Diagnosis and management of interstitial lung diseases

A proportion of patients with interstitial lung diseases can also develop a progressive-fibrosing phenotype. A diagnosis of progressive lung fibrosis is associated with failing respiratory symptoms, limited response to immunomodulation therapies,lung function decline, decreased quality of life and early death. Idiopathic pulmonary fibrosis (IPF) may be regarded as the primary type of progressive-fibrosing interstitial lung diseases.

This research paper focuses on other interstitial lung diseases that can display progressive-fibrosing phenotypes such as unclassifiable interstitial pneumonia, idiopathic nonspecific interstitial pneumonia and connective tissue disease-associated interstitial lung diseases such as fibrotic chronic hypersensitivity pneumonitis, rheumatoid arthritis, chronic sarcoidosis and interstitial lung diseases related to other environmental exposures. Differential diagnosis of these interstitial lung diseases can be difficult, and require a very detailed evaluation of radiological studies, clinical and histopathological features.Early diagnosis is crucial to ensure that these patients are treated optimally.