{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T20:04:03Z","timestamp":1790193843367,"version":"4.1.0"},"reference-count":51,"publisher":"Springer Science and Business Media LLC","issue":"2","license":[{"start":{"date-parts":[[2011,4,14]],"date-time":"2011-04-14T00:00:00Z","timestamp":1302739200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/2.0\/"},{"start":{"date-parts":[[2011,4,14]],"date-time":"2011-04-14T00:00:00Z","timestamp":1302739200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/2.0\/"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Arthritis Res Ther"],"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                        <jats:title>Introduction<\/jats:title>\n                        <jats:p>Systemic sclerosis (SSc) complicated by pulmonary arterial hypertension (PAH) carries a poor prognosis, despite pulmonary vascular dilating therapy. Platelet-derived growth factor receptor-\u03b2 (PDGFR-\u03b2) and epidermal growth factor receptor (EGFR) are potential therapeutic targets for PAH because of their proliferative effects on vessel remodelling. To explore their role in SScPAH, we compared PDGFR- and EGFR-mmunoreactivity in lung tissue specimens from SScPAH. We compared staining patterns with idiopathic PAH (IPAH) and pulmonary veno-occlusive disease (PVOD), as SScPAH vasculopathy differs from IPAH and sometimes displays features of PVOD. Immunoreactivity patterns of phosphorylated PDGFR-\u03b2 (pPDGFR-\u03b2) and the ligand PDGF-B were evaluated to provide more insight into the patterns of PDGFR-b activation.<\/jats:p>\n                     <\/jats:sec><jats:sec>\n                        <jats:title>Methods<\/jats:title>\n                        <jats:p>Lung tissue specimens from five SScPAH, nine IPAH, six PVOD patients and five controls were examined. Immunoreactivity was scored for presence, distribution and intensity.<\/jats:p>\n                     <\/jats:sec><jats:sec>\n                        <jats:title>Results<\/jats:title>\n                        <jats:p>All SScPAH and three of nine IPAH cases (<jats:italic>P<\/jats:italic> = 0.03) showed PDGFR-\u03b2-immunoreactivity in small vessels (arterioles\/venules); of five SScPAH <jats:italic>vs<\/jats:italic>. two of nine IPAH cases (<jats:italic>P<\/jats:italic> = 0.02) showed venous immunoreactivity. In small vessels, intensity was stronger in SScPAH <jats:italic>vs<\/jats:italic>. IPAH. No differences were found between SScPAH and PVOD. One of five normal controls demonstrated focally mild immunoreactivity. There were no differences in PDGF-ligand and pPDGFR-b-immunoreactivity between patient groups; however, pPDGFR-b-immunoreactivity tended to be more prevalent in SScPAH small vasculature compared to IPAH. Vascular EGFR-immunoreactivity was limited to arterial and arteriolar walls, without differences between groups. No immunoreactivity was observed in vasculature of normals.<\/jats:p>\n                     <\/jats:sec><jats:sec>\n                        <jats:title>Conclusions<\/jats:title>\n                        <jats:p>PDGFR-\u03b2-immunoreactivity in SScPAH is more common and intense in small- and post-capillary vessels than in IPAH and does not differ from PVOD, fitting in with histomorphological distribution of vasculopathy. PDGFR-\u03b2 immunoreactivity pattern is not paralleled by pPDGFR-\u03b2 or PDGF-B patterns. PDGFR-\u03b2- and EGFR-immunoreactivity of pulmonary vessels distinguishes PAH patients from controls.<\/jats:p>\n                     <\/jats:sec>","DOI":"10.1186\/ar3315","type":"journal-article","created":{"date-parts":[[2011,6,21]],"date-time":"2011-06-21T06:18:55Z","timestamp":1308637135000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":47,"title":["Platelet-derived growth factor receptor-\u03b2 and epidermal growth factor receptor in pulmonary vasculature of systemic sclerosis-associated pulmonary arterial hypertension versus idiopathic pulmonary arterial hypertension and pulmonary veno-occlusive disease: a case-control study"],"prefix":"10.1186","volume":"13","author":[{"given":"Maria J","family":"Overbeek","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anco","family":"Boonstra","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandre E","family":"Voskuyl","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Madelon C","family":"Vonk","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anton","family":"Vonk-Noordegraaf","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria PA","family":"van Berkel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wolter J","family":"Mooi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ben AC","family":"Dijkmans","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laurens S","family":"Hondema","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Egbert F","family":"Smit","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katrien","family":"Gr\u00fcnberg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2011,4,14]]},"reference":[{"key":"3078_CR1","doi-asserted-by":"publisher","first-page":"1989","DOI":"10.1056\/NEJMra0806188","volume":"360","author":"A Gabrielli","year":"2009","unstructured":"Gabrielli A, Avvedimento EV, Krieg T: Scleroderma. 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