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.
Highlights
BackgroundSystemic sclerosis is an autoimmune disease that continues to challenge the medical fraternity due to its disparate nature [1]
We briefly reviewed the discussion of clinical features and management and the mortality associated with the sequelae
Any delay in diagnosing pulmonary artery hypertension (PAH) in Systemic sclerosis (SSc) patients will lead to poor prognosis; studies recommending early detection and immediate treatment are critical for successful management with better prognostic outcomes [31] (Table 1)
Summary
Systemic sclerosis is an autoimmune disease that continues to challenge the medical fraternity due to its disparate nature [1]. Despite recent breakthroughs in treatment, the survival rates for the above complications remain dismal, especially for SScPAH patients with a median survival period of only three years [13,14] Given such poor long-term outcomes, it is logical and imperative to screen early disease manifestations in the aforementioned illnesses. 2. Evaluate the importance and highlight the benefits of early screening of ILD and PAH in SSc. The fundamental concern of researchers looking into mortality associated with systemic sclerosis (SSc) is progressive pulmonary deterioration since pulmonary complications have replaced scleroderma renal crisis as the primary cause of SSc-related death [16]. ILD and pulmonary arterial hypertension (PAH) are the leading causes of death in patients with systemic autoimmune disorders like SSc [18]. Any delay in diagnosing PAH in SSc patients will lead to poor prognosis; studies recommending early detection and immediate treatment are critical for successful management with better prognostic outcomes [31] (Table 1)
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