Extract

Pulmonary arterial hypertension (PAH) in SLE: should we bother? Yes, but, as we shall discuss, only in patients with increased chance to develop this complication.

PAH is thought to be a life-threatening complication of CTDs, which is mainly based on reports of patients with SSc. Before advanced therapies became available, the prevalence of PAH in SSc was estimated to be between 7.5 and 12% with a 1-year survival rate of only 45% [1]. Despite the lack of data on survival and efficacy of therapeutic intervention in SSc–PAH, early diagnosis was advocated, which made sense. With the introduction of advanced therapies such as prostanoids, endothelin-receptor antagonists (ERAs) and phosphodiesterase (PDE) V inhibitors [2, 3] for PAH, clinicians now have the tools to improve pulmonary haemodynamics, functional status and probably even survival of patients with SSc–PAH. In this era of modern treatment, 1-year survival of SSc–PAH has increased from 45 to 78% [4].

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