Primary antiphospholipid syndrome: functional outcome after 10 years

To analyze the 10 year functional outcome of primary antiphospholipid syndrome (APS).

We identified 39 patients with primary APS (35 female, 4 male) who developed a first thrombotic or pregnancy event before 1990. Patients meeting American College of Rheumatology criteria for systemic lupus erythematosus or other connective tissue disorders (secondary APS) were excluded. Medical records were reviewed for detailed histories and functional outcomes.

At 10 years' followup, 15 patients (38.4%) had organ damage in the form of hemiparesis (n = 8), dementia (n = 3), quadriplegia (n = 1), dilated cardiomyopathy-myocardial infarction (n = 1), vascular insufficiency-massive pulmonary infarction (n = 1), and endstage renal disease (n = 1). Eight patients (20.5%) with organ damage were unable to perform everyday activities important to their quality of life (functionally impaired). Causes of functional impairment were cognitive dysfunction (n = 3), cardiovascular disease (New York Heart Association Functional Classification Class IV) (n = 2), aphasia (n = 1), expressive aphasia (n = 1), and locked-in syndrome (n = 1).

Functional prognosis is poor in an important minority of primary APS patients with > 10 years of disease. One-third of primary APS patients had organ damage and one-fifth were functionally impaired.