SHRINKING LUNG SYNDROME IN SYSTEMIC LUPUS ERYTHEMATOSUS-CLINICAL AND THERAPEUTIC CHALLENGES
DOI:
https://doi.org/10.31435/ijitss.1(49).2026.4607Keywords:
Systemic Lupus Erythematosus, Shrinking Lung Syndrome, Diaphragmatic Dysfunction, Autoimmune Disease, Respiratory Failure, Immunosuppressive Therapy, SLE ComplicationsAbstract
Background: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by multisystem involvement. One of its rare but clinically significant complications is the shrinking lung syndrome (SLS), which affects less than 1% of patients and presents diagnostic and therapeutic challenges.
Aim: This paper aims to present a comprehensive overview of shrinking lung syndrome as a rare manifestation of SLE, with a focus on its pathophysiology, diagnostic difficulties, and current therapeutic approaches.
Materials and Methods: A narrative review was conducted based on selected scientific publications indexed in PubMed, Scopus, and Google Scholar. The inclusion criteria included articles describing the pathophysiology, clinical features, diagnosis, and treatment of SLS in the context of SLE.
Results: SLS is characterized by progressive dyspnea, diaphragmatic elevation, and restrictive defects on pulmonary function testing. The exact pathogenesis remains unclear but involves neuromuscular dysfunction, diaphragmatic weakness, pleural involvement, and potential autoantibody-mediated mechanisms. Diagnosis is challenging due to the absence of specific markers and requires exclusion of other causes. Treatment primarily includes corticosteroids and immunosuppressants, with adjunctive respiratory rehabilitation. Despite therapy, pulmonary restriction may persist.
Conclusions: SLS remains an under-recognized and underdiagnosed complication of SLE. Increased clinical awareness and early diagnostic interventions are key to preventing long-term respiratory impairment. Future research is needed to establish standardized diagnostic protocols and treatment guidelines.
References
Szczeklik, A. (Ed.). (2024). Szczeklik’s interna. Medycyna Praktyczna.
Lalosević, V., Ristić, G. G., Lakičević, D., et al. (2022). Myositis with prominent B-cell aggregates causing shrinking lung syndrome in systemic lupus erythematosus: A case report. BMC Rheumatology, 6, Article 12. https://doi.org/10.1186/s41927-021-00240-0
Colquhoun, M., & Akram, S. (2023). Shrinking lung syndrome. In StatPearls. StatPearls Publishing. https://www.ncbi.nlm.nih.gov/books/NBK568683/
Luo, Y., Qian, Y., Li, S., et al. (2022). Systemic lupus erythematosus and lung involvement: A comprehensive review. Journal of Clinical Medicine, 11(22), Article 6714. https://doi.org/10.3390/jcm11226714
Fosam, H. (2017, September 11). Advancing understanding of shrinking lung syndrome in SLE. Rheumatology Advisor. https://www.rheumatologyadvisor.com/features/advancing-understanding-of-shrinking-lung-syndrome-in-sle/
Allen, D., Gladman, D. D., Ibañez, D., & Urowitz, M. B. (2000). The shrinking lungs syndrome in systemic lupus erythematosus. Mayo Clinic Proceedings, 75(5), 467–472. https://doi.org/10.4065/75.5.467
Robles-Perez, A., Lazaro, E., Valls-Roc, M., et al. (2016). Shrinking lung syndrome in systemic lupus erythematosus: A case series and review of the literature. Medicine, 95(33), Article e4626. https://doi.org/10.1097/MD.0000000000004626
Mier, R. J., Brody, J. S., & Wasserman, K. (1989). Diaphragm strength in the shrinking lung syndrome of systemic lupus erythematosus. American Review of Respiratory Disease, 139(5), 1269–1273. https://doi.org/10.1164/ajrccm/139.5.1269
Tam, L. S., Wong, S. H., Szeto, C. C., et al. (2011). Shrinking lung syndrome caused by lupus myopathy. QJM, 104(3), 259–262. https://doi.org/10.1093/qjmed/hcq174
Toyoda, D., Bhargava, M., Kobayashi, H., et al. (2013). Shrinking lung syndrome as a manifestation of pleuritis: A new model based on pulmonary physiological studies. Chest, 143(2), 502–510. https://doi.org/10.1378/chest.12-0951
Kelley, M. A., Coulson, W. F., & Kennedy, T. P. (2001). Bilateral phrenic paralysis in a patient with systemic lupus erythematosus. Chest, 119(4), 1287–1289. https://doi.org/10.1378/chest.119.4.1287
Toyoda, D., Bhargava, M., Kobayashi, H., et al. (2013). Shrinking lung syndrome as a manifestation of pleuritis. The Journal of Rheumatology, 40(3), 273–278. https://doi.org/10.3899/jrheum.120662
Agarwal, A., Aggarwal, A., Gupta, R., et al. (2021). Shrinking lung syndrome treated with rituximab in pediatric systemic lupus erythematosus: A case report and review of the literature. Pediatric Rheumatology Online Journal, 19(1), Article 3. https://doi.org/10.1186/s12969-020-00491-0
Chen, M., et al. (2024). Shrinking lung syndrome with diaphragm myopathy and phrenic neuropathy [Manuscript].
