{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,11]],"date-time":"2026-10-11T03:06:40Z","timestamp":1791688000660,"version":"4.3.4"},"reference-count":31,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2019,3,15]],"date-time":"2019-03-15T00:00:00Z","timestamp":1552608000000},"content-version":"vor","delay-in-days":14,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["ACR Open Rheumatology"],"published-print":{"date-parts":[[2019,3]]},"abstract":"Objective<\/jats:title>We sought to determine if any histopathologic component of the pulmonary microcirculation can distinguish systemic sclerosis (SS<\/jats:styled-content>c)\u2010related pulmonary fibrosis (PF<\/jats:styled-content>) with and without pulmonary hypertension (PH<\/jats:styled-content>).<\/jats:p><\/jats:sec>Methods<\/jats:title>Two pulmonary pathologists blindly evaluated 360 histologic slides from lungs of 31 SS<\/jats:styled-content>c\u2010PF<\/jats:styled-content> explants or autopsies with (n = 22) and without (n = 9) PH<\/jats:styled-content>. The presence of abnormal small arteries, veins, and capillaries (pulmonary microcirculation) was semiquantitatively assessed in areas of preserved lung architecture. Capillary proliferation (CP<\/jats:styled-content>) within the alveolar walls was measured by its distribution, extent (CP<\/jats:styled-content> % involvement), and maximum number of layers (maximum CP<\/jats:styled-content>). These measures were then evaluated to determine the strength of their association with right heart catheterization\u2013proven PH<\/jats:styled-content>.<\/jats:p><\/jats:sec>Results<\/jats:title>Using consensus measures, all measures of CP<\/jats:styled-content> were significantly associated with PH<\/jats:styled-content>. Maximum CP<\/jats:styled-content> had the strongest association with PH<\/jats:styled-content> (P<\/jats:italic> = 0.013; C statistic 0.869). Maximum CP<\/jats:styled-content> 2 or more layers and CP<\/jats:styled-content> % involvement 10% or greater were the optimal thresholds that predicted PH<\/jats:styled-content>, both with a sensitivity of 56% and specificity of 91%. The CP<\/jats:styled-content> was typically multifocal rather than focal or diffuse and was associated with a background pattern of usual interstitial pneumonia. There was a significant but weaker relationship between the presence of abnormal small arteries and veins and PH<\/jats:styled-content>.<\/jats:p><\/jats:sec>Conclusion<\/jats:title>In the setting of advanced SS<\/jats:styled-content>c\u2010PF<\/jats:styled-content>, the histopathologic feature of the pulmonary microcirculation best associated with PH<\/jats:styled-content> was capillary proliferation in architecturally preserved lung areas.<\/jats:p><\/jats:sec>","DOI":"10.1002\/acr2.1003","type":"journal-article","created":{"date-parts":[[2019,3,15]],"date-time":"2019-03-15T07:26:16Z","timestamp":1552634776000},"page":"26-36","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Capillary Proliferation in Systemic\u2010Sclerosis\u2010Related Pulmonary Fibrosis: Association with Pulmonary 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