{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,25]],"date-time":"2026-08-25T12:27:12Z","timestamp":1787660832470,"version":"build-2736575974"},"reference-count":27,"publisher":"SAGE Publications","issue":"10","license":[{"start":{"date-parts":[[2026,6,16]],"date-time":"2026-06-16T00:00:00Z","timestamp":1781568000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"funder":[{"DOI":"10.13039\/501100002322","name":"Coordena\u00e7\u00e3o de Aperfei\u00e7oamento de Pessoal de N\u00edvel Superior - Brasil","doi-asserted-by":"publisher","award":["001"],"award-info":[{"award-number":["001"]}],"id":[{"id":"10.13039\/501100002322","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Lupus"],"published-print":{"date-parts":[[2026,9]]},"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Shrinking lung syndrome (SLS) is a rare manifestation of systemic lupus erythematosus (SLE) lacking standardized management. This systematic review summarizes the current evidence on therapeutic interventions and clinical outcomes.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Following PRISMA guidelines, we searched six databases (e.g., PubMed, Scopus, and Web of Science) for studies on adult SLS patients published up to January 2026. Methodological quality was assessed using JBI tools.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Forty studies (111 patients) were included (30 case reports and 10 case series); 87.5% presented a low risk of bias. There was a marked female predominance (91%), with a mean age of 33.5 \u00b1 9.8\u00a0years. All patients showed a restrictive pattern (forced vital capacity: 47\u201352%). Glucocorticoids were the mainstay of therapy (95\u201396%). Rituximab emerged as an effective primary biologic for refractory cases. Non-pharmacological interventions, such as inspiratory muscle training and non-invasive ventilation, improved diaphragmatic strength. Although symptomatic resolution occurred in 75\u201395% of patients, complete functional recovery (normalization of pulmonary function tests) was achieved in only 20\u201323% of patients. Mortality directly attributed to SLS was low (2\u20135.5%).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Early glucocorticoid therapy remains essential, but complete functional recovery is rare, with 80% of patients maintaining chronic restriction. For refractory cases, multidisciplinary care integrating rituximab and respiratory rehabilitation should be considered to optimize functional outcomes.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1177\/09612033261461973","type":"journal-article","created":{"date-parts":[[2026,6,16]],"date-time":"2026-06-16T15:49:23Z","timestamp":1781624963000},"page":"1040-1047","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":0,"title":["Treatment strategies and clinical outcomes in shrinking lung syndrome in systemic lupus erythematosus: An updated systematic review"],"prefix":"10.1177","volume":"35","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7762-3439","authenticated-orcid":false,"given":"J\u00falia Ribeiro","family":"Lemos","sequence":"first","affiliation":[{"name":"Augusto Motta University Center (UNISUAM)"},{"name":"Rio de Janeiro State University (UERJ)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8598-4878","authenticated-orcid":false,"given":"Agnaldo Jos\u00e9","family":"Lopes","sequence":"additional","affiliation":[{"name":"Augusto Motta University Center (UNISUAM)"},{"name":"Rio de Janeiro State University (UERJ)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6097-1456","authenticated-orcid":false,"given":"Patr\u00edcia dos Santos","family":"Vig\u00e1rio","sequence":"additional","affiliation":[{"name":"Augusto Motta University Center (UNISUAM)"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2026,6,16]]},"reference":[{"issue":"2","key":"e_1_3_5_2_2","article-title":"Tofacitinib treatment in refractory systemic lupus erythematosus with shrinking lung syndrome: a case-based review","volume":"54","author":"He et al. Y","year":"2024","unstructured":"He