DOI: 10.12890/2026_007315
Shrinking lung syndrome with favourable response to belimumab and corticosteroids alone
Authors
remy.pere@outlook.com
Keywords
Shrinking lung syndrome, belimumabAbstract
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.
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