Treatment strategies and clinical outcomes in shrinking lung syndrome in systemic lupus erythematosus: An updated systematic review

Abstract

Background

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.

Methods

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.

Results

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 ± 9.8 years. All patients showed a restrictive pattern (forced vital capacity: 47–52%). Glucocorticoids were the mainstay of therapy (95–96%). 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 75–95% of patients, complete functional recovery (normalization of pulmonary function tests) was achieved in only 20–23% of patients. Mortality directly attributed to SLS was low (2–5.5%).

Conclusions

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.

Keywords

systemic lupus erythematosus shrinking lung syndrome respiratory muscle training immunosuppression systematic review

Introduction

Shrinking lung syndrome (SLS) is a rare but debilitating pleuropulmonary manifestation of systemic lupus erythematosus (SLE).^1–3 SLS is characterized by progressive exertional dyspnea, pleuritic chest pain, and a restrictive ventilatory pattern associated with diaphragmatic elevation, in the absence of parenchymal or vascular disease.^4–6 Since its initial description in 1965,⁷ the pathophysiology of SLS has been the subject of academic debate. Proposed hypotheses range from diaphragmatic myopathy and phrenic neuropathy to reflex inhibition of deep inspiration triggered by pleural inflammation.^8–10

Despite its impact on patients’ quality of life, therapeutic management is hindered by the lack of an established gold standard.¹¹ While glucocorticoids are traditionally employed as first-line therapy, evidence for managing refractory cases remains purely empirical and lacks standardized protocols.^5,12–14 Furthermore, complete functional recovery is achieved in only approximately 20% of patients,^8,10,15,16 suggesting a significant gap in current long-term management strategies. As more than half of the available literature on SLS has emerged in the last decade,^4,12,17–19 an updated, rigorous synthesis is required. The aim of this systematic review was to synthesize current evidence on pharmacological and non-pharmacological treatment modalities for SLS, aiming to provide a clearer clinical framework in the absence of formal guidelines.

Methods

Study design and research question

This systematic review was conducted in accordance with the PRISMA 2020²⁰ guidelines. The research question was anchored in the PICO framework²¹: (P) adults with SLE and SLS; (I) pharmacological and non-pharmacological interventions; (C) not applicable; and (O) clinical and pulmonary functional responses.

Search strategy

A systematic search was performed in PubMed, Lilacs, Scopus, Web of Science, PEDro, and SciELO for records published up to January 2026. The search string “shrinking lung*” AND “lupus” was used. Additionally, the reference lists of the included studies were manually screened to identify further relevant records.

Eligibility criteria

Inclusion criteria comprised clinical trials, observational studies, case series, and case reports describing therapeutic interventions and outcomes in adult patients. We excluded pediatric cases, SLS associated with other connective tissue diseases, purely diagnostic studies, narrative reviews, conference abstracts, and studies without full-text access.

Study Selection and data extraction

Two independent reviewers screened the titles and abstracts, followed by full-text assessment. Any disagreements were solved by consensus. Extracted data included demographics, treatment protocols and respiratory functional response.

Quality assessment

Methodological quality was assessed by two independent reviewers using JBI Critical Appraisal Tools for Case Reports and Case Series.²² The risk of bias was categorized by the percentage of “yes” responses as follows: low (≥70%), moderate (50–69%), or high (<50%).

Data synthesis

Due to clinical rarity and methodological heterogeneity, a meta-analysis was not performed. Instead, a qualitative narrative synthesis was conducted to summarize the evidence.

Results

Study Selection and quality assessment

The initial search yielded 114 records. After screening, 40 studies involving 111 patients were included: 30 were case reports, and 10 were case series (Figure 1). Although two studies were prospective and interventional, they were classified as prospective non-randomized interventional studies due to the lack of randomization and control groups. The overall methodological quality was high. According to JBI criteria, 35 studies (87.5%) presented a low risk of bias (≥70%) and five (12.5%) a moderate risk (50–69%). No studies were excluded due to a high risk of bias. Detailed quality scores are provided in Supplemental Tables S2 and S3.

