0122: Frequency of PAH in scleroderma Algerian patients

Abstract

Systemic Sclerosis (SSc) represents the main connective tissue disease associated with PAH. This PAH is the leading cause of death, which only screening and early diagnosis can improve prognosis. Echocardiographic screening represents so fare the ideal tool for the early detection of this PAH. Evaluate the prevalence of PAH in Scleroderma Algerian Population and its distribution according to clinical forms of the disease. We conducted a cross-sectional descriptive study from Dec. 2010 to Dec. 2013. 202 patients (177 women and 25 men) with SSc were enrolled. We used a screening algorithm based on the maximum velocity of tricuspid regurgitation (VIT) and dyspnea. This defined patients at high risk (VIT >3 m/s or VIT between 2.8 and 3 m/s with dyspnea unexplained by another cause). Who were submitted to right heart catheterization (RHC) to confirm PH. Pulmonary hypertension suspected on echocardiography was confirmed by RHC in 68.2% of cases.It was a pre-capillary PH.VIT ≥3,48 m/s strongly suggests PH. The prevalence of PAH was 6%. The mean delay of it occurrence was 7.08 years after first diagnosis of SSc. PAH occurred in the first five years in 58.3% of cases and after five years in 41.6% of cases. It concerns the diffuse cutaneous forms in 33.3% or 0.5% of diffuse forms of our patient’s population and limited in 58.3% of cases or 0.58% limited cutaneous forms in our patients population. A frequency of 0,08% was found in limited form. Dyspnea is the primary symptom found, 67% of patients were in class III and IV at diagnosis. Prevalence of PAH in scleroderma was 6%, our findings are similar to the results reported in the literature: 5-12%, through echocardiographic screening and catheterize confirmation. This strengthens the fact that an echocardiogram should be performed at the time of SSc diagnostic and this regardless of the clinical form of SSc.