[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"doc-seo-455118-105":3,"detail-sidebar-cat-0-en-105":81,"doc-detail-455118-en":131},{"code":4,"msg":5,"data":6},0,"ok",{"site_id":7,"language":8,"slug":9,"title":10,"keywords":11,"description":12,"schema_data":13,"social_meta":74,"head_meta":76,"extra_data":78,"updated_unix":80},105,"en","three-year-diagnostic-delay-in-shrinking-lung-syndrome-rituximab-theophylline-combination-as-rescue-therapy","Three-Year Diagnostic Delay in Shrinking Lung Syndrome - Rituximab-Theophylline Combination as Rescue Therapy","","Shrinking lung syndrome (SLS) is described as an uncommon complication of systemic lupus erythematosus (SLE) that can be missed for years. A 41-year-old woman with a 10-year SLE history experienced dyspnea, chest pain, and dry cough with poor medication adherence and repeated hospitalizations over 3 years. Imaging showed bilateral basal atelectasis and an elevated right hemidiaphragm, while pulmonary function tests demonstrated a restrictive pattern after other causes were excluded. Treatment with rituximab, prednisolone, and theophylline improved pulmonary function and symptoms. Clinicians are advised to consider SLS in SLE patients with characteristic imaging to reduce morbidity associated with delayed diagnosis.",{"@graph":14,"@context":73},[15,34,56],{"@type":16,"itemListElement":17},"BreadcrumbList",[18,23,27,31],{"item":19,"name":20,"@type":21,"position":22},"https://docshare.wps.com","Home","ListItem",1,{"item":24,"name":25,"@type":21,"position":26},"https://docshare.wps.com/document/","Document",2,{"item":28,"name":29,"@type":21,"position":30},"https://docshare.wps.com/document/healthcare/","Healthcare",3,{"item":32,"name":10,"@type":21,"position":33},"https://docshare.wps.com/document/three-year-diagnostic-delay-in-shrinking-lung-syndrome-rituximab-theophylline-combination-as-rescue-therapy/455118/",4,{"url":32,"name":10,"@type":35,"image":36,"author":41,"headline":10,"publisher":44,"fileFormat":47,"inLanguage":8,"description":12,"dateModified":48,"datePublished":49,"encodingFormat":47,"isAccessibleForFree":50,"interactionStatistic":51},"DigitalDocument",{"url":37,"@type":38,"width":39,"height":40},"https://docshare.wps.com/thumbnails/three-year-diagnostic-delay-in-shrinking-lung-syndrome-rituximab-theophylline-combination-as-rescue-therapy/455118.png","ImageObject",300,407,{"name":42,"@type":43},"Guten tag","Person",{"url":19,"name":45,"@type":46},"DocShare","Organization","application/pdf","2026-10-30","2026-09-30",true,{"@type":52,"interactionType":53,"userInteractionCount":55},"InteractionCounter",{"@type":54},"ViewAction",11,{"@type":57,"mainEntity":58},"FAQPage",[59,65,69],{"name":60,"@type":61,"acceptedAnswer":62},"What clinical features suggested shrinking lung syndrome in this SLE patient?","Question",{"text":63,"@type":64},"The patient had dyspnea, pleuritic chest pain, and dry cough. Imaging showed bilateral basal atelectasis with an elevated right hemidiaphragm, and pulmonary function tests showed a restrictive pattern.","Answer",{"name":66,"@type":61,"acceptedAnswer":67},"How long was the diagnostic delay before SLS was identified?",{"text":68,"@type":64},"Symptoms began in 2020 and led to multiple hospitalizations for 3 years without a definitive diagnosis, indicating a prolonged delay.",{"name":70,"@type":61,"acceptedAnswer":71},"What treatment improved outcomes after diagnosis of SLS?",{"text":72,"@type":64},"Rituximab, prednisolone, and theophylline were used, with improvement in pulmonary function tests and respiratory symptoms at