Year: 2026
Type: article
Study design: Case Report
Abstract: Introduction: Shrinking lung syndrome (SLS) is a rare complication of systemic lupus erythematosus. It is characterized by dyspnoea and a restrictive ventilatory defect, often associated with pleuritic chest pain. Given its rarity, there is no standardized treatment. Most published case reports describe a favourable outcome with corticosteroids alone or in combination with other immunosuppressive agents, such as cyclophosphamide or rituximab. Only a few cases treated with belimumab have been reported. Case description: We present the case of a 16-year-old female presenting with pleuritic chest pain, orthopnoea, and a restrictive ventilatory defect. Further investigations revealed positive antinuclear antibodies, a small left pleural effusion, and mild elevation of the left hemidiaphragm, findings consistent with SLS. Treatment with corticosteroids and belimumab was initiated, leading to significant clinical and biological improvement. Conclusion: This case highlights the potential role of belimumab as a first-line treatment for SLS in combination with corticosteroids alone. LEARNING POINTS: Shrinking lung syndrome is a rare condition with no standardized treatment.Most reported cases describe the use of corticosteroids in combination with azathioprine, mycophenolate mofetil or rituximab. Only a few cases have reported the use of belimumab.Belimumab may be considered as a first-line treatment option which could induce remission.
DOI: 10.12890/2026_007315
Authors: Rémy Père, Anouk Le Goueff, Marina Nguenang
Institution: Centre Hospitalier de Luxembourg
Language: English
Subfield: Rheumatology
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $323
OpenAlex ID: W7222461631