Figure 1.

Flowchart illustrating the details of the search strategy, screening of potentially qualifying reports (n), selection of the included articles, and reasons for study exclusion.

Patient characteristics

The demographic and clinical profile of the 111 patients is summarized in Table 1. There was a marked female predominance (91%), with a mean age at diagnosis of 33.5 ± 9.8 years. The largest cohorts were provided by Deeb et al.⁸ (n = 22), Duron et al.⁹ (n = 15), and Perrone et al.⁵ (n = 11). The data reflects a global distribution, including cases from the Americas, Europe, the Middle East, and Asia.

Table 1.

Demographic and epidemiological profile of patients with Shrinking Lung Syndrome in the included studies (N = 40).

Study (Author, Year)	Country	Sample Size (n)	Age (Years)	Sex (F/M)
Walz-Leblanc et al. (1992)	Canada	1	35	F
Van Veen et al. (1993)	Netherlands	1	58	F
Soubrier et al. (1995)	France	3	32.0 ± 8.9	F
Muñoz-Rodríguez et al. (1997)	Spain	1	22	F
Hawkings et al. (2001)	United Kingdom	1	29	F
Singh et al. (2002)	United States of America	1	22	M
Al-Kheir et al. (2003)	Saudi Arabia	1	38	F
Costa et al. (2004)	Brazil	1	36	F
Oud et al. (2005)	Netherlands	5	38.6 ± 8.5	F
Al-Raqum et al. (2006)	Kuwait	1	21	F
Cavallasca et al. (2006)	Argentina	2	24/34	1 F/1 M
Allen et al. (2007)	United States of America	1	45	F
Benham et al. (2010)	Australia	1	27	F
De Santis et al. (2010)	Portugal	1	24	F
Ernest & Leung (2010)	Australia	1	49	F
Pérez-de-Llano et al. (2011)	Spain	1	22	F
Hemmati & Blocka (2012)	Canada	1	44	F
Langenskiöld et al. (2012)	Switzerland	1	28	F
Guleria et al. (2014)	India	1	28	F
Pillai et al. (2014)	United States of America	1	18	F
Toribio et al. (2014)	Spain	1	57	F
Borrell et al. (2016)	Spain	9	42.3 ± 11.9	F
Duron et al. (2016)	France	15	32.7 ± 16.0	F
Goswami et al. (2016)	India	1	38	F
Panchabhai et al. (2016)	United States of America	1	26	F
Deeb et al. (2017)	Canada	22	35.7 ± 14.6	20 F/2 M
Smyth et al. (2018)	Ireland	2	32/33	1 F/1 M
Choudhury et al. (2020)	United States of America	1	51	F
Almajed et al. (2022)	Kuwait	1	26	F
Datoo & Abdelghani (2022)	USA	1	41	F
Roy et al. (2022)	Canada	1	51	F
Al-Karaja et al. (2023)	Jordan	1	35	F
Casey et al. (2024)	Ireland	9	39.3 ± 16	6 F/3 M
He et al. (2024)	China	1	35	F
Perrone et al. (2024)	Argentina	11	32.3 ± 11.9	9 F/2 M
Sari et al. (2024)	Turkey	3	25.0 ± 2.6	F
Shah et al. (2024)	United States of America	1	38	F
Chaudhry et al. (2025)	United Kingdom	1	20	F
Fernandes et al. (2025)	Portugal	1	38	F
Roostaei et al. (2025)	Iran	1	33	F
Total/Mean	-	111	33.5 ± 9.8	91.0 % F

Legend: n: number of patients; F: Female; M: Male; NR: Not Reported. Age is expressed in years.

Note. A complete list of references for these 40 studies is provided in the Supplementary Material.

