Vanishing Lung Syndrome

Q; What is Vanishing Lung Syndrome?

Answer: Vanishing Lung Syndrome or Shrinking Lung Syndrome is a complication of systemic lupus erythematosus (SLE). Most prominent complain is episodic pleuritic chest pains. Interestingly, and as name implied, there is no evidence of interstitial fibrosis on radiological workup. Various explanations have been sought but so far no concrete mechanism has been established. Most accepted theory is the myopathy causing diaphragmatic weakness, resulting in elevation of the diaphragms, impaired deep inspiration followed by parenchymal reorganization resulting in decrease lung compliance. PFT usually shows a restrictive ventilatory defect. It is a dignosis of exclusion. Treatments described include steroids, theophylline and immunosuppressive therapy.


References:

1. Karim MY, Miranda LC, Tench CM, et al. Presentation and prognosis of the shrinking lung syndrome in systemic lupus erythematosus. Semin Arthritis Rheum 2002; 31:289. 

2. Warrington KJ, Moder KG, Brutinel WM. The shrinking lungs syndrome in systemic lupus erythematosus. Mayo Clin Proc 2000; 75:467.