Shrinking Lung Syndrome with Favourable Response to Belimumab and Corticosteroids Alone.

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.

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Cite

BibTeX RIS

BibTeX

@article{pere2026shrinking,
  title = {{Shrinking Lung Syndrome with Favourable Response to Belimumab and Corticosteroids Alone.}},
  author = {Rémy Père and Anouk Le Goueff and Marina Nguenang},
  journal = {European journal of case reports in internal medicine},
  year = {2026},
  month = oct,
  doi = {10.12890/2026_007315},
  url = {https://europepmc.org/article/MED/43012020},
  note = {CC BY-NC-ND},
}

RIS

TY  - JOUR
TI  - Shrinking Lung Syndrome with Favourable Response to Belimumab and Corticosteroids Alone.
AU  - Rémy Père
AU  - Anouk Le Goueff
AU  - Marina Nguenang
PY  - 2026
DA  - 2026/10/16
JO  - European journal of case reports in internal medicine
DO  - 10.12890/2026_007315
UR  - https://europepmc.org/article/MED/43012020
AB  - 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.
SN  - 2284-2594
ER  -

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