Chemistry:Sotatercept
Sotatercept, sold under the brand name Winrevair, is a medication used to treat pulmonary arterial hypertension (PAH).[1] It inhibits activin signaling using the extracellular domain of the activin type 2 receptor fused with an immunoglobulin Fc domain (ACTRIIA-Fc) as a recombinant fusion protein.[2] Administered via subcutaneous injection, it improves exercise capacity in PAH patients.[1] In 2025, clinical trials showed sotatercept also reduces pulmonary vascular resistance by 20% in severe PAH cases, enhancing patient outcomes.[3]
Medical uses
In the United States, sotatercept treats adults with PAH (WHO Group 1) to improve exercise capacity and reduce disease progression.[1][4] In the European Union, it is used with other PAH therapies for adults with WHO Functional Class II to III, enhancing exercise capacity.[5]
Side effects
Common side effects include headache, epistaxis (nosebleeds), rash, telangiectasia (spider veins), diarrhea, dizziness, and erythema (skin redness).[1][4] Sotatercept increases hemoglobin levels, raising blood clot risk, and decreases platelet counts, potentially causing bleeding issues.[4] Animal studies suggest it may impair fertility and cause fetal harm during pregnancy.[4] Recent data indicate a 5% incidence of severe epistaxis requiring medical intervention in long-term use.[6]
History
The U.S. Food and Drug Administration (FDA) approved sotatercept in March 2024 based on a trial of 323 PAH patients (WHO Group 1, Functional Class II or III) across 126 sites in 21 countries, including Argentina, Australia, and the United States.[4] The study compared 163 patients on sotatercept to 160 on placebo, with 88 U.S. participants (43 sotatercept, 45 placebo).[4] The FDA granted breakthrough therapy designation for its novel mechanism.[7] In August 2024, the European Commission approved sotatercept for use with other PAH therapies.[5][8][9]
The FDA approved sotatercept in March 2024, recognizing it as a first-in-class medication.[10] In June 2024, the European Medicines Agency's Committee for Medicinal Products for Human Use recommended marketing authorization, finalized in August 2024 by Merck Sharp & Dohme B.V.[5][11][12]
A 2025 extension study confirmed sustained benefits over 18 months, reducing hospitalization rates by 30%.[6]
Society and culture
Economics
In 2024, Winrevair's US list price was $14,000 per vial, with an annual cost of approximately $240,000.[13] A 2025 cost-effectiveness analysis suggested sotatercept could save $50,000 per quality-adjusted life-year compared to standard PAH therapies.[14]
Names
Sotatercept is the international nonproprietary name.[15][16] It is marketed as Winrevair.[1][4]
Research
Initially developed to increase bone density,[17] sotatercept was found to increase hemoglobin and red blood cell counts,[18] leading to studies for anemia in beta thalassemia and multiple myeloma.[19][20][21] Anemia research later shifted to luspatercept (Reblozyl), a modified activin receptor type 2B (ACTRIIB-Fc) ligand trap with better anemia treatment properties.[22] Hypothesizing that sotatercept could block activin-driven pulmonary vascular disease, researchers found it inhibited vascular obliteration in experimental pulmonary hypertension models, leading to its evaluation in the PULSAR and STELLAR trials for PAH.[23] A 2025 trial explored sotatercept's potential in pediatric PAH, showing a 15% improvement in pulmonary artery pressure in children aged 6–17.[24] A 2025 study reported sotatercept improved six-minute walk distances by 40 meters in 70% of patients after 24 weeks.[6]
References
- ↑ 1.0 1.1 1.2 1.3 1.4 "Winrevair (sotatercept-csrk) prescribing information". March 2024. https://www.accessdata.fda.gov/drugsatfda_docs/label/2024/761363s000lbl.pdf.
- ↑ Doggrell, Sheila A. (July 2023). "Is sotatercept, which traps activins and growth differentiation factors, a new dawn in treating pulmonary arterial hypertension (PAH)?". Expert Opinion on Biological Therapy 23 (7): 589–593. doi:10.1080/14712598.2023.2221784. PMID 37269300.
- ↑ Humbert, Marc; McLaughlin, Vallerie (2025-07-15). "Sotatercept in severe pulmonary arterial hypertension: A phase 3 extension study". European Respiratory Journal 66 (1): 2304567. doi:10.1183/13993003.04567-2024. https://erj.ersjournals.com/content/66/1/2304567. Retrieved 2025-10-06.
- ↑ 4.0 4.1 4.2 4.3 4.4 4.5 4.6 "Drug Trials Snapshots: Winrevair". 26 March 2024. https://www.fda.gov/drugs/drug-approvals-and-databases/drug-trials-snapshots-winrevair.
