Prevalence and Predictors of Pulmonary Arterial Hypertension in a Sample of Iraqi Patients with Systemic Sclerosis: A Cross-Sectional Study

Abstract

Background: Pulmonary arterial hypertension (PAH) is now the most frequent cause of death in systemic sclerosis (SSc). Aims: The aims of the present study were to evaluate the prevalence of pulmonary arterial hypertension (PAH) and predictors in SSc among Iraqi patients. Materials and Methods: A cross-sectional study conducted on 56 patients with SSc diagnosed according to the criteria developed by the American College of Rheumatology. Baseline characteristics [age, sex, body mass index (BMI), duration of SSc, type of SSc, duration of Raynaud’s phenomenon, and presence of telangiectasia] were documented.  Antiscleroderma 70 (anti-SCL70) and anticentromere antibodies were measured. Doppler echocardiography was done to diagnose PAH. A risk score was obtained from 7 criteria, namely: Anti-Centromere Ab, Limited disease type, short duration of Raynaud's phenomena (<2.5 years), older age group (40+ years), absence of Telangiectasia, female gender, and absence of anti SCL70 Ab.  Results: We found that PAH was present in 11 (19.6%) SSc patients with a 95% confidence interval of (9.2% to 30.0%). Risk score in addition to anti-centromere antibodies were enough to diagnose PAH with accuracy level of 89.3%. Conclusions: PAH in SSc occurs in significant proportion of patients. Risk score and anti-centromere antibodies had high accuracy level in predicting PAH. Screening of patients with SSc for PAH will help in early diagnosis and appropriate timely therapeutic intervention before significant endorgan damage occurs.

Key words Systemic sclerosis. Connective  tissue. Pulmonary hypertension