{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T20:04:05Z","timestamp":1790193845682,"version":"4.1.0"},"reference-count":17,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2005,12,9]],"date-time":"2005-12-09T00:00:00Z","timestamp":1134086400000},"content-version":"vor","delay-in-days":5425,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Arthritis &amp; Rheumatism"],"published-print":{"date-parts":[[1991,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>We describe a patient who developed fatal pulmonary artery hypertension secondary to diffuse venulitis. This otherwise healthy young woman first presented with generalized venulopathy, with chemosis, facial swelling, pleural effusions, and pericardial tamponade. The symptoms partially responded to steroid therapy, but over a 2\u2010year course, a rapidly progressive and fatal venoocclusive disease developed. No other primary condition was diagnosed, and at autopsy, the patient had striking venulitis throughout, including the pulmonary bed. We believe that this is a unique case of pulmonary hypertension resulting from a generalized venulopathy.<\/jats:p>","DOI":"10.1002\/art.1780340217","type":"journal-article","created":{"date-parts":[[2007,2,14]],"date-time":"2007-02-14T23:43:48Z","timestamp":1171496628000},"page":"228-233","source":"Crossref","is-referenced-by-count":15,"title":["Fatal pulmonary venoocclusive disease secondary to a generalized venulopathy: A new syndrome presenting with facial swelling and pericardial tamponade"],"prefix":"10.1002","volume":"34","author":[{"given":"Matthew H.","family":"Liang","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steve","family":"Stern","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul R.","family":"Fortin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diane C.","family":"Louie","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James D.","family":"Marsh","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"suffix":"Jr.","given":"Gilbert H.","family":"Mudge","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"George","family":"Murphy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2005,12,9]]},"reference":[{"key":"e_1_2_1_2_2","volume-title":"Diagnosis and management of primary pulmonary hypertension, Pulmonary Hypertension","author":"Weir EK","year":"1984"},{"key":"e_1_2_1_3_2","unstructured":"Hatano S Strasser T editors:Primary pulmonary hypertension Meeting of the World Health Organization. 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