{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,10]],"date-time":"2026-02-10T08:26:14Z","timestamp":1770711974265,"version":"3.49.0"},"reference-count":10,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2026,1,27]],"date-time":"2026-01-27T00:00:00Z","timestamp":1769472000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,2,2]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Pulmonary arterial hypertension (PAH) is a progressive disease characterized by increased pulmonary vascular resistance and right ventricular failure. Sotatercept, a novel activin receptor ligand trap, has demonstrated promising haemodynamic benefits in PAH treatment. Eosinophilic granulomatosis with polyangiitis (EGPA) is an extremely rare, Th2-driven, small-vessel vasculitis, and its overlap with PAH is scarcely reported.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case summary<\/jats:title>\n                    <jats:p>We report the case of a woman with heritable PAH initially stabilized with oral therapy. In 2024, after further clinical decline, subcutaneous treprostinil was initiated. Subsequently, sotatercept was added, resulting in brief clinical improvement. Within weeks, however, the patient developed severe eosinophilia and exhibited laboratory and histopathological evidence of p-ANCA-positive necrotizing vasculitis, accompanied by renal and hepatocellular dysfunction.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Discussion<\/jats:title>\n                    <jats:p>This case suggests that sotatercept\u2019s modulation of the TGF-\u03b2 pathway may unmask latent autoimmune diseases such as EGPA in predisposed individuals. Although the temporal relationship between sotatercept initiation and the onset of vasculitis is compelling, both causality and underlying molecular mechanisms remain to be elucidated. Further studies are necessary to understand the potential immunomodulatory mechanisms of sotatercept.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/ehjcr\/ytag025","type":"journal-article","created":{"date-parts":[[2026,1,23]],"date-time":"2026-01-23T12:44:33Z","timestamp":1769172273000},"source":"Crossref","is-referenced-by-count":0,"title":["ANCA-associated vasculitis following sotatercept initiation in a patient with heritable pulmonary arterial hypertension and previously silent eosinophilic granulomatosis with polyangiitis: a case report"],"prefix":"10.1093","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0009-0001-7418-504X","authenticated-orcid":false,"given":"Angelo","family":"Laconi","sequence":"first","affiliation":[{"name":"Clinical and Interventional Cardiology, AOU Sassari , Sassari 07100 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University of Sassari , Sassari 07100 ,","place":["Italy"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2026,1,27]]},"reference":[{"key":"2026020907465118900_ytag025-B1","doi-asserted-by":"crossref","first-page":"1204","DOI":"10.1056\/NEJMoa2024277","article-title":"Sotatercept for the treatment of pulmonary arterial hypertension","volume":"384","author":"Humbert","year":"2021","journal-title":"N Engl J Med"},{"key":"2026020907465118900_ytag025-B2","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1002\/art.37715","article-title":"2012 revised international Chapel Hill consensus conference nomenclature of vasculitides","volume":"65","author":"Jennette","year":"2013","journal-title":"Arthritis Rheum"},{"key":"2026020907465118900_ytag025-B3","doi-asserted-by":"crossref","first-page":"1560","DOI":"10.1093\/rheumatology\/kep304","article-title":"Incidence and survival rates in Wegener\u2019s granulomatosis, microscopic 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