Clinical and diagnostic findings

Exertional dyspnea was nearly universal (95.5–100%), often accompanied by pleuritic chest pain (74–93%). Pulmonary function tests (PFT) revealed a restrictive pattern in 100% of patients (mean forced vital capacity [FVC]: 47–52%; mean total lung capacity: 54–60%). Diffusing capacity for carbon monoxide (DLCO) was reduced (48–49%) and diaphragmatic elevation was radiologically present in 60–96% of cases.

Therapeutic strategies and outcomes

A synthesis of treatment protocols and clinical outcomes is presented in Table 2, while the comprehensive data extraction for all individual studies is detailed in Supplemental Table S1. Moderate-to-high dose glucocorticoids were the mainstay of therapy (95–96%). Adjuvant immunosuppressants included azathioprine (30–35%) and cyclophosphamide (18–23%). Rituximab (RXT) emerged as the leading biological therapy for refractory SLS. In the analyzed cohort, RTX was used in two main ways: (1) as targeted rescue therapy for pulmonary failure after conventional immunosuppressants were exhausted (e.g., in cases reported by Langenskiöld et al., 2012 and Al-Karaja et al., 2023), and (2) as systemic therapy for patients with severe concomitant SLE activity, such as lupus nephritis or hematologic crises. Regardless of the primary indication, RTX administration was consistently associated with the stabilization or improvement of FVC and resolution of respiratory symptoms, suggesting a potent effect on the underlying diaphragmatic or neural inflammation. Non-pharmacological interventions, such as inspiratory muscle training (IMT) and non-invasive ventilation (NIV), provided additional benefits in terms of diaphragmatic strength and mobility.

Table 2.

Overview of treatment regimens and functional responses in representative studies.

Study (Author, Year)	JBI (risk of bias)	Interventions	Outcomes	Baseline FVC (% pred)	Final FVC (% pred)
Van Veen (1993)	Low	PO THEO (750 mg/d) after PDN/AZA failure	Significant clinical and functional improvement	51^a	81 (↑ 30)^a
Langenskiöld (2012)	Low	IV RTX (1g x 2) + IV CYC (600 mg/m² x 7) + B2A + THEO	Significant clinical and functional improvement	43^a	79 (↑ 36)^a
Duron (2016)	Low	CS (11/15) + IS (MMF or CYC or MTX or RTX in 8/15)	Significant improvement (9/12 improved; 3/12 stable)	46.6 ± 16.3	NR
Deeb (2017)	Low	PO PDN (28,7 mg/d) + AZA (100-150 mg/d), MTX (15-25 g/wk), or MMF (2-3 g/d)	Significant clinical and functional improvement	59.1 ± 13.2	NR
He (2024)	Low	PO Tofacitinib (10 mg BID) after PO CS (40 mg/d) + AZA (100 mg/d) + MMF (750 mg BID) failure	Sustained remission	36.7	61.9 (↑ 25.2)
Sari (2024)	Low	PO CS + IMT (starting at 30% MIP, 30 min/d, 7 days/wk, for 12 weeks)	Significant improvement in respiratory muscle strength (MIP 86 ± 3.6 cmH₂O → 111 ± 10 cmH₂O) and diaphragmatic mobility; stable lung volumes on PFT	92.3 ± 9.8	92.3 ± 9.8 (↔)
Fernandes (2025)	Low	Nocturnal NIV (iVAPS, EPAP: 6; PS: 5–10 cmH₂O) + pulmonary rehabilitation program	Clinical and functional stability	46	47 (↔)

Note. This table presents a synthesis of the primary findings. Detailed study-by-study data, including all 40 included reports, are available in Supplemental Table S1.

