Catastrophic Antiphospholipid Syndrome: Clinical and Laboratory Features of 50 Patients

Abstract

We analyzed the clinical and laboratory characteristics of 50 patients with catastrophic antiphospholipid syndrome (APS) (5 from our clinics and 45 from a MEDLINE computer-assisted review of the literature from 1992 through 1996). Thirty-three (66%) patients were female and 17 (34%) were male. Twenty-eight (56%) patients had primary APS, 15 (30%) had defined systemic lupus erythematosus (SLE), 6 (12%) had "lupus-like" syndrome, and 1 (2%) had rheumatoid arthritis. Mean age of patients in this series was 38 +/- 14 years (range, 11-74 yr). Three (6%) patients developed the clinical picture of catastrophic APS under the age of 15 years, and 11 (22%) were 50 years old or more. In 11 (22%) patients, precipitating factors contributed to the development of catastrophic APS (infections in 3, drugs in 3, minor surgical procedures in 3, anticoagulation withdrawal in 2, and hysterectomy in 1). The presentation of the acute multi-organ failure was usually complex, involving multiple organs simultaneously or in a very short period of time. The majority of patients manifested microangiopathy--that is, occlusive vascular disease affecting predominantly small vessels of organs, particularly kidney, lungs, brain, heart, and liver--with a minority of patients experiencing only large vessel occlusions. Thrombocytopenia was reported in 34 (68%) patients, hemolytic anemia in 13 (26%), disseminated intravascular coagulation in 14 (28%), and schistocytes in 7 (14%). The following antibodies were detected: lupus anticoagulant (94%), anticardiolipin antibodies (94%), anti-dsDNA (87% of patients with SLE), antinuclear antibodies (58%), anti-Ro/SS-A (8%), anti-RNP (8%), and anti-La/SS-B (2%). Anticoagulation was used in 70% of the patients, steroids in 70%, plasmapheresis in 40%, cyclophosphamide in 34%, intravenous gammaglobulins in 16%, and splenectomy in 4%. Most patients, however, received a combination of nonsurgical therapies. Death occurred in 25 of the 50 (50%) 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. Among the 20 patients who received the combination of anticoagulation, steroids, and plasmapheresis or intravenous gammaglobulins, recovery occurred in 14 (70%) patients. The use of ancrod and defibrotide appeared to be effective in the 2 respective patients in whom they were used.

Obstetrical Considerations and Management of Antiphospholipid Syndrome

“…These thromboses are most often found on histopathology of the kidneys, lungs, and gastrointestinal tract with sparing of the heart, liver, and adrenal glands. A review of 50 affected patients found renal involvement on presentation in 78%, often with concomitant malignant hypertension [12]. Pulmonary involvement was present in 66% of patients, with severe dyspnea as a common symptom.…”

The Infectious Origin of the Antiphospholipid Syndrome

“…14 Since then, we have analysed the pathogenesis of catastrophic APS in more than 300 patients. [15][16][17][18][19] We observed that ''triggering'' factors become increasingly apparent and were present in 51% of cases in the latest analysis. 17 These factors included trauma (including surgical, both major and minor), anticoagulation withdrawal, a variety of carcinomas and, most importantly and commonly, infections, which were identified in 24% of these patients.…”

Clinical Features and Pathogenesis of Catastrophic Antiphospholipid Syndrome

“…Catastrophic antiphospholipid syndrome (CAPS) is defined by the clinical manifestations of sudden onset of multiple organ involvements including renal failure or acute respiratory distress syndrome in patients with primary or secondary APS (6)(7)(8). CAPSis characterized by microangiopathy, that is, occlusive vascular disease affecting predominantly small vessels of different organs, particularly kidney, lung, brain, heart, and liver.…”

“…The activity of von Willebrand factor-cleaving protease in patients with CAPS should be analyzed to assess the possibility of this mechanism of thrombosis in such patients. 3) Plasmapheresis has been reported to be effective in both patients with CAPSand TTP (7,8,15), and plasmapheresis was performed in the patient of Mizuno et al (10). However, a precise prospective study has not been carried out.…”