Table 6.

Recommended Surveillance for Individuals with Predisposition to Pulmonary Fibrosis

System/ConcernEvaluationFrequency
Pulmonary
fibrosis
  • PFTs: spirometry (incl FVC), plethysmography, & DLCO
  • High-resolution chest CT
  • Oxygen saturation by pulse oximetry at rest & w/exertion (exercise-related hypoxemia precedes development of hypoxemia at rest)
  • 6-MWT distance & oxygen saturation measurements
The frequency of surveillance has not been established. It is common to obtain baseline testing at age ~40 yrs. Those w/features of early disease need frequent follow up (every 3-12 mos) to determine durability & progression of findings. Those w/no evidence of early disease could have less frequent repeat testing. Since early initiation of antifibrotic therapeutics is recommended, surveillance testing at regular intervals is strongly recommended. Repeat PFTs, oxygen desaturation studies, & 6-MWT incur less radiation exposure than repeat chest CTs.
Dyskeratosis
congenita 		See Dyskeratosis Congenita and Related Telomere Biology Disorders, Surveillance.

6-MWT = six-minute walk test; DLCO = diffusion capacity of the lung for carbon monoxide; FVC = forced vital capacity; PFT = pulmonary function test

From: Pulmonary Fibrosis Predisposition Overview

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