Armour, M. (n.d.). Respiratory changes in the course of systemic connective tissue diseases. Practical Medicine. https://www.czytelniamedyczna.pl/1553
Shrinking lung syndrome in systemic lupus erythematosus patients: A diagnosis we should suspect more often. (n.d.). ACR Abstracts. https://acrabstracts.org/abstract/shrinking-lung-syndrome-in-systemic-lupus-erythematosus-patients-a-diagnosis-we-should-suspect-more-often/
Mekinian, A., Maalouf, G., Tournadre, A., et al. (2014). Shrinking lung syndrome in systemic lupus erythematosus-scleroderma overlap. Clinical Medicine Insights: Case Reports, 7, 63–66. https://doi.org/10.4137/CCRep.S16227
Suárez-Díaz, S., Márquez, J., Medina, A., et al. (2021). Shrinking lung syndrome: A rare pulmonary manifestation of lupus. Revista Colombiana de Reumatología (English Edition), 28(3), 177–180. https://doi.org/10.1016/j.rcreue.2021.02.002
Duron, L., Fortin, J., Elhai, M., et al. (2017). Shrinking lung syndrome in systemic lupus erythematosus: A single-center experience. Lupus, 26(11), 1161–1165. https://doi.org/10.1177/0961203317722411
Agarwal, A., Aggarwal, A., Gupta, R., et al. (2021). Shrinking lung syndrome treated with rituximab in pediatric SLE: A case report. Pediatric Rheumatology. https://pmc.ncbi.nlm.nih.gov/articles/PMC7789161/
Ali, R., Waseem, M., Shaikh, O., et al. (2025). Unshrinking the lung: Successful improvement of shrinking lung syndrome in systemic lupus erythematosus. Cureus, 17(3), Article e350253. https://doi.org/10.7759/cureus.350253
Ramos-Casals, M., Brito-Zerón, P., Sisó-Almirall, A., & Bosch, X. (2021). Respiratory manifestations in systemic lupus erythematosus. Pharmaceuticals, 14(3), Article 276. https://doi.org/10.3390/ph14030276
Duron, L., et al. (2016). Shrinking lung syndrome associated with systemic lupus erythematosus: A multicenter collaborative study of 15 new cases. Autoimmunity Reviews, 15(9), 885–889. https://doi.org/10.1016/j.autrev.2016.06.005
Valero, C. P., Segura, B. T., Ricart, M. J., et al. (2015). Rituximab in the treatment of shrinking lung syndrome in systemic lupus erythematosus. Reumatología Clínica, 11(2), 121–123. https://doi.org/10.1016/j.rheumae.2014.01.003
Case report: Diaphragm ultrasound reveals shrinking lung syndrome. (2024, July 9). The Rheumatologist. https://www.the-rheumatologist.org/article/case-report-diaphragm-ultrasound-reveals-shrinking-lung-syndrome/
El-Refaei, A. F., El-Fakharany, Y., Ibrahim, M. A., et al. (2024). Shrinking lung syndrome in systemic lupus erythematosus. Middle East Journal of Medical Research. https://mjmr.journals.ekb.eg/article_325876
Samanta, A., et al. (2020). Lupus and the lungs: Assessment and management of pulmonary manifestations of SLE. Frontiers in Medicine, 7, Article 610257. https://doi.org/10.3389/fmed.2020.610257
Deeb, M., Tselios, K., Gladman, D. D., Su, J., & Urowitz, M. B. (2018). Shrinking lung syndrome in systemic lupus erythematosus: A single-center experience. Lupus, 27(3), 365–371. https://doi.org/10.1177/0961203317722411
Mittoo, S., & Fell, C. D. (2014). Pulmonary manifestations of systemic lupus erythematosus. Seminars in Respiratory and Critical Care Medicine, 35(2), 249–254. https://doi.org/10.1055/s-0034-1371537
Kamen, D. L., & Strange, C. (2016). Shrinking lung syndrome in systemic lupus erythematosus: A case series and review of the literature. Medicine, 95(33), Article e4626. https://doi.org/10.1097/MD.0000000000004626
Borrell, H., Narváez, J., Alegre, J. J., Castellví, I., Mitjavila, F., Aparicio, M., et al. (2016). Shrinking lung syndrome in a female with systemic lupus erythematosus. Medicine, 95(33), Article e4626. https://doi.org/10.1097/MD.0000000000004626
Blanco Jimenez, J., & Aftab, G. (2021). Shrinking lung syndrome in a female with systemic lupus erythematosus. Chest, 160(4), A2106. https://doi.org/10.1016/j.chest.2021.08.038
Downloads
Published
Issue
Section
License
Copyright (c) 2026 Barbara Reizer, Zofia Botto, Marzena Swojnóg, Klaudia Krystek, Patrycja Felisiak, Dominika Kowalczyk, Dominika Bieszczad, Magdalena Barczewska, Zofia Śliwa, Dominik Andrzej Ślazyk
This work is licensed under a Creative Commons Attribution 4.0 International License.
All articles are published in open-access and licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0). Hence, authors retain copyright to the content of the articles.
CC BY 4.0 License allows content to be copied, adapted, displayed, distributed, re-published or otherwise re-used for any purpose including for adaptation and commercial use provided the content is attributed.
How to Cite
