{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,21]],"date-time":"2026-10-21T03:29:48Z","timestamp":1792553388167,"version":"4.5.0"},"reference-count":38,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2023,10,24]],"date-time":"2023-10-24T00:00:00Z","timestamp":1698105600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100003711","name":"MOST","doi-asserted-by":"publisher","award":["2021YFC2501301\u20105"],"award-info":[{"award-number":["2021YFC2501301\u20105"]}],"id":[{"id":"10.13039\/501100003711","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003711","name":"MOST","doi-asserted-by":"publisher","award":["2017YFC0907601\u20103"],"award-info":[{"award-number":["2017YFC0907601\u20103"]}],"id":[{"id":"10.13039\/501100003711","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100009592","name":"Beijing Municipal Science and Technology Commission, Adminitrative Commission of Zhongguancun Science Park","doi-asserted-by":"publisher","award":["Z201100005520022, 23, and 25\u201027"],"award-info":[{"award-number":["Z201100005520022, 23, and 25\u201027"]}],"id":[{"id":"10.13039\/501100009592","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Arthritis &amp; Rheumatology"],"published-print":{"date-parts":[[2023,12]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>Pulmonary arterial hypertension (PAH) is a severe complication of systemic lupus erythematosus (SLE). However, the genetic signatures of SLE\u2010associated PAH have not been well studied. We aimed to identify genetic variants implicated in SLE\u2010associated PAH susceptibility within the major histocompatibility complex (MHC) region and assess the contribution to clinical outcomes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A total of 172 patients with SLE\u2010associated PAH confirmed by right heart catheterization, 1,303 patients with SLE without PAH, and 9,906 healthy controls were included. Deep sequencing of the MHC region was performed to identify alleles, single\u2010nucleotide polymorphisms, and amino acids. We compared patients with SLE\u2010associated PAH with patients with SLE without PAH and healthy controls. Clinical association study was conducted to explore the contribution to phenotypes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A total of 19,881 genetic variants were identified within the MHC region. <jats:italic>HLA\u2010DQA1<\/jats:italic>*03:02 was identified as a novel genetic variant associated with SLE\u2010associated PAH in the discovery cohort (<jats:italic>P<\/jats:italic> =\u20095.68 \u00d7\u200910<jats:sup>\u221212<\/jats:sup>) and authenticated in an independent replication cohort (<jats:italic>P<\/jats:italic> =\u20091.30 \u00d7\u200910<jats:sup>\u22129<\/jats:sup>). The strongest associated amino acid position was mapped to HLA\u2010DQ\u03b11 in the region affecting MHC\/peptide\u2010CD4<jats:sup>+<\/jats:sup> T cell receptor affinity and antigen binding. Clinical association study demonstrated that patients with SLE\u2010associated PAH with <jats:italic>HLA\u2010DQA1<\/jats:italic>*03:02 had significantly lower rates of target role achievement (<jats:italic>P<\/jats:italic> =\u20090.005) and survival (<jats:italic>P<\/jats:italic> =\u20090.04).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>This study, based on the largest cohort of SLE\u2010associated PAH, is the first to investigate how MHC region genetic variants contribute to SLE\u2010associated PAH susceptibility. <jats:italic>HLA\u2010DQA1<\/jats:italic>*03:02 is a novel genetic risk factor and a prognostic factor in SLE\u2010associated PAH. Patients with SLE with this allele require regular monitoring and careful follow\u2010up for early diagnosis and interventions for potential PAH.<\/jats:p><\/jats:sec>","DOI":"10.1002\/art.42641","type":"journal-article","created":{"date-parts":[[2023,6,29]],"date-time":"2023-06-29T07:40:59Z","timestamp":1688024459000},"page":"2207-2215","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Association Study Identified <scp><i>HLA\u2010DQA1<\/i><\/scp> as a Novel Genetic Risk of Systemic Lupus <scp>Erythematosus\u2010Associated<\/scp> Pulmonary Arterial Hypertension"],"prefix":"10.1002","volume":"75","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6909-539X","authenticated-orcid":false,"given":"Junyan","family":"Qian","sequence":"first","affiliation":[{"name":"Department of Rheumatology and Clinical Immunology Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science and Technology, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education and State Key Laboratory of Complex Severe and Rare Diseases, Ministry of Science and Technology  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ying","family":"Chen","sequence":"additional","affiliation":[{"name":"BGI\u2010Shenzhen  Shenzhen China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xinzhuang","family":"Yang","sequence":"additional","affiliation":[{"name":"Medical Research Center, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qian","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Clinical Immunology Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science and Technology, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education and State Key Laboratory of Complex Severe and Rare Diseases, Ministry of Science and Technology  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jiuliang","family":"Zhao","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Clinical Immunology Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science and Technology, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education and State Key Laboratory of Complex Severe and Rare Diseases, Ministry of Science and 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Ministry of Science and Technology, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education and State Key Laboratory of Complex Severe and Rare Diseases, Ministry of Science and Technology  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shengjie","family":"Li","sequence":"additional","affiliation":[{"name":"Medical Research Center, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4550-7070","authenticated-orcid":false,"given":"Yongtai","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Peking Union Medical College Hospital Peking Union Medical College &amp; Chinese Academy of Medical Sciences  Beijing 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