{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,21]],"date-time":"2026-03-21T20:15:57Z","timestamp":1774124157439,"version":"3.50.1"},"reference-count":37,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2005,11,29]],"date-time":"2005-11-29T00:00:00Z","timestamp":1133222400000},"content-version":"vor","delay-in-days":7182,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Arthritis &amp; Rheumatism"],"published-print":{"date-parts":[[1986,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Pulmonary hypertension (PHT) occurred in 59 (9%) of 673 systemic sclerosis patients seen between 1963 and 1983. In 30 patients, all with the CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias), the pulmonary hypertension was isolated, i.e., independent of other pulmonary or cardiac conditions. In 20 patients, isolated PHT was demonstrated by cardiac catheterization. All had normal or only mildly decreased lung volumes, and mild or no pulmonary interstitial fibrosis on chest roentgenogram. In comparison with 287 CREST syndrome patients without PHT, these 20 patients had markedly reduced diffusing capacity for carbon monoxide (DLco) (mean 39% of predicted normal). In 6 patients, the low DLco antedated clinical evidence of PHT by 1\u20136 years. At autopsy there was marked intimal fibrosis with hyalinization and smooth muscle hypertrophy in the small\u2010 and medium\u2010sized arteries, without significant parenchymal fibrosis or inflammation. Patients with isolated PHT did not respond favorably to vasodilators and had a very poor prognosis, with a 2\u2010year cumulative survival rate of 40%. A DLco &lt; 45% of predicted in the absence of pulmonary interstitial fibrosis may be an important predictor of the subsequent development of isolated PHT.<\/jats:p>","DOI":"10.1002\/art.1780290409","type":"journal-article","created":{"date-parts":[[2007,2,14]],"date-time":"2007-02-14T21:11:32Z","timestamp":1171487492000},"page":"515-524","source":"Crossref","is-referenced-by-count":283,"title":["Pulmonary hypertension in the crest syndrome variant of systemic sclerosis"],"prefix":"10.1002","volume":"29","author":[{"given":"Angela M.","family":"Stupi","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Virginia D.","family":"Steen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gregory R.","family":"Owens","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"E. Leon","family":"Barnes","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gerald P.","family":"Rodnan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"suffix":"Jr.","given":"Thomas A.","family":"Medsger","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2005,11,29]]},"reference":[{"key":"e_1_2_1_2_2","first-page":"994","volume-title":"Systemic sclerosis (scleroderma), eosinophilic fasciitis, and calcinosis, Arthritis and Allied Conditions","author":"Medsger TA","year":"1985"},{"key":"e_1_2_1_3_2","doi-asserted-by":"publisher","DOI":"10.1002\/art.1780270202"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-86-4-394"},{"key":"e_1_2_1_5_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0025-6196(12)62055-2"},{"key":"e_1_2_1_6_2","first-page":"752","article-title":"Clinical comparison of two variants of progressive systemic sclerosis: diffuse scleroderma and CREST syndrome (abstract)","volume":"23","author":"Steen VD","year":"1980","journal-title":"Arthritis Rheum"},{"key":"e_1_2_1_7_2","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-97-5-652"},{"key":"e_1_2_1_8_2","doi-asserted-by":"publisher","DOI":"10.1378\/chest.84.5.546"},{"key":"e_1_2_1_9_2","first-page":"55","article-title":"Recommended standardized procedures for pulmonary function testing","volume":"118","author":"American Thoracic Society","year":"1978","journal-title":"Am Rev Respir Dis (suppl)"},{"key":"e_1_2_1_10_2","doi-asserted-by":"publisher","DOI":"10.1002\/art.1780230602"},{"key":"e_1_2_1_11_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(19)86738-8"},{"key":"e_1_2_1_12_2","doi-asserted-by":"publisher","DOI":"10.1093\/clinchem\/26.3.396"},{"key":"e_1_2_1_13_2","doi-asserted-by":"publisher","DOI":"10.1109\/MC.1979.1658533"},{"key":"e_1_2_1_14_2","doi-asserted-by":"publisher","DOI":"10.1002\/art.1780230510"},{"key":"e_1_2_1_15_2","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-74-5-714"},{"key":"e_1_2_1_16_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF01994406"},{"key":"e_1_2_1_17_2","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-60-4-611"},{"key":"e_1_2_1_18_2","doi-asserted-by":"publisher","DOI":"10.1136\/ard.30.4.390"},{"key":"e_1_2_1_19_2","doi-asserted-by":"publisher","DOI":"10.2214\/ajr.124.3.461"},{"key":"e_1_2_1_20_2","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(79)90478-9"},{"key":"e_1_2_1_21_2","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(83)91169-5"},{"key":"e_1_2_1_22_2","doi-asserted-by":"publisher","DOI":"10.1378\/chest.28.6.665"},{"key":"e_1_2_1_23_2","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(78)90455-2"},{"key":"e_1_2_1_24_2","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(69)90044-8"},{"key":"e_1_2_1_25_2","first-page":"164A","article-title":"Cardiopulmonary function in the CREST syndrome (abstract)","volume":"29","author":"Germain BF","year":"1981","journal-title":"Clin Res"},{"key":"e_1_2_1_26_2","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-89-6-881"},{"key":"e_1_2_1_27_2","doi-asserted-by":"publisher","DOI":"10.1097\/00005792-197401000-00001"},{"key":"e_1_2_1_28_2","doi-asserted-by":"publisher","DOI":"10.1002\/art.1780241111"},{"key":"e_1_2_1_29_2","doi-asserted-by":"publisher","DOI":"10.1378\/chest.69.2.304"},{"key":"e_1_2_1_30_2","doi-asserted-by":"publisher","DOI":"10.1097\/00005792-198311000-00001"},{"key":"e_1_2_1_31_2","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(84)90183-9"},{"key":"e_1_2_1_32_2","first-page":"S19","article-title":"Clinical associations of anti\u2010Scl 70 antibody in patients with systemic sclerosis (abstract)","volume":"27","author":"Powell DL","year":"1984","journal-title":"Arthritis Rheum (suppl)"},{"key":"e_1_2_1_33_2","first-page":"83","article-title":"Unexplained pulmonary hypertension","volume":"26","author":"Wade G","year":"1957","journal-title":"Q J Med"},{"key":"e_1_2_1_34_2","doi-asserted-by":"publisher","DOI":"10.1001\/archinte.1960.00270140055006"},{"key":"e_1_2_1_35_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.22.6.1055"},{"key":"e_1_2_1_36_2","first-page":"607","article-title":"Anticentromere antibody and pulmonary hypertension","volume":"9","author":"Seibold JR","year":"1982","journal-title":"J Rheumatol"},{"key":"e_1_2_1_37_2","doi-asserted-by":"publisher","DOI":"10.1097\/00005792-198403000-00003"},{"key":"e_1_2_1_38_2","first-page":"499","article-title":"Severe pulmonary involvement in mixed connective tissue disease","volume":"124","author":"Wiener\u2010Kronish JP","year":"1981","journal-title":"Am Rev Respir Dis"}],"container-title":["Arthritis &amp; 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