{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T23:05:23Z","timestamp":1787007923218,"version":"build-2736575974"},"reference-count":40,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2016,12,1]],"date-time":"2016-12-01T00:00:00Z","timestamp":1480550400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2016,12,1]],"date-time":"2016-12-01T00:00:00Z","timestamp":1480550400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Pulm. circ."],"published-print":{"date-parts":[[2016,12]]},"abstract":"<jats:p>\n                    The fractional exhaled concentration of nitric oxide (FE\n                    <jats:sub>NO<\/jats:sub>\n                    ) has been shown to be reduced in idiopathic pulmonary arterial hypertension (PAH) but has not been adequately studied in PAH associated with systemic sclerosis (SSc). We measured FE\n                    <jats:sub>NO<\/jats:sub>\n                    at an expiratory flow rate of 50\u00a0mL\/s in 21 treatment\u2010naive patients with SSc\u2010associated PAH (SSc\u2010PAH), 94 subjects with SSc without pulmonary involvement, and 84 healthy volunteers. Measurements of FE\n                    <jats:sub>NO<\/jats:sub>\n                    at additional flow rates of 100, 150, and 250\u00a0mL\/s were obtained to derive the flow\u2010independent nitric oxide exchange parameters of maximal airway flux (J\u2032aw\n                    <jats:sub>NO<\/jats:sub>\n                    ) and steady\u2010state alveolar concentration (CA\n                    <jats:sub>NO<\/jats:sub>\n                    ). FE\n                    <jats:sub>NO<\/jats:sub>\n                    at 50\u00a0mL\/s was similar (\n                    <jats:italic>P<\/jats:italic>\n                    = 0.22) in the SSc\u2010PAH group (19 \u00b1 12 parts per billion [ppb]) compared with the SSc group (17 \u00b1 12 ppb) and healthy control group (21 \u00b1 11 ppb). No change was observed after 4\u00a0months of targeted PAH therapy in 14 SSc\u2010PAH group patients (\n                    <jats:italic>P<\/jats:italic>\n                    = 0.9). J\u2032aw\n                    <jats:sub>NO<\/jats:sub>\n                    was modestly reduced in SSc group subjects without lung disease (1.2 \u00b1 0.5\u00a0nl\/s) compared with healthy controls (1.64 \u00b1 0.9;\n                    <jats:italic>P<\/jats:italic>\n                    &lt; 0.05) but was similar to that in the SSc\u2010PAH group. CA\n                    <jats:sub>NO<\/jats:sub>\n                    was elevated in individuals with SSc\u2010PAH (4.8 \u00b1 2.6 ppb) compared with controls with SSc (3.3 \u00b1 1.4 ppb) and healthy subjects (2.6 \u00b1 1.5 ppb;\n                    <jats:italic>P<\/jats:italic>\n                    &lt; 0.001 for both). However, after adjustment for the diffusing capacity of CO, there was no significant difference in CA\n                    <jats:sub>NO<\/jats:sub>\n                    between individuals with SSc\u2010PAH and controls with SSc. We conclude that FE\n                    <jats:sub>NO<\/jats:sub>\n                    is not useful for the diagnosis of PAH in SSc. Increased alveolar nitric oxide in SSc\u2010PAH likely represents impaired diffusion into pulmonary capillary blood.\n                  <\/jats:p>","DOI":"10.1086\/688768","type":"journal-article","created":{"date-parts":[[2016,8,9]],"date-time":"2016-08-09T11:07:26Z","timestamp":1470740846000},"page":"545-550","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":9,"title":["Exhaled Nitric Oxide in Pulmonary Arterial Hypertension Associated with Systemic Sclerosis"],"prefix":"10.1002","volume":"6","author":[{"given":"Zeling","family":"Cao","sequence":"first","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine Johns Hopkins University School of Medicine Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen C.","family":"Mathai","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine Johns Hopkins University School of 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