et al. Y Tofacitinib treatment in refractory systemic lupus erythematosus with shrinking lung syndrome: a case-based review. Biomed J Sci Tech Res 2024; 54(2). [cited 2026 Jan 1] Available from: https:\/\/biomedres.us\/fulltexts\/BJSTR.MS.ID.008533.php","journal-title":"Biomed J Sci Tech Res"},{"key":"e_1_3_5_3_2","doi-asserted-by":"publisher","DOI":"10.1002\/rcr2.70255"},{"key":"e_1_3_5_4_2","doi-asserted-by":"publisher","DOI":"10.1177\/0961203314536248"},{"issue":"7","key":"e_1_3_5_5_2","first-page":"e63990","article-title":"Shrinking lung syndrome: a rare pulmonary complication of systemic lupus erythematosus","volume":"16","author":"Shah K","year":"2024","unstructured":"Shah K, Kondakindi H, Enabi J, et al. Shrinking lung syndrome: a rare pulmonary complication of systemic lupus erythematosus. Cureus 2024; 16(7): e63990. https:\/\/doi.org\/10.7759\/cureus.63990, [cited 2026 Jan 1]; Available from: https:\/\/www.cureus.com\/articles\/262954-shrinking-lung-syndrome-a-rare-pulmonary-complication-of-systemic-lupus-erythematosus","journal-title":"Cureus"},{"key":"e_1_3_5_6_2","unstructured":"Perrone I Buffarini L Szwarstein P et al. S\u00edndrome del pulm\u00f3n encogido asociado a lupus eritematoso sist\u00e9mico [Shrinking lung syndrome associated with systemic lupus erythematosus]. Medicina (B Aires). 2024;84(4):635-640. Spanish. 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[cited 2025 Dec 28]; Available from: https:\/\/www.cureus.com\/articles\/137965-shrinking-lung-syndrome-in-a-systemic-lupus-erythematous-patient-improved-by-rituximab-a-case-report-with-literature-review","journal-title":"Cureus"},{"issue":"11","key":"e_1_3_5_13_2","first-page":"e97612","article-title":"Improved lung function with cyclophosphamide and rituximab in a case of shrinking lung syndrome","volume":"17","author":"Chaudhry S","year":"2025","unstructured":"Chaudhry S, Bokhary H, Quinn M. Improved lung function with cyclophosphamide and rituximab in a case of shrinking lung syndrome. 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[cited 2026 Jan 1]; Available from: https:\/\/www.cureus.com\/articles\/95040-shrinking-lung-syndrome-in-a-young-female-a-rare-pulmonary-manifestation-of-systemic-lupus-erythematosus","journal-title":"Cureus"},{"issue":"7","key":"e_1_3_5_15_2","first-page":"e27311","article-title":"Shrinking lung syndrome: a case report","volume":"14","author":"Datoo A","year":"2022","unstructured":"Datoo A, Abdelghani L. Shrinking lung syndrome: a case report. Cureus 2022; 14(7): e27311. 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[cited 2026 Jan 1]; Available from: https:\/\/www.cureus.com\/articles\/32163-shrinking-lung-syndrome-a-rare-manifestation-of-systemic-lupus-erythematosus","journal-title":"Cureus"},{"key":"e_1_3_5_20_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.medcle.2023.10.024"},{"key":"e_1_3_5_21_2","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.n71"},{"key":"e_1_3_5_22_2","doi-asserted-by":"publisher","DOI":"10.7326\/ACPJC-1995-123-3-A12"},{"key":"e_1_3_5_23_2","volume-title":"JBI Manual for Evidence Synthesis","author":"Chapter 7","year":"2020","unstructured":"Chapter 7. Systematic reviews of etiology and risk. In: JBI Manual for Evidence Synthesis. JBI, 2020. [cited 2026 Jan 12]. Available from: https:\/\/jbi-global-wiki.refined.site\/space\/MANUAL\/355863557\/Previous+versions?attachment=\/download\/attachments\/355863557\/JBI_Reviewers_Manual_2020June.pdf&type=application\/pdf&filename=JBI_Reviewers_Manual_2020June.pdf#page=217"},{"key":"e_1_3_5_24_2","doi-asserted-by":"publisher","DOI":"10.1097\/RHU.0b013e3181d0757f"},{"issue":"9","key":"e_1_3_5_25_2","first-page":"617","article-title":"Shrinking lung syndrome in systemic lupus erythematosus successfully treated with rituximab. QJM","volume":"109","author":"Goswami RP","year":"2016","unstructured":"Goswami RP, Mondal S, Lahiri D, et al. Shrinking lung syndrome in systemic lupus erythematosus successfully treated with rituximab. QJM. 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