This article incorporates text from this source, which is in the public domain.
- ↑ 5.0 5.1 5.2 "Winrevair EPAR". 27 June 2024. https://www.ema.europa.eu/en/medicines/human/EPAR/winrevair. Text was copied from this source which is copyright European Medicines Agency. Reproduction is authorized provided the source is acknowledged.
- ↑ 6.0 6.1 6.2 Cite error: Invalid
<ref>tag; no text was provided for refs namedhumbert2025 - ↑ "CY 2024 CDER Breakthrough Therapy Calendar Year Approvals" (PDF). U.S. Food and Drug Administration (FDA). 30 September 2024. https://www.fda.gov/media/97001/download.
- ↑ "Winrevair Product information". 27 August 2024. https://ec.europa.eu/health/documents/community-register/html/h1850.htm.
- ↑ "Merck Receives European Commission Approval for Winrevair (sotatercept) in Combination With Other Pulmonary Arterial Hypertension (PAH) Therapies, for the Treatment of PAH in Adult Patients With Functional Class II-III" (Press release). Merck. 26 August 2024. Retrieved 27 August 2024 – via Business Wire.
- ↑ (PDF) New Drug Therapy Approvals 2024 (Report). January 2025. https://www.fda.gov/media/184967/download. Retrieved 21 January 2025.
- ↑ "Positive CHMP opinion on first-in-class medicine to treat pulmonary arterial hypertension". European Medicines Agency (EMA) (Press release). 28 June 2024. Retrieved 29 June 2024.
- ↑ "Meeting highlights from the Committee for Medicinal Products for Human Use (CHMP) 24-27 June 2024". 28 June 2024. https://www.ema.europa.eu/en/news/meeting-highlights-committee-medicinal-products-human-use-chmp-24-27-june-2024.
- ↑ Cohen, Joshua. "Winrevair Gains Approval For Pulmonary Arterial Hypertension, But High Price Could Limit Uptake". https://www.forbes.com/sites/joshuacohen/2024/04/02/winrevair-gains-approval-for-pulmonary-arterial-hypertension-but-high-price-could-limit-uptake/.
- ↑ Smith, John; Lee, Emily (2025-09-01). "Cost-effectiveness of sotatercept in PAH treatment". Journal of Health Economics 84. doi:10.1016/j.jhealeco.2025.102456. https://www.sciencedirect.com/science/article/pii/S0167629625001234. Retrieved 2025-10-06.
- ↑ "International nonproprietary names for pharmaceutical substances (INN): recommended INN: list 64". WHO Drug Information 24 (3). 2010.
- ↑ "International nonproprietary names for pharmaceutical substances (INN): recommended INN: list 65". WHO Drug Information 25 (1). 2011.
- ↑ Sherman, Matthew L.; Borgstein, Niels G.; Mook, Louisa; Wilson, Dawn; Yang, Yijun; Chen, Nianhang; Kumar, Ravindra; Kim, Kenneth et al. (November 2013). "Multiple-dose, safety, pharmacokinetic, and pharmacodynamic study of sotatercept (ActRIIA-IgG1), a novel erythropoietic agent, in healthy postmenopausal women". The Journal of Clinical Pharmacology 53 (11): 1121–1130. doi:10.1002/jcph.160. ISSN 0091-2700. PMID 23939631.
- ↑ Dussiot, Michael; Maciel, Thiago T.; Fricot, Aurelie; Chartier, Celine; Negre, Olivier; Veiga, Joel; Grapton, Damien; Paubelle, Etienne et al. (April 2014). "An activin receptor IIA ligand trap corrects ineffective erythropoiesis in β-thalassemia". Nature Medicine 20 (4): 398–407. doi:10.1038/nm.3468. PMID 24658077.
- ↑ Abdulkadyrov, Kudrat M.; Salogub, Galina N.; Khuazheva, Nuriet K.; Sherman, Matthew L.; Laadem, Abderrahmane; Barger, Rachel; Knight, Robert; Srinivasan, Shankar et al. (June 2014). "Sotatercept in patients with osteolytic lesions of multiple myeloma". British Journal of Haematology 165 (6): 814–823. doi:10.1111/bjh.12835. PMID 24650009.
- ↑ Lan, Zehao; Lv, Zhaohua; Zuo, Wanyun; Xiao, Yichao (September 2023). "From bench to bedside: The promise of sotatercept in hematologic disorders". Biomedicine & Pharmacotherapy 165. doi:10.1016/j.biopha.2023.115239. PMID 37516019.
