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.
History and etymology
The term was initially coined by B.I. Hoffbrand and E.R. Beck in 1965 4.
Treatment and prognosis
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.
- 1. Singh R, Huang W, Menon Y et-al. Shrinking lung syndrome in systemic lupus erythematosus and Sjogren's syndrome. J Clin Rheumatol. 2007;8 (6): 340-5. Pubmed citation
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- 4. Karim MY, Miranda LC, Tench CM et-al. Presentation and prognosis of the shrinking lung syndrome in systemic lupus erythematosus. Semin. Arthritis Rheum. 2002;31 (5): 289-98. Pubmed citation
- 5. Warrington KJ, Moder KG, Brutinel WM. The shrinking lungs syndrome in systemic lupus erythematosus. Mayo Clin. Proc. 2000;75 (5): 467-72. doi:10.4065/75.5.467 - Pubmed citation