Connective Tissue Disease–associated Pulmonary Arterial Hypertension in the Modern Treatment Era

Abstract

Survival of patients with SSc-PAH in the modern treatment era is better than in historical series. A significant proportion of patients with mild functional impairment or exercise-induced SSc-PAH have evidence of disease progression. Survival of patients with respiratory disease-associated pulmonary hypertension is inferior. SLE-PAH has a better prognosis than SSc-PAH.

Exercise‐induced pulmonary hypertension associated with systemic sclerosis: Four distinct entities

“…Isolated survival of SSc patients with PAH while receiving pulmonary vasodilator therapy was recently reported to be 78% (1-year rate), 68% (2-year rate), and 47% (3-year rate) (7,19,20). Importantly, Tolle and coworkers have suggested that exercise-induced pulmonary hypertension (PH) may represent an intermediate phenotype "between" normal and resting PH (21).…”

“…Importantly, Tolle and coworkers have suggested that exercise-induced pulmonary hypertension (PH) may represent an intermediate phenotype "between" normal and resting PH (21). In addition, Condliffe and coworkers found that 19% of SSc patients with PAH who had exercise-induced PH developed resting PAH after ϳ2.3 years (19). Others, however, have argued that a certain degree of exerciseinduced PH may be explained by increasing age, persistent hypoxia, and/or systemic hypertension (22,23).…”

“…The UK group prospectively reported survival in 42 SSc patients with exercise-induced PH, of whom 8 (19%) progressed to having resting PAH within a mean Ϯ SD 838 Ϯ 477 days and of whom 4 (9.5%) died secondary to complications of pulmonary vascular disease within 3 years of diagnosis (19). PH in SSc may be an abnormal hemodynamic response and part of a continuum from normal to resting PH.…”

“…Few studies have used right heart catheterization to address whether an mPAP Ͼ30 mm Hg during exercise represents an aberrant pulmonary vascular system in "at-risk" populations (19,21,28,34,38,39,43). Recently, Tolle and coworkers evaluated a large cohort of non-SSc symptomatic patients who underwent simultaneous cardiopulmonary exercise testing and right heart catheterization (21).…”

Impact of bosentan therapy on stress-induced pulmonary hypertension in patients with scleroderma systematica

“…Это мнение подтвержда-ется и другими авторами, обнаружившими, что у 19% па-циентов с ССД, продемонстрировавшими СИ-ЛГ, разви-вается ЛГ в покое в течение примерно 2,3 года [21]. Таким образом, выявление СИ-ЛГ у пациентов с ССД может по-мочь ранней установке диагноза, инициации терапии и, возможно, улучшению прогноза [22].…”