Analysis of clinical features of mixed connective tissue disease associated with pulmonary arterial hypertension
Objective To investigate the clinical characteristics and risk factors of mixed connective tissue disease associated with pulmonary arterial hypertension(MCTD-PAH).Methods Twelve MCTD-PAH patients diagnosed by right heart catheterization(RHC)at Tianjin Medical University General Hospital were retrospectively included,and 36 MCTD patients without pulmonary arterial hypertension(MCTD-non-PAH)were randomly selected from the same period of hospitalization based on gender and age.The clinical features and auxiliary examination of the two groups were compared,and the survival status of the two groups was compared.Results The proportion of dyspnea after activity,myositis and pericardial effusion were higher in the MCTD-PAH group than those of the control group.Serum sedimentation rate and immunoglobulin G(IgG)levels were higher in the MCTD-PAH group.Multivariate Logistic regression analysis showed that dyspnea after activity and high level of IgG were risk factors for predicting the occurrence of PAH in MCTD.Three patients(16.7%)died in the MCTD-PAH group,and no patients died in the control group.Conclusion Pulmonary arterial hypertension is one of the serious complications of MCTD.MCTD patients have shortness of breath after activity and high level of IgG should be wary of concomitant PAH.
mixed connective tissue diseasepulmonary arterial hypertensionright heart catheterizationclinical features
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