Legends: FVC, forced vital capacity; % pred, percentage of predicted value; THEO, Theophylline; CYC, Cyclophosphamide; RTX, Rituximab; BEL, Belimumab; PDN, Prednisone; MP, Methylprednisolone; MMF, Mycophenolate Mofetil; AZA, Azathioprine; HCQ, Hydroxychloroquine; CS, Corticosteroids; IS, Immunosuppressant; B2A, Beta-2 agonists; IV, intravenous; PO, per os; BID, Twice daily; OD, Once daily; NR, not reported; NIV, Non-invasive Ventilation; iVAPS, intelligent Volume-Assured Pressure Support; Bilevel, Bilevel Positive Airway Pressure; PH, Pulmonary Hypertension; MIP, Maximal Inspiratory Pressure; MEP, Maximal Expiratory Pressure; EPAP, Expiratory Positive Airway Pressure; PS, Pressure Support. PFT, Pulmonary Function Tests.

^aIn this study, VC was reported instead of FVC.

Regarding outcomes, clinical improvement, defined as resolution of dyspnea and pain, was achieved in 75–95% of patients. However, complete functional recovery, defined as normalization of PFT was rare, occurring in only 20–23% of the cohort. To facilitate a standardized comparison of therapeutic responses, the variation in FVC was consolidated in Table 2 and Supplemental Table S1. This approach was chosen to mitigate discrepancies in reporting styles and to provide a clearer overview of individual lung volume recovery across the included cohort.

Mortality directly attributed to SLS was low (2–5.5%). Notably, intravenous RXT consistently induced remission in cases failing conventional therapy, while inotropic agents (theophylline/beta-agonists) were effective when diaphragmatic weakness was the primary driver.

Discussion

This systematic review provides an updated, comprehensive analysis of SLS management, incorporating 111 patients. Notably, 51.3% of our sample (57 out of 111 patients) was reported within the last decade, reflecting modern clinical practices that were not captured in previous non-systematic reviews. Despite its rarity—with an estimated prevalence between 0.5% and 1.53%—SLS remains a critical manifestation of SLE that significantly impairs quality of life.

Demographics and pathophysiology

Our findings confirm a striking female predominance (91%), primarily affecting women of reproductive age (mean age 33.5 ± 9.8 years). Although SLS is traditionally associated with long-standing SLE, our analysis highlights that it can present as the initial manifestation in approximately 9–10% of cases. Understanding of its pathogenesis has evolved from simple surfactant deficiency theories to a complex multifactorial model involving diaphragmatic myopathy, phrenic neuropathy, and reflex inhibition of deep inspiration triggered by pleural inflammation. The universal restrictive pattern observed in PFT, coupled with significant reductions in Maximal Inspiratory Pressure (MIP) and Maximal Expiratory Pressure (MEP; 80–89% of cases), reinforces the hypothesis that respiratory muscle weakness is the primary functional driver of the syndrome.

Diagnostic and therapeutic challenges

Diagnosis remains a process of exclusion, in which high-resolution computed tomography is essential for ruling out interstitial lung disease or pulmonary vascular involvement. While glucocorticoids remain the first-line therapy (95–96%), our review underscores the emergence of biologic therapies and non-pharmacological interventions. RXT has shown promise in refractory cases, consistently inducing functional recovery where conventional immunosuppressants have failed.^9,15,23–25 Our analysis shows that RTX was effective both when used as a targeted rescue therapy for SLS and when administered for concomitant systemic flares, such as lupus nephritis. This superior response may be attributed to its ability to deplete B-cells involved in the underlying autoimmune-mediated diaphragmatic or neural inflammation, which often proves resistant to broad-spectrum agents. However, it is important to note that although symptomatic relief is often observed within the first two months, functional stabilization of PFT typically requires a longer period, often between 6 and 12 months. Furthermore, given the high rates of clinical improvement reported (75–95%), there is a strong likelihood of publication bias, whereby successful outcomes are overrepresented in the literature compared to therapeutic failures. This bias may potentially overstate the real-world efficacy of both glucocorticoids and RXT in this population.

