What is Idiopathic interstitial pneumonia

The term idiopathic interstitial pneumonia actually refers to a group of diseases rather than to a specific diagnosis. These diseases pertain to problems within the lung space. The diseases classified within this group include:

  • usual interstitial pneumonitis (UIP) – the most common idiopathic interstitial pneumonia
  • nonspecific interstitial pneumonitis (NSIP)
  • bronchiolitis obliterans-organizing pneumonia (BOOP)
  • respiratory bronchiolitis associated interstitial lung disease (RB-ILD)
  • desquamative interstitial pneumonitis (DIP)
  • lymphocytic interstitial pneumonitis (LIP)
  • acute interstitial pneumonitisThe idiopathic interstitial pneumonia group of diseases can have similar clinical, radiographic and physiological presentations and are a subset of a group of diseases called ‘Interstitial lung disease.’ For such a diagnosis, all known causes of interstitial lung diseases must be ruled out.

    Statistics on Idiopathic interstitial pneumonia

    It is not clear what the true incidence of idiopathic interstitial pneumona is. The overall incidence of interstitial lung disease is approximately 60-80 cases per 100 000 people. Idiopathic interstitial pneumonia is thought to make up between 20 to 50 percent of these cases.
    Idiopathic interstitial pneumonia has no specific geographical distribution; it is found in equal proportions in urban and rural environments.

  • Usual interstitial pneumonitis – average age at diagnosis = 51 years
  • Desquamative interstitial pneumonitis – average age at diagnosis = 42 years
  • Acute interstitial pneumonitis – average age at diagnosis = 28 years

    Risk Factors for Idiopathic interstitial pneumonia

    Patients with idiopathic interstitial pneumonia are generally elderly or middle-aged with approximately two-thirds of patients being older than 60 years at diagnosis.
    A history of smoking has been associated with an increased risk of idiopathic intestitial pneumonia, but general dust exposure has not.

    How is Idiopathic interstitial pneumonia Diagnosed?

    A chest x-ray is usually performed to locate the problem within the lung. A chest CT may also be performed for a higher resolution view – this modality may decrease the need for surgical lung biopsy.

    How is Idiopathic interstitial pneumonia Treated?

    There are several treatment options for idiopathic interstitial pneumonia and sometimes the treatment depends on which type the patient has. Steroid treatment is often used. After this treatments may include immunosuppressives (which dampen a persons immune response), cytotoxic (cell destroying) or antifibrotic (stops fibrosis forming in the lung) agents. All patients are greatly encouraged to stop smoking.

    Idiopathic interstitial pneumonia References

    1. Chapman, Terry. Idiopathic interstitial lung disease. The Cleveland Clinic, 2002. http://www.clevelandclinicmeded.com/diseasemanagement/pulmonary/idiopathic/idiopathic.htm
    2. Katzenstein AL, Myers JL. Nonspecific interstitial pneumonia and the other idiopathic interstitial pneumonias: classification and diagnostic criteria. Am J Surg Pathol. 2000;24:1-3.