{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T02:00:34Z","timestamp":1790301634735,"version":"4.1.0"},"reference-count":9,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2026,9,3]],"date-time":"2026-09-03T00:00:00Z","timestamp":1788393600000},"content-version":"vor","delay-in-days":2,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T00:00:00Z","timestamp":1788220800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Respirology Case Reports"],"published-print":{"date-parts":[[2026,9]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    <jats:styled-content style=\"fixed-case\">COPA<\/jats:styled-content>\n                    syndrome is a rare autosomal dominant autoimmune disorder caused by\n                    <jats:styled-content style=\"fixed-case\">COPA<\/jats:styled-content>\n                    gene mutations, leading to immune dysregulation and multisystem involvement. We report a 45\u2010year\u2010old man with recurrent respiratory failure, haemoptysis, renal dysfunction and arthritis. Symptoms began at Age\u200927 with diffuse alveolar damage requiring mechanical ventilation. Imaging showed progressive interstitial lung disease and biopsy revealed follicular bronchiolitis. Immunosuppression provided temporary stabilisation. In 2024, next\u2010generation sequencing confirmed a likely pathogenic\n                    <jats:styled-content style=\"fixed-case\">COPA<\/jats:styled-content>\n                    variant. The patient later discontinued therapy and remained untreated for 1\u2009year, returning with worsening renal dysfunction and significant proteinuria, while pulmonary findings remained stable. Cyclophosphamide was initiated for suspected renal relapse. During treatment, he developed acute diverticulitis complicated by sepsis, progressing to septic shock and multiorgan failure despite surgery and antibiotics, resulting in death. Early recognition and multidisciplinary management are essential to improve outcomes of the disease.\n                  <\/jats:p>","DOI":"10.1002\/rcr2.70742","type":"journal-article","created":{"date-parts":[[2026,9,4]],"date-time":"2026-09-04T03:09:51Z","timestamp":1788491391000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["A Rare Case of\n                    <scp>COPA<\/scp>\n                    Syndrome: Multisystem Relapse and Fatal Septic Complication"],"prefix":"10.1002","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5973-2877","authenticated-orcid":false,"given":"Flavia","family":"Castro Velasco Fernandes","sequence":"first","affiliation":[{"name":"Faculdade de Medicina, Universidade Federal de Goi\u00e1s  Goi\u00e2nia Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8571-5667","authenticated-orcid":false,"given":"Pedro Paulo Teixeira e Silva","family":"Torres","sequence":"additional","affiliation":[{"name":"Hospital Israelita Albert Einstein  Goi\u00e2nia Goi\u00e1s Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4143-9007","authenticated-orcid":false,"given":"Elis\u00e2ngela","family":"de Paula Silveira\u2010Lacerda","sequence":"additional","affiliation":[{"name":"Instituto de Ci\u00eancias Biol\u00f3gicas, Universidade Federal de Goi\u00e1s  Goi\u00e2nia Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matheus","family":"Rabahi","sequence":"additional","affiliation":[{"name":"Faculdade de Medicina, Universidade Federal de Goi\u00e1s  Goi\u00e2nia Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0003-6923-4008","authenticated-orcid":false,"given":"Lucas","family":"Velasco Magalhaes Fernandes","sequence":"additional","affiliation":[{"name":"Lake Erie College of Osteopathic Medicine  Bradenton Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0002-6089-0482","authenticated-orcid":false,"given":"La\u00eds Rocha","family":"Lopes","sequence":"additional","affiliation":[{"name":"Faculdade de Medicina, Universidade Federal de Goi\u00e1s  Goi\u00e2nia Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marise Amaral Rebou\u00e7as","family":"Moreira","sequence":"additional","affiliation":[{"name":"Department of Pathology Universidade Federal de Goi\u00e1s  Goi\u00e2nia Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4050-5906","authenticated-orcid":false,"given":"Marcelo Fouad","family":"Rabahi","sequence":"additional","affiliation":[{"name":"Faculdade de Medicina, Universidade Federal de Goi\u00e1s  Goi\u00e2nia Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,9,3]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.2000028"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jbspin.2020.09.002"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.rdc.2023.06.005"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmedgenet\u20102018\u2010105560"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1155\/2020\/3624795"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/cti2.1243"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ard.2025.09.013"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12887\u2010022\u201003716\u20101"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1136\/thoraxjnl\u20102019\u2010213892"}],"container-title":["Respirology Case 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