The family history of patients with primary or secondary antiphospholipid syndrome (APS)

Objective: To evaluate familial history for evidence of antiphospholipid sy ndrome (APS) and autoimmune disease in rheumatology department patients wit h primary or secondary APS. Methods: We retrospectively studied patients with APS and systemic lupus er ythematosus (SLE) managed at the Rheumatology Department of the Bichat Univ ersity Hospital, Paris, between 1987 and 1996. Data were collected by chart review and by a 1997 standardized telephone interview. Results: We identified 108 patients with APS managed during the ten-year st udy period. According to classical classification criteria, 39 patients had primary antiphospholipid syndrome (PAPS) and 69 secondary antiphospholipid syndrome (SAPS). Family history data were obtained for 29 (74%) and 55 (80 %) PAPS and SAPS patients, respectively (78% of the 108 patients). Twelve P APS (41%) and 19 SAPS (35%) patients had one or more relatives with evidenc e of at least one clinical feature of APS such as thrombosis or recurrent f etal loss; of these patients, seven in the PAPS (24%) and 11 in the SAPS (2 0%) group had two or more relatives with evidence of a clinical feature of APS. Three PAPS (10%) and 14 SAPS (25%) patients had one or more family mem bers with an autoimmune disease. Conclusion: A positive family history for autoimmune disease and/or antipho spholipid syndrome is common in patients with PAPS or SAPS. This finding su pports a genetic contribution to APS. The percentage of a positive family h istory for autoimmune disease tend to be higher in patients with SAPS than in those with PAPS.