 (9%). The following antibodies were detected: anticardiolipin antibodies (98%), lupus anticoagulant (68%), anti-dsDNA (53% of patients with SLE), antinuclear antibodies (63%), and anti-ENA (17%). Anticoagulation was used in 84% of the episodes, steroids in 80%, cyclophosphamide in 35%, plasmapheresis in 20%, and intravenous gammaglobulins in 19%. Most patients, however, received a combination of nonsurgical therapies. Death occurred in 38 of the 80 (48%) patients. In most, cardiac problems seemed to be the major cause of death. In several of these, respiratory failure was also present, usually due to acute respiratory distress syndrome and diffuse alveolar hemorrhage. Recovery occurred in 62% of episodes treated with anticoagulation versus 23% in those who were not (p = 0.014), and in 64% of episodes treated with anticoagulation plus steroids versus 38% in those who were not (p = 0.053). No other treatment combination was associated with a significant improvement in outcome.