Table 1 Indications for lung transplantation in interstitial lung disease reported to the ISHLT/UNOS Registry64
Idiopathic pulmonary fibrosis (IPF)*68%
Other forms of interstitial lung disease32%
Pulmonary lymphangiomyomatosis4%
Connective tissue diseases2%
Histiocytosis X1%
  • Interstitial lung disease is the diagnosis in 25% of all lung transplantations.

  • *Recorded in the Registry as IPF, not as histological subtype. However, a large majority will be the usual interstitial pneumonia (UIP) form of idiopathic interstitial pneumonia.

  • †Not specified but including occupational lung diseases; chemotherapy/radiotherapy related; extrinsic allergic alveolitis.