European Journal of Case Reports in Internal Medicine
Shrinking lung syndrome with favourable response to belimumab and corticosteroids alone
By Rémy Père, Anouk Le Goueff and Marina Nguenang.
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.