{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,19]],"date-time":"2026-08-19T18:01:46Z","timestamp":1787162506681,"version":"build-2736575974"},"reference-count":12,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,2,16]],"date-time":"2026-02-16T00:00:00Z","timestamp":1771200000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100002855","name":"Ministry of Science and Technology of the People's Republic of China","doi-asserted-by":"publisher","award":["2023YFC2507204"],"award-info":[{"award-number":["2023YFC2507204"]}],"id":[{"id":"10.13039\/501100002855","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:p>Pulmonary light chain deposition disease (PLCDD) is a rare disorder characterized by the deposition of immunoglobulin light chains in the lungs, often associated with lymphoplasmacytic proliferative and autoimmune disease such as primary Sj\u00f6gren disease (pSjD). Due to its rarity, there is currently no established definitive management of PLCDD. In this case report, we present the clinical presentation of a 42-year-old female with a history of pSjD who experienced recurrent hemoptysis over a period of 3 years. Radiological and pathological assessments confirmed pulmonary involvement of light chain deposition disease. Despite initial failure of glucocorticoids and immunosuppressive agents in targeting pSjD, subsequent combination therapy with bortezomib and dexamethasone (BD) resulting in significant clinical and radiological improvement. The successful use of this combination treatment in PLCDD represents a significant breakthrough, highlighting the potential effectiveness of targeted therapies for PLCDD secondary to autoimmune disease.<\/jats:p>","DOI":"10.3389\/fimmu.2026.1747455","type":"journal-article","created":{"date-parts":[[2026,2,16]],"date-time":"2026-02-16T06:36:53Z","timestamp":1771223813000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":1,"title":["Pulmonary light chain deposition disease secondary to Sj\u00f6gren disease: a case report"],"prefix":"10.3389","volume":"17","author":[{"given":"Xiaotong","family":"Xu","sequence":"first","affiliation":[{"name":"Department of Respiratory and Critical Care Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lingjian","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Respiratory and Critical Care Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chunsheng","family":"Zhou","sequence":"additional","affiliation":[{"name":"4 + 4 Medical Doctor Program, Chinese Academy of Medical Sciences & Peking Union Medical College","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lu","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ting","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Respiratory and Critical Care Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Min","family":"Peng","sequence":"additional","affiliation":[{"name":"Department of Respiratory and Critical Care Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rui\u2019e","family":"Feng","sequence":"additional","affiliation":[{"name":"Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juhong","family":"Shi","sequence":"additional","affiliation":[{"name":"Department of Respiratory and Critical Care Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2026,2,16]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmed.2020.105896","article-title":"Pulmonary nodular and cystic light chain deposition disease: A retrospective review of 10 cases","volume":"164","author":"Baqir","year":"2020","journal-title":"Respir Med"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00132007","article-title":"Pathomechanisms of cyst formation in pulmonary light chain deposition disease","volume":"32","author":"Colombat","year":"2008","journal-title":"Eur Respir J"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200510-1620CR","article-title":"Pulmonary cystic disorder related to light chain deposition disease","volume":"173","author":"Colombat","year":"2006","journal-title":"Am J Respir Crit Care Med"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1136\/ard.61.6.554","article-title":"Classification criteria for sj\u00f6gren\u2019s syndrome: A revised version of the european criteria proposed by the american-european consensus group","volume":"61","author":"Vitali","year":"2002","journal-title":"Ann Rheum Dis"},{"key":"B5","doi-asserted-by":"publisher","first-page":"588","DOI":"10.21037\/atm-20-2708","article-title":"Pulmonary light chain deposition disease: A case series and literature review","volume":"8","author":"Wei","year":"2020","journal-title":"Ann Transl Med"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1097\/00000478-198805000-00009","article-title":"Systemic light chain deposition disease presenting as multiple pulmonary nodules. A case report and review of the literature","volume":"12","author":"Kijner","year":"1988","journal-title":"Am J Surg Pathol"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.26569","article-title":"Immunoglobulin light chain amyloidosis: 2022 update on diagnosis, prognosis, and treatment","volume":"97","author":"Gertz","year":"2022","journal-title":"Am J Hematol"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.14740\/jh1038","article-title":"Treatment of light chain deposition disease: A systematic review","volume":"11","author":"Masood","year":"2022","journal-title":"J Hematol"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0609.2012.01821.x","article-title":"Bortezomib\/dexamethasone followed by autologous stem cell transplantation as front line treatment for light-chain deposition disease","volume":"89","author":"Tovar","year":"2012","journal-title":"Eur J Haematol"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.1016\/j.chest.2021.02.041","article-title":"Autologous stem cell transplantation in the treatment of pulmonary light chain deposition disease","volume":"160","author":"Rendo","year":"2021","journal-title":"Chest"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.1517\/13543780903002074","article-title":"Clinical development of novel proteasome inhibitors for cancer treatment","volume":"18","author":"Yang","year":"2009","journal-title":"Expert Opin Investig Drugs"},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2018.00835","article-title":"Targeting B cells and plasma cells in autoimmune diseases","volume":"9","author":"Hofmann","year":"2018","journal-title":"Front Immunol"}],"container-title":["Frontiers in Immunology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2026.1747455\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,2,16]],"date-time":"2026-02-16T06:36:54Z","timestamp":1771223814000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2026.1747455\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,2,16]]},"references-count":12,"alternative-id":["10.3389\/fimmu.2026.1747455"],"URL":"https:\/\/doi.org\/10.3389\/fimmu.2026.1747455","relation":{},"ISSN":["1664-3224"],"issn-type":[{"value":"1664-3224","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,2,16]]},"article-number":"1747455"}}