Systemic Sclerosis-Associated Pulmonary Arterial Hypertension

Fatal Cardiac Tamponade in Newly Diagnosed Systemic Sclerosis with Early Cutaneous Disease: A Case Report

“…A right heart catheterization could not be done because the facility is not available in our setting. Pulmonary arterial hypertension (PAH) is associated with a 50% three-year mortality rate in SSc and it is found in up to 12% of all patients with SSc [11,12]. There are conflicting reports on the more prevalent disease between SSc-associated PAH and idiopathic PAH with some studies suggesting that the former may be as many as four times the latter and others finding the opposite [13,14].…”

“…This is likely responsible for a threefold increase in the risk of death as compared with patients with idiopathic PAH. Moreover, for unknown reasons, conventional treatments for idiopathic PAH are less effective in PAH in SSc [11]. Primary myocardial disease, among other factors, is associated with the presence of various degrees of diastolic dysfunction in some patients with SSc [15].…”

Early diagnosis and management of systemic sclerosis

“…Up to 80% of patients with SSc may develop lung fibrosis, and this is clinically significant in around one-third. 9 Around 10% of patients with SSc may develop PAH, 10 and this has a significant impact on survival. Three-year survival in SSc patients with PAH has been reported to be 56% compared to 94% in those without PAH.…”

Investigation of Lung Involvement in Connective Tissue Disorders

“…As already stated, PAH severely influences survival since less than half of patients with scleroderma-related PAH are still alive 3 years after its diagnosis. In comparison with idiopathic PAH, patients with scleroderma-related PAH have a threefold increased risk of death [135,153] . Dyspnea, fatigue and palpitations which are the most common symptoms of pulmonary hypertension are often underestimated or/and are frequently attributed to heart and lung comorbidities leading to a significant delay of the diagnosis unless the patient is regularly screened or develops more alarming and specific figure 7.…”

“…table 3). In this latter clinical setting in case of positive anticentromere autoantibodies scleroderma-related PAH may ensue and adversely affects prognosis [135] . Diffuse cutaneous scleroderma additionally affects the skin of the chest and abdominal wall plus internal organs, mainly the lungs.…”