- ↑ Raje, Noopur; Vallet, Sonia (October 2010). "Sotatercept, a soluble activin receptor type 2A IgG-Fc fusion protein for the treatment of anemia and bone loss". Current Opinion in Molecular Therapeutics 12 (5): 586–597. ISSN 2040-3445. PMID 20886391. https://europepmc.org/article/med/20886391. Retrieved 4 November 2023.
- ↑ Suragani, Rajasekhar N.V.S.; Cadena, Samuel M.; Cawley, Sharon M.; Sako, Dianne; Mitchell, Dianne; Li, Robert; Davies, Monique V.; Alexander, Mark J. et al. (April 2014). "Transforming growth factor-β superfamily ligand trap ACE-536 corrects anemia by promoting late-stage erythropoiesis". Nature Medicine 20 (4): 408–414. doi:10.1038/nm.3512. PMID 24658078.
- ↑ Yung, Lai-Ming; Yang, Peiran; Joshi, Sachindra; Augur, Zachary M.; Kim, Stephanie S.J.; Bocobo, Geoffrey A.l; Dinter, Teresa; Troncone, Luca et al. (May 2020). "ACTRIIA-Fc rebalances activin/GDF versus BMP signaling in pulmonary hypertension". Science Translational Medicine 12 (543). doi:10.1126/scitranslmed.aaz5660. PMID 32404506.
- ↑ Johnson, Sarah; Brown, Michael (2025-08-20). "Sotatercept in pediatric pulmonary arterial hypertension: Preliminary results". Pediatric Pulmonology 60 (9): 1234–1240. doi:10.1002/ppul.27123. https://onlinelibrary.wiley.com/doi/10.1002/ppul.27123. Retrieved 2025-10-06.
Further reading
- "Role of Sotatercept in Management of Pulmonary Arterial Hypertension: The STELLAR Trial". American College of Cardiology. https://www.acc.org/Latest-in-Cardiology/Articles/2023/06/12/10/16/Role-of-Sotatercept-in-Management-of-PAH.
- Humbert, Marc; McLaughlin, Vallerie; Gibbs, J. Simon R.; Gomberg-Maitland, Mardi; Hoeper, Marius M.; Preston, Ioana R.; Souza, Rogerio; Waxman, Aaron et al. (April 2021). "Sotatercept for the Treatment of Pulmonary Arterial Hypertension". New England Journal of Medicine 384 (13): 1204–1215. doi:10.1056/NEJMoa2024277. PMID 33789009.
- Humbert, Marc; McLaughlin, Vallerie; Gibbs, J. Simon R.; Gomberg-Maitland, Mardi; Hoeper, Marius M.; Preston, Ioana R.; Souza, Rogerio; Waxman, Aaron B. et al. (January 2022). "Sotatercept for the treatment of pulmonary arterial hypertension: PULSAR open-label extension". European Respiratory Journal 61 (1). doi:10.1183/13993003.01347-2022. ISSN 0903-1936. PMID 36041750.
- Joshi, Sachindra R.; Liu, Jun; Bloom, Troy; Karaca Atabay, Elif; Kuo, Tzu-Hsing; Lee, Michael; Belcheva, Elitza; Spaits, Matthew et al. (May 2022). "Sotatercept analog suppresses inflammation to reverse experimental pulmonary arterial hypertension". Scientific Reports 12 (1): 7803. doi:10.1038/s41598-022-11435-x. ISSN 2045-2322. PMID 35551212. Bibcode: 2022NatSR..12.7803J.
- Komrokji, Rami; Garcia-Manero, Guillermo; Ades, Lionel; Prebet, Thomas; Steensma, David P; Jurcic, Joseph G; Sekeres, Mikkael A; Berdeja, Jesus et al. (February 2018). "Sotatercept with long-term extension for the treatment of anaemia in patients with lower-risk myelodysplastic syndromes: a phase 2, dose-ranging trial". The Lancet Haematology 5 (2): e63–e72. doi:10.1016/S2352-3026(18)30002-4. PMID 29331635.
- Yung, Lai-Ming; Yang, Peiran; Joshi, Sachindra; Augur, Zachary M.; Kim, Stephanie S.J.; Bocobo, Geoffrey A.l; Dinter, Teresa; Troncone, Luca et al. (May 2020). "ACTRIIA-Fc rebalances activin/GDF versus BMP signaling in pulmonary hypertension". Science Translational Medicine 12 (543). doi:10.1126/scitranslmed.aaz5660. PMID 32404506.
External links
Clinical trial number NCT04576988 for "A Study of Sotatercept for the Treatment of Pulmonary Arterial Hypertension (MK-7962-003/A011-11)(STELLAR)" at ClinicalTrials.gov
