Lupus & antiphospholipid syndrome
Shrinking lung syndrome in systemic lupus erythematosus: Insights from a case series and a systematic review of the past decade
Authors' conclusion
SLS represents a potentially severe and underrecognized pulmonary complication of SLE.
Study context
- Population
- Patients with systemic lupus erythematosus (SLE) and shrinking lung syndrome (SLS)
- Participants
- 52 cases
- Comparison
- Case series vs Literature
- Case seriesn = 8
- Literaturen = 44
- Outcome
- Improvement in pulmonary function
Methodological detailsPRISMA, studies published 2015–2025
- PRISMA
- Studies published 2015–2025
Pooled result
As reported in the abstract
- Stabilization
- approximately one-quarter of the patients
- Clinical worsening, including one death
- 2
Further pooled results
- Patient characteristics · values are mean age — Literature
- mean 38.6 years
- Reduced lung volume with elevated diaphragmatic domes (most common imaging finding) — Literature
- 77.2%
- Patient characteristics: Our mean 31.5 (SD 7.5) years
- Reduced lung volume with elevated diaphragmatic domes (most common imaging finding): Our all the patients
Patient characteristics
- Marked female predominance; all patients had prior SLE diagnosis; dyspnea in all, followed by pleuritic chest pain and dry cough
Reduced lung volume with elevated diaphragmatic domes (most common imaging finding)
- Followed by basal atelectasis
Diaphragmatic US and pulmonary function tests
- Diaphragmatic US (half of our patients) showed reduced diaphragmatic excursion
- Pulmonary function tests consistently restrictive with reduced DLCO
Treatment
- Immunosuppressive therapy as GC-sparing strategy used in all of our cohort and most frequently reported in literature; RTX most commonly used agent
Original publication
Shrinking lung syndrome in systemic lupus erythematosus: Insights from a case series and a systematic review of the past decade. Lupus. Published 13 March 2026. doi:10.
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