Prevalence of Pulmonary Arterial Hypertension in Patients With Connective Tissue in Egyptian Patients ( Single Center Study)

PH is defined as a mean pulmonary artery pressure greater than 25 mmHg. PAH can be assessed by different means, however right heart catheterization (RHC) is the gold standard diagnostic method .

Among the different subgroups of pulmonary arterial hypertension (PAH), those associated with connective tissue diseases (CTDs) are a specific entity with distinct hemodynamic and prognostic features reflecting the importance of a rheumatological assessment in PAH, especially with the unfavorable prognostic impact in these patients.

Compared to patients with idiopathic PAH, patients with CTD-associated PAH have a higher mortality and a lower walking distance on the 6-minute walk test, higher levels of B-type natriuretic peptide, worse right ventricular function, more left-sided heart dysfunction, lower lung function, and more pericardial disease.

That's why, PAH screening is routinely performed in systemic sclerosis (SSc) and in other CTDs and also, as suggested by international guidelines, it is important to rule out CTDs in patients with PAH, because patients diagnosed as idiopathic PAH (iPAH) may have an associated CTD previously unrecognized.

Although the similarities in pathogenesis of PAH in CTD such as systemic sclerosis (SSc), systemic lupus erythematosus (SLE), and mixed connective tissue disease (MCTD), there are variabilities in outcomes in patients with CTD-associated PAH.