Primary antiphospholipid syndrome: Functional outcome after 10 years

Objective. To analyze the 10 year functional outcome of primary antiphospho lipid syndrome (APS). Methods. We identified 39 patients with primary APS (35 female, 4 male) who developed a first thrombotic or pregnancy event before 1990. Patients meet ing American College of Rheumatology criteria for systemic lupus erythemato sus or other connective tissue disorders (secondary APS) were excluded. Med ical records were reviewed for detailed histories and functional outcomes. Results. At 10 years' followup, 15 patients (38.4%) had organ damage in the form of hemiparesis (n = 8), dementia (n = 3), quadriplegia (n = 1), dilat ed cardiomyopathy-myocardial infarction (n = 1), vascular insufficiency-mas sive pulmonary infarction (n = 1), and endstage renal disease (n = 1). Eigh t patients (20.5%) with organ damage were unable to perform everyday activi ties important to their quality of life (functionally impaired). Causes of functional impairment were cognitive dysfunction (n = 3), cardiovascular di sease (New York Heart Association Functional Classification Class IV) (n = 2), aphasia (n = 1), expressive aphasia (n = 1), and locked-in syndrome (n = 1). Conclusion. Functional prognosis is poor in an important minority of primar y APS patients with > 10 years of disease. One-third of primary APS patient s had organ damage and one-fifth were functionally impaired.