The lung is a common site of complications of systemic connective tissue disease (CTD),
and lung involvement can present in several ways. Interstitial lung disease (ILD) and …

Background: Right ventricular (RV) adaptation to acute and chronic pulmonary hypertensive
syndromes is a significant determinant of short- and long-term outcomes. Although …

… Conflict of interest: SC Mathai reports personal fees for consultancy and data and safety
monitoring board membership from Actelion, personal fees for consultancy from United …

Rationale: Right ventricular (RV) function is an important determinant of prognosis in pulmonary
hypertension. However, noninvasive assessment of the RV function is often limited by …

Background— Right ventricular failure from increased pulmonary vascular loading is a major
cause of morbidity and mortality, yet its modulation by disease remains poorly understood. …

Objective Pulmonary arterial hypertension related to scleroderma (PAH‐Scl) is associated
with high morbidity and mortality as well as poorer response to therapy and worse outcomes …

Objective Pulmonary hypertension (PH) is an important cause of mortality in systemic sclerosis
(SSc), where it can be isolated (pulmonary arterial hypertension [PAH]) or associated with …

Rationale: Although commonly used as the primary outcome measure of clinical trials in
pulmonary arterial hypertension (PAH), the minimal important difference (MID) of the 6-minute …

Rationale: Pulmonary arterial hypertension (PAH) related to systemic sclerosis (SSc) has a
poorer prognosis compared with other forms of PAH for reasons that remain unexplained. …

Background— Systemic sclerosis–associated pulmonary artery hypertension (SScPAH) has
a worse prognosis compared with idiopathic pulmonary arterial hypertension (IPAH), with a …