Shrinking lung syndrome

Dr Henry Knipe and Dr Yuranga Weerakkody et al.

Shrinking lung syndrome (SLS) refers to a rare complication of systemic lupus erythematosus (SLE), and is characterized by:

  • unexplained dyspnea
  • restrictive pattern on pulmonary function tests
  • elevated hemidiaphragm

As with SLE in general, it is thought to carry a increased female predilection. It primarily occurs during the later stage of disease.

It could affect all the components of the respiratory system, inclusive of upper airways, lung parenchyma, pulmonary vasculature, pleura, and respiratory muscles

Chest x-ray often shows small but clear lungs with diaphragmatic elevation. Occasional basal atelectasis may be present. 

CT chest often shows reduced lung volumes with diaphragmatic elevation +/- occasional basal atelectasis but without any major parenchymal lung or pleural disease 2

The term was initially coined by B.I. Hoffbrand and E.R. Beck in 1965 4.

Shrinking lung syndrome may cause significant morbidity and occasional mortality. There is no definitive therapy while corticosteroids may lessen symptoms and improve pulmonary function in some patients 5.

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Article information

rID: 46004
System: Vascular, Chest
Synonyms or Alternate Spellings:
  • Shrinking lung syndrome in SLE
  • Shrinking lung syndrome in systemic lupus erythematosus
  • Shrinking lung syndrome (SLS)

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