What is the prognosis of restrictive lung disease?

Updated: Sep 16, 2020
  • Author: Jonathan Robert Caronia, DO; Chief Editor: John J Oppenheimer, MD  more...
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Answer

The natural history of interstitial lung diseases is variable. It depends on the specific diagnosis and the extent and severity of lung involvement based on high-resolution CT scanning and lung biopsy. [12] IPF is typically a relentless progressive disorder, and patients have a mean survival of 3-6 years after diagnosis. [13] Early recognition of IPF is important to help understand the natural history of the disorder, which may be beneficial for directing patient management and predicting (possibly improving) prognosis. [14]

Pulmonary sarcoidosis has a relatively benign self-limiting course, with spontaneous recovery or stabilization in most cases. [15] However, up to 20% of patients develop pulmonary fibrosis and disability. [16]

Prognosis for collagen-vascular diseases, eosinophilic pneumonia, cryptogenic organizing pneumonia (COP), and drug-induced lung disease is generally favorable with treatment. [17, 18, 19]

Patients with chest wall diseases and neuromuscular disorders develop progressive respiratory failure and often succumb during an intercurrent pulmonary infection. [20]


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