Background Systemic sclerosis (SSc) is a rare, complex, connective tissue disorder. Interstitial
lung disease (ILD) is common in SSc, occurring in 35–52% of patients and accounting for …

Pulmonary sarcoidosis presents substantial management challenges, with limited evidence
on effective therapies and phenotypes. In the absence of definitive evidence, expert …

Background Pulmonary hypertension (PH) is a common complication of interstitial lung disease
(ILD) and is associated with worse outcomes and increased mortality. Evaluation of PH …

Background Riociguat is effective in delaying the time to clinical worsening (TCW) in
patients with groups 1 and 4 pulmonary hypertension. Research Question Is riociguat more …

Introduction: Alpha-1 antitrypsin deficiency (AATD) is a genetic disease that may be manifested
by chronic obstructive pulmonary disease. Despite professional society guidelines that …

A variety of phenotypic categorisations have been developed for sarcoidosis. Phenotyping
has been used for genetics studies and to guide treatment selection. The authors participated …

Alpha-1 antitrypsin deficiency (AATD) is a common hereditary disorder caused by mutations
in the SERPINA1 gene, which encodes alpha-1 antitrypsin (AAT; also known as alpha 1-…

Intravenous infusion of alpha-1 antitrypsin (AAT) was approved by the United States Food
and Drug Administration (FDA) to treat emphysema associated with AAT deficiency (AATD) in …

Oral treprostinil was recently labeled for treatment of pulmonary arterial hypertension. Similar
to the period immediately after parenteral treprostinil was approved, there is a significant …

… Author links open overlay panel Franck F. Rahaghi a , Maria Giovanna Trivieri b c , …
PATENT-PLUS [20] Randomized, double-blind, placebo-controlled trial 12 weeks + LTE f Riociguat (…