Idiopathic pulmonary fibrosis

---was recently reviewed in the Orphanet Journal of Rare Diseases.

Key points:

While idiopathic pulmonary fibrosis (IPF) is often a wastebasket term for many patients with lung fibrosis it is actually a rare disease with specific diagnostic criteria. Because of its rarity and dismal prognosis (survival and quality of life worse than many forms of cancer) patients should not be given this label unless specific diagnostic criteria are met.

Terminology has been confusing, as the term IPF has been used interchangeably with usual interstitial pneumonia (UIP) and cryptogenic fibrosing alveolitis (CFA). UIP refers to the histologic pattern characteristic of IPF, not a particular disease. CFA is the former European term for IPF.

IPF is one of a large family of diseases popularly referred to as interstitial lung disease (ILD). ILD is an inaccurate choice of terms because these diseases usually have a phase or component of alveolitis. The preferred term is diffuse parenchymal lung disease (DPLD).

Pharmacologic options, covered in the review, are poor. Transplantation is appropriate in a select group of patients.