• Clinical science

Interstitial lung disease


Interstitial lung diseases (ILDs) are a heterogeneous group of disorders marked by inflammatory changes in the alveoli. ILDs may be idiopathic or due to secondary causes such as autoimmune disease, pharmacotherapeutic changes, or exposure to toxic substances. These changes can cause irreversible fibrosis and impaired pulmonary function. The main symptoms are exertional dyspnea and a dry cough. Bibasilar inspiratory crackles or rales are usually heard on auscultation. Treatment is based on the underlying cause. Immune modulators and corticosteroids are used in cases of unknown etiology. In advanced stages of disease ILD can result in pulmonary insufficiency and respiratory heart failure with right ventricular insufficiency.



Clinical features



Due to the wide variety of subtypes and symptoms, there is no generally recommended diagnostic algorithm. Physical examination, serology, pulmonary function tests, and imaging (chest x-ray , CT scan) is performed almost always, while lavage or biopsy depend on the individual case.



  • In secondary disease, the first step is to limit exposure to the toxic substance, cease therapy with the drug causing symptoms, or treat the underlying disease.
  • Antibiotics if bacterial interstitial pneumonia is suspected
  • Corticosteroids and immune modulators
  • Oxygen for symptomatic or end-stage ILD
  • Lung transplantation in end-stage ILD
  • Pirfenidone and nintedanib are commonly used medication for ILD.