Prevalence of Pulmonary Arterial Hypertension in Patients With Connective Tissue Diseases in Egyptian Patients
Prevalence of Pulmonary Arterial Hypertension in Patients With Connective Tissue Diseases in Egyptian Patients
-  Measuring the incidence and prevalence of PAH in Egyptian patients with different types
 of MCD

 -  Determination of survival & the risk factors of mortality.
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.
Inclusion Criteria:

 - Patients diagnosed as connective tissue diseases fulfilling ACR criteria will be
 recruited

 Exclusion Criteria:

 non

 -