While RXT presents the most robust body of evidence regarding clinical and functional success in refractory SLS, the role of alternative biological agents remains poorly defined.^1,11,12 Belimumab, a B-lymphocyte stimulator inhibitor, has demonstrated clinical benefit in isolated case reports, yet its efficacy lacks validation in broader cohorts.^1,12,18 Conversely, TNF-alpha inhibitors, such as infliximab, have been associated with unsatisfactory outcomes or even paradoxical respiratory deterioration.^1,9,10 This disparity underscores that B-cell depletion therapy may target a more fundamental axis of the autoimmune-mediated diaphragmatic or neural inflammation, which is characteristic of SLS. Other pathways appear to be less reliable in reversing established restrictive patterns.

A significant finding of this review is the role of respiratory rehabilitation. Interventions such as IMT and NIV have demonstrated efficacy in improving muscle strength and quality of life. Studies by Sari et al. (2024)¹⁷ and Fernandes et al. (2025),²⁶ although not classified as formal randomized trials, provide evidence that structured rehabilitation should be an integral part of multidisciplinary care.

Physiologically, IMT exerts benefits by applying a controlled resistive load to the diaphragm and accessory respiratory muscles. This triggers hypertrophy and enhances the recruitment of type I and type II muscle fibers.^17,27 This targeted conditioning is particularly relevant in SLS, where diaphragmatic thinning and reflex inhibition lead to chronic disuse and reduced thoracic compliance. Furthermore, by improving MIP, structured rehabilitation helps overcome the mechanical disadvantage imposed by the elevated diaphragm, potentially reducing the neural drive for dyspnea and improving the ventilatory threshold during physical activity.^17,27 These findings pave the way for larger clinical trials focusing on non-pharmacological interventions, which offer a favorable safety profile and cost-effectiveness, representing a viable adjunct to intensive immunosuppression.

Comparison with previous literature

A landmark review by Duron et al. (2016)⁹ compiled 155 cases; however, it was primarily a literature review accompanying a case series and lacked a formal systematic methodology. Our study fills this gap by employing PRISMA guidelines and standardized JBI critical appraisal. Furthermore, our data suggest that, although symptomatic improvement is achieved in up to 95% of patients, complete functional recovery (PFT normalization) is rare (20–23%). This discrepancy suggests that long-standing SLS may lead to permanent diaphragmatic fibrosis or parenchymal reorganization. Thus, early intervention is crucial to prevent irreversible damage.

Limitations

This review has limitations inherent to the rarity of SLS, as evidence remains restricted to observational data with no randomized controlled trials identified. Reliance on case reports and small case series carries a risk of publication bias. Significant heterogeneity in reporting PFT and diaphragmatic mobility, along with inconsistent follow-up periods, precluded a meta-analysis and limits the assessment of long-term sustainability. Despite these constraints, this study provides a rigorous synthesis of a large, modern SLS cohort that followed strict methodological guidelines.

Conclusions

SLS is a complex manifestation of SLE with a multifaceted pathophysiology involving diaphragmatic dysfunction and pleural inflammation. Early diagnosis and the prompt initiation of moderate-to-high dose glucocorticoids therapy appear essential for symptom control. RXT and non-pharmacological strategies, such as NIV, IMT, and respiratory rehabilitation, emerge as promising adjuvant therapies for refractory cases. Although clinical resolution of dyspnea and pain is common, complete functional recovery is rare, with approximately 80% of patients maintaining a chronic restrictive defect. This persistent volume restriction necessitates long-term functional monitoring. Standardizing therapeutic protocols through prospective multicenter studies is essential to optimize outcomes and ensure that respiratory muscle rehabilitation is integrated early into the multidisciplinary care of SLE patients.

Supplemental material

Supplemental material - Treatment strategies and clinical outcomes in shrinking lung syndrome in systemic lupus erythematosus: An updated systematic review

Supplemental material for Treatment strategies and clinical outcomes in shrinking lung syndrome in systemic lupus erythematosus: An updated systematic review by Júlia Ribeiro Lemos, Agnaldo José Lopes and Patrícia dos Santos Vigário in Lupus.

