High impact of antiphospholipid syndrome on irreversible organ damage and survival of patients with systemic lupus erythematosus.
BACKGROUND
Thrombosis is a frequent cause of morbidity and death in patients with systemic lupus erythematosus (SLE). Whether antiphospholipid syndrome (APS) is the cause of increased irreversible organ damage and mortality in lupus patients is not well established.


METHODS
Prospective inception cohort of 202 patients with SLE (American College of Rheumatology criteria). Antiphospholipid syndrome was defined according to the Sapporo criteria. Irreversible damage was measured using the Systemic Lupus International Collaborating Clinics-American College of Rheumatology damage index (SDI) at 6 months and 1, 3, 5, 10, 15, 20, and 25 years after the diagnosis of SLE. All deaths were documented.


RESULTS
A total of 88% of patients were women. Twenty-eight patients met criteria for definite APS. Mean (SD) follow-up was 9.7 (6.0) years. Nine patients could not be contacted for follow-up. All patients with APS experienced thrombosis, most of them in the arterial bed. Damage was more severe in patients with APS than in those without APS (median SDI score, 2 vs 0 at 5 years; P<.001; 4 vs 1 at 15 years; P<.001). Cumulative survival at 15 years was lower in patients with APS than in those without APS (65% vs 90%, P =.03). Older age at diagnosis, lupus nephritis, and APS were independent predictors of mortality.


CONCLUSIONS
Antiphospholipid syndrome with thrombotic manifestations is a major predictor of irreversible organ damage and death in patients with SLE.
Ruiz-Irastorza
Guillermo
G
Servicio de Medicina Interna, Hospital de Cruces, Universidad del Pais Vasco/Euskal Herriko Unibertsitatea, Bizkaia, Spain. r.irastorza@euskalnet.net
Egurbide
Maria-Victoria
MV
Ugalde
Jon
J
Aguirre
Ciriaco
C
D000328:Adult; D000367:Age Factors; D016736:Antiphospholipid Syndrome / Q000150:complications / Q000401:mortality*; D015331:Cohort Studies; D005260:Female; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000401:mortality*; D008181:Lupus Nephritis / Q000150:complications / Q000401:mortality; D008297:Male; D009102:Multiple Organ Failure / Q000209:etiology / Q000401:mortality*; D016016:Proportional Hazards Models; D011446:Prospective Studies; D016019:Survival Analysis; D013927:Thrombosis / Q000209:etiology / Q000401:mortality