ReviewPulmonary circulation2026
Sotatercept in Pulmonary Langerhans Cell Histiocytosis-Associated Pulmonary Hypertension: A Case Series and Systematic Review.
Review in Pulmonary circulation, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.
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.
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.
Who cites it
1 citing paper in PubMed.
- Sotatercept in Pulmonary Langerhans Cell Histiocytosis-Associated Pulmonary Hypertension: A Case Series and Systematic Review.Pulmonary circulation · 2026Review
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
8 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Pulmonary Langerhans cell histiocytosis (PLCH) frequently complicated by pulmonary hypertension (PH), which markedly worsens prognosis. We retrospectively reviewed three institutional PLCH-PH cases treated with off-label Sotatercept added to background triple therapy and performed a systematic review of published PLCH-PH reports (PubMed/Embase through May 2025). Our three patients (ages 69, 62, 49; all female) had progressive vascular disease despite optimized vasodilator therapy. Following Sotatercept, all experienced ≥ 3-fold increases in 6-min walk distance and improved functional class. Hemodynamics improved substantially: right-atrial pressure -78.6%, pulmonary vascular resistance -75.5%, pulmonary artery systolic pressure -58.5%, mean PAP - 56.0%, PA diastolic pressure -51.0%, PAWP - 47.1%; cardiac output and index rose +75.9% and +73.8%, respectively. BNP/NT-proBNP normalized. Systematic review identified 34 published cases (2010-2025): mean age 37.2 ± 13.7 years, 44.1% female, 45.7% current/former smokers. Reported management strategies included targeted vasodilators, cytotoxic PLCH therapies (e.g., cladribine), smoking cessation, and selective surgery/transplant. In this small series, Sotatercept added to background therapy produced marked clinical and hemodynamic gains in refractory PLCH-PH. These effects of Sotatercept in Group 5 PH-are encouraging but limited by sample size and retrospective design. Prospective, collaborative studies are needed to define safety, efficacy, and optimal patient selection.
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Registered trials
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.