Risk factor of mortality in systemic sclerosis of Han nationality / 中华风湿病学杂志

ABSTRACT

Objective To determine the prognostic factors in systemic sclerosis.Methods Clinical data of definite systemic sclerosis patients were collected,including disease onset age,sex,disease course,Raynaud's phenomenon,skin involvement,gastroesophageal reflux,interstitial pneumonia,cardiac lesions,kidney lesions and scleroderma renal crisis.serum antibodies to scl-70.RNP and anti-centromere antibody were detected.Pulmonary artery pressure was measured by ultrasound cardiography.Cox hazard ratio model was employed to assess the mortality risk of systemic sclerosis patients.Results No difference in Raynaud's phenomenon,gastroesophageal reflux,anti-nuclear antybody,anti-sol-70 antibody,anti-centromere antibody,interstitial pneumonia,diffusion capacity (DLco),coronary artery disease,and peripheral artery atherosclerotic disease could be found between the dead and alive systemic sclerosis patients(P>0.05).Dead systemic sclerosis patients had later disease,onset(older than 60 years old)(P=0.002).Male gender(P=0.023),more diffuse skin involvement(P=0.000),more positive anti-RNP antibody(P=0.014),more pulmonary artery hypertension(P=0.000).more cardiac lesions(P=0.000),more cerebral infarets (P=0.035),more kidney lesions(P=0.000),and more scleroderma renal crisis(P=0.000) could be found jn dead sclerosis patients.Cox regression analysis showed that,onset later than 60 years old(OR=5.441.95% CI 2.126～13.926,P=0.000),male sex (OR=5.531,95%CI 2.014～15.190,P=0.001),anti-RNP antibedy positivity (OR=2.664,95%CI 1.016～6.592,P=0.034),diffuse skin involvement(OR=3.432,95%CI 1.400～8.411,P=0.007),pulmonary artery hypertension (OR=25.718,95% CI 5.954～111.085,P=0.000),cardiac lesions (OR=4.141.95%CI 1.685～10.159,P=0.002),kidney lesions(OR=4.214,95%CI 1.654～10.737,P=0.003) and scleroderma renal crisis (OR=20.677,95% CI 4.161～102.764.P=0.000)were risk factors for mortality in systemic sclerosis.Severe pulmonary hypertension was the most strong predictive factor for mortality in systemic sclerosis (OR=55.809,95% CI 12.879～241.832.P=0.000).Conclusion Aggressive therapy should be given to those systemic sclerosis patients with onset later than 60 years old,male sex,diffuse skin involvement,anti-RNP antibody positivity,cardiac involvement,kidney lesions,scleroderma renal crisis and pulmonary artery hypertension,especially seevere pulmonary hypertension.