Footnotes

Acknowledgements

The authors would like to sincerely thank Míriam Raquel Meira Mainenti and Leonardo Ribeiro Marques for their valuable contributions and support throughout the development of this study. This study was supported in part by the Coordenação de Aperfeiçoamento de Pessoal de Nível Superior - Brasil (CAPES) - Finance Code 001. The authors also acknowledge the financial support provided by the Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq) and the Fundação Carlos Chagas Filho de Amparo à Pesquisa do Estado do Rio de Janeiro (FAPERJ).

ORCID iDs

Júlia Ribeiro Lemos

Agnaldo José Lopes

Patrícia dos Santos Vigário

Funding

The authors disclosed receipt of the following financial support for the research, authorship, and/or publication of this article: This study was financed in part by the Coordenação de Aperfeiçoamento de Pessoal de Nível Superior - Brasil (CAPES) - Finance Code 001.

Declaration of conflicting interests

The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Supplemental material

Supplemental material for this article is available online.

References

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

Roostaei

Kazemizadeh

Rahimi

, et al. Shrinking lung syndrome as an early manifestation of systemic lupus erythematosus: a case report with literature review. Respirol Case Rep 2025; 13(7): e70255. https://doi.org/10.1002/rcr2.70255

Pillai

Mehta

Levin

, et al. Shrinking lung syndrome presenting as an initial pulmonary manifestation of SLE. Lupus 2014; 23(11): 1201–1203. https://doi.org/10.1177/0961203314536248

Shah

Kondakindi

Enabi

, 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

Perrone

Buffarini

Szwarstein

, et al. Síndrome del pulmón encogido asociado a lupus eritematoso sistémico [Shrinking lung syndrome associated with systemic lupus erythematosus]. Medicina (B Aires). 2024;84(4):635-640. Spanish. PMID: 39172562.

Roy

Korathanakhun

Karamchandani

, et al. Myositis with prominent B-cell aggregates causing shrinking lung syndrome in systemic lupus erythematosus: a case report. BMC Rheumatol 2022; 6(1): 11. https://doi.org/10.1186/s41927-021-00240-0

Hoffbrand

Beck

. “unexplained” dyspnoea and shrinking lungs in systemic lupus erythematosus. BMJ 1965; 1(5445): 1273–1277. https://doi.org/10.1136/bmj.1.5445.1273

Deeb

Tselios

Gladman

, et al. Shrinking lung syndrome in systemic lupus erythematosus: a single-centre experience. Lupus 2018; 27(3): 365–371. https://doi.org/10.1177/0961203317722411

Duron

Cohen-Aubart

Diot

, et al. Shrinking lung syndrome associated with systemic lupus erythematosus: a multicenter collaborative study of 15 new cases and a review of the 155 cases in the literature focusing on treatment response and long-term outcomes. Autoimmun Rev 2016; 15(10): 994–1000. https://doi.org/10.1016/j.autrev.2016.07.021

10.

Borrell

Narváez

Alegre

, et al. Shrinking lung syndrome in systemic lupus erythematosus: a case series and review of the literature. Medicine (Baltim) 2016; 95(33): e4626. https://doi.org/10.1097/MD.0000000000004626

11.

Al-Karaja

Alshayeb

Amro

, et al. Shrinking lung syndrome in a systemic lupus erythematous patient improved by rituximab: a case report with literature review. Cureus 2023; 15(12): e50229. [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

12.

Chaudhry

Bokhary

Quinn

. Improved lung function with cyclophosphamide and rituximab in a case of shrinking lung syndrome. Cureus 2025; 17(11): e97612. https://doi.org/10.7759/cureus.97612, [cited 2026 Jan 1]; Available from: https://www.cureus.com/articles/420115-improved-lung-function-with-cyclophosphamide-and-rituximab-in-a-case-of-shrinking-lung-syndrome

13.

