Causes of Cystic Interstitial Lung Disease Pattern

What disorders produce the cystic interstitial pattern?

The cystic interstitial pattern is the least common of the interstitial patterns on chest radiography, and the most common cause of the cystic pattern is not interstitial lung disease, but emphysema. Emphysema usually appears as a decrease in the number and conspicuity of the normal lines seen on chest radiography. It has been recognized for many years, however, that emphysema occasionally appears as an increase in the number and conspicuity of interstitial lines. When emphysema does this, it appears in the cystic interstitial pattern. Diffuse bronchiectasis may also appear as many ring shadows distributed throughout the lung parenchyma. Two rare disorders that may also cause this pattern are Langerhans cell histiocytosis (which was previously discussed along with the nodular pattern) and lymphangioleiomyomatosis. Lymphangioleiomyomatosis is a rare hormonally mediated disorder of young and middle-aged women. Proliferation of interstitial smooth muscle results in air trapping and production of small uniform cystic spaces in the lung. Here lists the causes of the cystic interstitial lung disease pattern.

Causes of Cystic Interstitial Lung Disease Pattern

  • Emphysema
  • Cystic lung disease
    • Langerhans cell histiocytosis (eosinophilic granuloma)
    • Lymphangioleiomyomatosis
  • Diffuse bronchiectasis
    • Cystic fibrosis
    • Primary cilia dyskinesia
    • Allergic bronchopulmonary aspergillosis
    • Immunodeficiency states
    • • Common variable immunodeficiency
    • • Hyper-IgE syndrome
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