What does this research mean for the field?

Early screening and timely initiation of combination therapy in patients with connective tissue disease-associated pulmonary arterial hypertension reduces clinical worsening and mortality, though long-term outcomes remain uncertain. Novelty: ClaimNovelty.SYNTHESIS. Consensus alignment: ConsensusAlignment.NEUTRAL.

What question did this study set out to answer?

To evaluate evidence on long-term health outcomes and identify clinical practices for early detection, screening, and treatment initiation in patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH).

September 10, 2026Modern RheumatologyOpen Access

Early detection, treatment initiation, and long-term health outcomes in patients with connective tissue disease-associated pulmonary arterial hypertension: A systematic literature review and expert consensus

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Authors

TATatsuya Atsumi SBSang-Cheol Bae WHWen-Nan Huang

Discussion

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Overview

Systematic review and Delphi consensus reveals benefits of early screening and combination therapy in connective tissue disease-associated pulmonary arterial hypertension, highlighting treatment...

Key Points

To evaluate evidence on long-term health outcomes and identify clinical practices for early detection, screening, and treatment initiation in patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH).
Conducted a systematic literature review across observational studies, randomized controlled trials, and clinical guidelines evaluating adult CTD-PAH, including systemic lupus erythematosus, systemic sclerosis, and mixed connective tissue disease.
Convened an expert panel through three Delphi consensus rounds to develop clinical recommendations for screening protocols and treatment strategies.
Synthesized 63 publications (54 studies and 9 guidelines), finding that combination therapy reduced mortality and disease progression compared to monotherapy.
Achieved consensus across three Delphi rounds supporting early screening in systemic sclerosis, systemic lupus erythematosus, and mixed connective tissue disease, alongside timely therapy with endothelin receptor antagonists, phosphodiesterase-5 inhibitors, and vasodilators.
Identified that long-term outcome data remain limited and uncertain across existing literature.

Cite This Study

Atsumi et al. (2026) studied this question.

synapsesocial.com/papers/6aa27bcf58559d80afc7553b https://doi.org/10.1093/mr/roag076

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September 10, 2026Modern RheumatologyOpen Access

Early detection, treatment initiation, and long-term health outcomes in patients with connective tissue disease-associated pulmonary arterial hypertension: A systematic literature review and expert consensus

View Full Paper

Ask AI

Bookmark

Authors

TATatsuya Atsumi SBSang-Cheol Bae WHWen-Nan Huang

Discussion

Member takes

Overview

Systematic review and Delphi consensus reveals benefits of early screening and combination therapy in connective tissue disease-associated pulmonary arterial hypertension, highlighting treatment...

Key Points

To evaluate evidence on long-term health outcomes and identify clinical practices for early detection, screening, and treatment initiation in patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH).
Conducted a systematic literature review across observational studies, randomized controlled trials, and clinical guidelines evaluating adult CTD-PAH, including systemic lupus erythematosus, systemic sclerosis, and mixed connective tissue disease.
Convened an expert panel through three Delphi consensus rounds to develop clinical recommendations for screening protocols and treatment strategies.
Synthesized 63 publications (54 studies and 9 guidelines), finding that combination therapy reduced mortality and disease progression compared to monotherapy.
Achieved consensus across three Delphi rounds supporting early screening in systemic sclerosis, systemic lupus erythematosus, and mixed connective tissue disease, alongside timely therapy with endothelin receptor antagonists, phosphodiesterase-5 inhibitors, and vasodilators.
Identified that long-term outcome data remain limited and uncertain across existing literature.

Cite This Study

Atsumi et al. (2026) studied this question.

synapsesocial.com/papers/6aa27bcf58559d80afc7553b https://doi.org/10.1093/mr/roag076

View Full Paper

Ask AI

Bookmark