Almajed

Obri

Mahmood

, et al. Shrinking lung syndrome in a young female: a rare pulmonary manifestation of systemic lupus erythematosus. Cureus 2022; 14(4): e24320. https://doi.org/10.7759/cureus.24320. [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

14.

Datoo

Abdelghani

. Shrinking lung syndrome: a case report. Cureus 2022; 14(7): e27311. [cited 2026 Jan 1]; Available from: https://www.cureus.com/articles/96522-shrinking-lung-syndrome-a-case-report

15.

Langenskiöld

Bonetti

Fitting

, et al. Shrinking lung syndrome successfully treated with rituximab and cyclophosphamide. Respiration 2012; 84(2): 144–149. https://doi.org/10.1159/000334947

16.

Smyth

Flood

Kane

, et al. Shrinking lung syndrome and systemic lupus erythematosus: a case series and literature review. QJM Int J Med 2018; 111(12): 839–843. https://doi.org/10.1093/qjmed/hcx204

17.

Sari

Oskay

Tufan

. The effect of respiratory muscle training on respiratory muscle strength, diaphragm thickness/mobility, and exercise capacity in patients with systemic lupus erythematosus and associated shrinking lung syndrome. Lupus 2024; 33(3): 289–292. https://doi.org/10.1177/09612033241226755

18.

Choudhury

Ramos

Anjum

, et al. Shrinking lung syndrome: a rare manifestation of systemic lupus erythematosus. Cureus 2020; 12(5): e8216. [cited 2026 Jan 1]; Available from: https://www.cureus.com/articles/32163-shrinking-lung-syndrome-a-rare-manifestation-of-systemic-lupus-erythematosus

19.

Casey

Enghelmayer

Legarreta

, et al. Shrinking lung syndrome in systemic lupus erythematosus: a study of 9 patients. Med Clínica Engl Ed 2024; 162(7): 350–353. https://doi.org/10.1016/j.medcle.2023.10.024

20.

Page

McKenzie

Bossuyt

, et al. The PRISMA 2020 statement: an updated guideline for reporting systematic reviews. BMJ 2021; 372: n71.

21.

Richardson

Wilson

Nishikawa

, et al. The well-built clinical question: a key to evidence-based decisions. ACP J Club 1995; 123(3): A12–A13.

22.

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

23.

Benham

Garske

Vecchio

, et al. Successful treatment of shrinking lung syndrome with rituximab in a patient with systemic lupus erythematosus. JCR J Clin Rheumatol 2010; 16(2): 68–70. https://doi.org/10.1097/RHU.0b013e3181d0757f

24.

Goswami

Mondal

Lahiri

, et al. Shrinking lung syndrome in systemic lupus erythematosus successfully treated with rituximab. QJM. Sept 2016; 109(9): 617–618. https://doi.org/10.1093/qjmed/hcw093

25.

Toribio

Córica Albani

Mayos Pérez

, et al. Rituximab en el tratamiento del síndrome del pulmón encogido del lupus eritematoso sistémico. Reumatol Clínica 2014; 10(5): 325–327.

26.

Fernandes

Luis

Silva

, et al. Unshrinking the lung: successful improvement of shrinking lung syndrome in systemic lupus erythematosus through non-invasive ventilation and respiratory rehabilitation. Cureus 2025; Mar 11;17(3):e80400. https://doi.org/10.7759/cureus.80400, [cited 2026 Jan 1]; Available from: https://www.cureus.com/articles/350253-unshrinking-the-lung-successful-improvement-of-shrinking-lung-syndrome-in-systemic-lupus-erythematosus-through-non-invasive-ventilation-and-respiratory-rehabilitation

27.

Hill

Cecins

Eastwood

, et al. Inspiratory muscle training for patients with chronic obstructive pulmonary disease: a practical guide for clinicians. Arch Phys Med Rehabil 2010; 91(9): 1466–1470. https://doi.org/10.1016/j.apmr.2010.06.010

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