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This chapter focuses on the primary, secondary, seronegative, cartastrophic and other subsets of Antiphospholipid Syndrome (APS). Patients with primary APS present features of any patient with an APS such as thrombosis recurrent fetal losses, and/or thrombocytopenia, but without systemic lupus erythematosus (SLE) or any other well defined disease. They do not have any of the features closely associated with SLE such as frank arthritis, mouth ulcers, butterfly rashes, polyserositis, or immune complex type nephritis. They may, however, demonstrate vasculitis-like skin rashes and valve lesions, but not as frequently as in APS secondary to SLE. The patients with APS plus SLE had similar clinical and laboratory profiles to those with primary APS with the exception of autoimmune hemolytic anemia, endocardial valve disease, neutropenia and low C4 levels, all of which occurs more frequently in …