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Diffuse parenchymal lung disease

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Diffuse parenchymal lung disease (DPLD), also known as interstitial lung disease, refers to a group of lung diseases, affecting the alveolar epithelium, pulmonary capillary endothelium, basement membrane, perivascular and perilymphatic tissues. The term DPLD is used to distinguish these diseases from obstructive airways diseases. Most types of DPLD involve fibrosis, but this is not essential; indeed fibrosis is often a later feature. Hence the term pulmonary fibrosis has fallen out of favor.

Causes

End-stage pulmonary fibrosis of unknown origin, taken from an autopsy in the 1980's.
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End-stage pulmonary fibrosis of unknown origin, taken from an autopsy in the 1980's.

DPLD may be classified according to the cause: -

  1. Inhaled substances
  2. * Inorganic
  3. ** Silica
  4. ** Asbestos
  5. ** Beryllium
  6. * Organic
  7. ** Hypersensitivity pneumonitis
  8. Drug induced
  9. * Antibiotics
  10. * Chemotherapeutic drugs
  11. * Antiarrhythmic drugs
  12. Connective tissue disease
  13. * Systemic sclerosis
  14. * Dermatomyositis
  15. * Systemic lupus erythematosus
  16. * Rheumatoid arthritis
  17. Infection
  18. * Atypical pneumonia
  19. * Pneumocystis carinii pneumonia
  20. * Tuberculosis
  21. Idiopathic
  22. * Sarcoidosis
  23. * Idiopathic pulmonary fibrosis
  24. * Hamman-Rich syndrome
  25. Malignancy
  26. * Lymphangitic carcinomatosis

Investigation

Investigation is tailored towards the symptoms and signs. Most patients have blood testing, chest x-ray, pulmonary function testing, and high resolution CT thorax.

Treatment

DPLD is not a single disease, but encompasses many different pathological processes. Hence treatment is different for each disease.

If a specific occupational exposure cause is found, the person should avoid that environment. If a drug cause is suspected, that drug should be discontinued.

Many idiopathic and connective tissue-based causes of DPLD are treated with prednisolone. Some patients respond to immunosuppressant treatment. Patients with hypoxemia may be given supplemental oxygen.

External links

 


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