Evidence map›Paper›PMID 41594679›Full record

ReviewBiomolecules2026

Connective Tissue Disease-Associated Pulmonary Arterial Hypertension: Current Therapeutic Strategies and Future Prospects.

Yukina Mizuno Yokoyama, Ryu Watanabe, Tomohiro Yamaguchi, Ryuhei Ishihara, Mayu Shiomi, Yuya Fujita, Masao Katsushima, Kazuo Fukumoto, Yoichiro Haji, Shinsuke Yamada and 1 more

Abstract readReview
In one paragraph

Review in Biomolecules, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

1 citing paper in PubMed.

  1. Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

11 authors.

Yukina Mizuno YokoyamaDepartment of Clinical Immunology, Osaka Metropolitan University Graduate School of Medicine, 1-4-3, Asahi-machi, Abeno-Ku, Osaka 545-8585, Japan.
Ryu WatanabeDepartment of Clinical Immunology, Osaka Metropolitan University Graduate School of Medicine, 1-4-3, Asahi-machi, Abeno-Ku, Osaka 545-8585, Japan.ORCID 0000-0002-1089-5296
Tomohiro YamaguchiDepartment of Cardiovascular Medicine, Osaka Metropolitan University Graduate School of Medicine, Osaka 545-8585, Japan.ORCID 0000-0003-2576-9409
Ryuhei IshiharaDepartment of Clinical Immunology, Osaka Metropolitan University Graduate School of Medicine, 1-4-3, Asahi-machi, Abeno-Ku, Osaka 545-8585, Japan.
Mayu ShiomiDepartment of Clinical Immunology, Osaka Metropolitan University Graduate School of Medicine, 1-4-3, Asahi-machi, Abeno-Ku, Osaka 545-8585, Japan.
Yuya FujitaDepartment of Clinical Immunology, Osaka Metropolitan University Graduate School of Medicine, 1-4-3, Asahi-machi, Abeno-Ku, Osaka 545-8585, Japan.
Masao KatsushimaDepartment of Clinical Immunology, Osaka Metropolitan University Graduate School of Medicine, 1-4-3, Asahi-machi, Abeno-Ku, Osaka 545-8585, Japan.
Kazuo FukumotoDepartment of Clinical Immunology, Osaka Metropolitan University Graduate School of Medicine, 1-4-3, Asahi-machi, Abeno-Ku, Osaka 545-8585, Japan.ORCID 0000-0002-8576-5816
Yoichiro HajiDepartment of Rheumatology, Daido Hospital, Nagoya 457-8511, Japan.
Shinsuke YamadaDepartment of Clinical Immunology, Osaka Metropolitan University Graduate School of Medicine, 1-4-3, Asahi-machi, Abeno-Ku, Osaka 545-8585, Japan.
Motomu HashimotoDepartment of Clinical Immunology, Osaka Metropolitan University Graduate School of Medicine, 1-4-3, Asahi-machi, Abeno-Ku, Osaka 545-8585, Japan.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) is a severe form of pulmonary hypertension with poor prognosis. It most commonly arises in systemic sclerosis (SSc), followed by systemic lupus erythematosus (SLE) and mixed connective tissue disease (MCTD). Its pathogenesis involves a complex interplay of immune dysregulation, chronic inflammation, endothelial injury, vascular remodeling, and fibrosis. Although vasodilators targeting the endothelin, nitric oxide, and prostacyclin pathways remain the therapeutic backbone, newer agents-including the activin signal inhibitor sotatercept and inhaled treprostinil-have expanded treatment options. Immune-targeted therapies such as glucocorticoids, cyclophosphamide, mycophenolate mofetil, rituximab, and IL-6 receptor inhibitors may benefit inflammation-dominant PAH phenotypes, while fibrotic phenotypes continue to demonstrate limited responsiveness. In addition to brain natriuretic peptide (BNP), N-terminal (NT)-proBNP and disease-specific autoantibodies, emerging biomarkers show promise for early detection, risk stratification, and personalized treatment, though validation in CTD-PAH is lacking. Advances in animal models replicating immune-mediated vascular injury and fibrosis have further improved mechanistic understanding. Despite these developments, substantial unmet needs remain, including the absence of disease-specific therapeutic strategies, limited biomarker integration into clinical practice, and a scarcity of large, well-designed trials targeting individual CTD subtypes. Addressing these gaps will be essential for improving prognosis in patients with CTD-PAH.

Indexed as

Connective Tissue DiseasesPulmonary Arterial HypertensionAnimalsBiomarkersHumansHypertension, PulmonaryScleroderma, SystemicBiomarkersanimal modelbiomarkerconnective tissue diseasepulmonary arterial hypertensionsystemic sclerosis

Identifiers

PMID41594679
PMCPMC12839107

What Socratic holds

Textmetadata
LicenceCC BY
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the Socratic graph.