Systemic Sclerosis: Highlighting Respiratory Complications and Significance of Early Screening

Abstract

Systemic sclerosis (SSc) is an autoimmune disease that leads the patient to have a diverse clinical presentation encompassing several systems and a worse prognosis, mainly when complications arise. Most SSc-related deaths are caused by pulmonary hypertension (PH) and interstitial lung disease (ILD). This article focuses on pulmonary artery hypertension (PAH) and ILD as pulmonary consequences of SSc. We examined the grave effects regarding SSc's respiratory complications, which are concealed by the disease's clinical heterogeneity. In this article, we briefly reviewed the discussion of clinical features and management and the mortality associated with the sequelae. We further addressed the benefits and significance of screening for the disease and associated respiratory complications in SSc patients in this study.

Scleroderma pulmonary arterial hypertension: the same as idiopathic pulmonary arterial hypertension?

“…The high mortality associated with both SSc-PAH and IPAH has spurred efforts to improve detection strategies in susceptible populations and diagnose PAH earlier in patients' disease courses [33][34][35][36]. Algorithms for early detection of PAH in SSc patients have been shown to identify disease in patients with milder hemodynamics and less severe symptoms [2].…”