follow-up.","https://schema.org",{"og:url":32,"og:type":75,"og:title":10,"og:site_name":45,"og:description":12},"article",{"robots":77,"canonical":32},"index,follow",{"doc_id":79,"site_id":7},455118,1791003709,{"code":4,"msg":82,"data":83},"success",[84,88,92,96,101,106,110,115,120,123,127],{"id":22,"doc_module":4,"doc_module_name":25,"category_name":85,"show_sort_weight":86,"slug":87},"Story & Novel",90,"story-novel",{"id":26,"doc_module":4,"doc_module_name":25,"category_name":89,"show_sort_weight":90,"slug":91},"Literature",80,"literature",{"id":33,"doc_module":4,"doc_module_name":25,"category_name":93,"show_sort_weight":94,"slug":95},"Exam",70,"exam",{"id":97,"doc_module":4,"doc_module_name":25,"category_name":98,"show_sort_weight":99,"slug":100},5,"Comic",60,"comic",{"id":102,"doc_module":4,"doc_module_name":25,"category_name":103,"show_sort_weight":104,"slug":105},6,"Technology",50,"technology",{"id":107,"doc_module":4,"doc_module_name":25,"category_name":29,"show_sort_weight":108,"slug":109},7,40,"healthcare",{"id":111,"doc_module":4,"doc_module_name":25,"category_name":112,"show_sort_weight":113,"slug":114},8,"Research & Report",30,"research-report",{"id":116,"doc_module":4,"doc_module_name":25,"category_name":117,"show_sort_weight":118,"slug":119},9,"Religion & Spirituality",20,"religion-spirituality",{"id":118,"doc_module":4,"doc_module_name":25,"category_name":121,"show_sort_weight":118,"slug":122},"World Cup","world-cup",{"id":124,"doc_module":4,"doc_module_name":25,"category_name":125,"show_sort_weight":124,"slug":126},10,"Lifestyle","lifestyle",{"id":128,"doc_module":4,"doc_module_name":25,"category_name":129,"show_sort_weight":97,"slug":130},19,"General","general",{"code":4,"msg":82,"data":132},{"doc_id":79,"user_id":133,"nickname":42,"user_avatar":134,"doc_module":4,"category_id":107,"category_name":29,"doc_title":10,"doc_description":12,"doc_content":135,"file_id":136,"file_url":137,"file_type":138,"file_size":139,"view_count":55,"is_deleted":4,"is_public":22,"is_downloadable":22,"audit_status":22,"page_count":33,"language":140,"language_code":8,"site_id":7,"html_lang":8,"table_of_contents":141,"faqs":142,"seo_title":143,"seo_description":12,"update_tm":144,"read_time":124,"preview_page_count":4},687212321768,"https://ap-avatar.wpscdn.com/avatar/a0010bdbe886d2fe77?x-image-process=image/resize,m_fixed,w_180,h_180&k=1789897067658708522","Respirology Case Reports  \nCASE REPORT  OPEN ACCESS   \nThree-Year Diagnostic Delay in Shrinking Lung Syndrome: Rituximab-Theophylline Combination as Rescue Therapy  \nSomayeh Motamed | Alireza Mirzamohamadi  | Shokufe Sadeghi | Vahid Ardestani Rostami  | Mehrdad Mahalleh  | Majid Alikhani   \nRheumatology Research Center, Tehran University of Medical Sciences, Tehran, Iran Correspondence: Majid Alikhani ([alikhanim@tums.ac.ir](alikhanim@tums.ac.ir))  \nReceived: 15 September 2025 | Revised: 6 December 2025 | Accepted: 16 December 2025  \nAssociate Editor: Kazuyuki Nakagome  \nKeywords: case report | rituximab | shrinking lung syndrome | systemic lupus erythematosus | theophylline  \nABSTRACT  \nShrinking lung syndrome (SLS) is an infrequent complication of systemic lupus erythematosus (SLE) . A 41-year-old woman with a 10-year history of SLE presented with dyspnea, chest pain, and dry cough. She had poor medication compliance and multiple hospitalizations over 3 years for similar symptoms, without a definitive diagnosis. Imaging revealed bilateral basal atelectasis and an elevated right hemidiaphragm, while pulmonary function tests (PFTs) showed a restrictive pattern. Other lung and cardiac conditions were excluded, leading to a diagnosis of SLS. Treatment with rituximab, prednisolone, and theophylline improved PFTs, chest pain, cough, and dyspnea at follow-up. Clinicians should consider SLS in SLE patients with dyspnea and characteristic imaging, as delayed diagnosis may increase morbidity.  \n1 | Introduction  \nShrinking lung syndrome (SLS) is a rare complication of autoimmune diseases, especially systemic lupus erythematosus (SLE), occurring in about 1% of patients after 4–6.5 years [1, 2] . Symptoms include dyspnea, reduced lung volume, elevated diaphragm, coughing, and chest pain [2] . Its possible causes include diaphragmatic weakness, abnormal chest wall expansion, pleural inflammation, and phrenic neuropathy [3] . Due to its rarity and nonspecific symptoms, SLS is often overlooked with significant diagnostic delays (mean 11 ± 20 months, with 48% of cases diagnosed after > 1 year of symptoms) and can be mistaken for conditions like pleuritis or pulmonary embolism [2] . Although there is no evidence-based guideline for treatment, previous reports suggest corticosteroids, inhaled β-2 agonists, theophylline, azathioprine, cyclophosphamide, analgesics, and physiotherapy [2] . Recent studies have shown that rituximab is emerging as a promising option in resistant cases, with case reports indicating success after initial steroid  \ntreatment fails [4] . However, further research is warranted to confirm its effectiveness.  \nHerein, we present a case of SLE with multiple hospitalizations over 3 years due to unexplained respiratory symptoms, ultimately diagnosed with SLS and treated with rituximab, prednisolone, and theophylline.  \n2 | Case Report  \nIn 2023, a 41-year-old Azeri woman with a 10-year history of SLE was referred to our clinic. The diagnosis was established according to the 2019 European League Against Rheumatism/ American College of Rheumatology Classification (EULAR/ ACR) criteria, supported by the following findings: polyarthritis, lupus nephritis (Class III), positive fluorescent antinuclear antibody (FANA) with a speckled pattern (titre = 1/80), low complement levels (C3 and C4), and a positive anti-double-stranded  \nThis is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.  \n© 2026 The Author(s). Respirology Case Reports published by John Wiley & Sons Australia, Ltd on behalf of The Asian Pacific Society of Respirology.  \nRespirology Case Reports, 2026; 14:e70461 1 of 4  \n[https://doi.org/10.1002/rcr2.70461](https://doi.org/10.1002/rcr2.70461)  \ndNA (ds-DNA) antibody. She presented with recurrent dyspnea (Modified Medical Research Council (mMRC) grade 2), pleuritic chest pain, ","cbCaif2jYbYBfifm","https://ap.wps.com/l/cbCaif2jYbYBfifm","pdf",1092753,"English","# Introduction\n## Case Report\n## Results and Findings\n## Discussion and Conclusion","[{\"question\":\"What clinical features suggested shrinking lung syndrome in this SLE patient?\",\"answer\":\"The patient had dyspnea, pleuritic chest pain, and dry cough. Imaging showed bilateral basal atelectasis with an elevated right hemidiaphragm, and pulmonary function tests showed a restrictive pattern.\"},{\"question\":\"How long was the diagnostic delay before SLS was identified?\",\"answer\":\"Symptoms began in 2020 and led to multiple hospitalizations for 3 years without a definitive diagnosis, indicating a prolonged delay.\"},{\"question\":\"What treatment improved outcomes after diagnosis of SLS?\",\"answer\":\"Rituximab, prednisolone, and theophylline were used, with improvement in pulmonary function tests and respiratory symptoms at follow-up.\"}]","Three-Year Diagnostic Delay in Shrinking Lung Syndrome - Rituximab-Theophylline Combination as Rescue Therapy | PDF",1790742158]