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

Abstract

BackgroundShrinking 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.MethodsFollowing 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.ResultsForty 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%).ConclusionsEarly 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.