CHRONIC INTERSTITIAL (RESTRICTIVE, INFILTRATIVE) LUNG DISEASES

Question 1

bilateral, often patchy, pulmonary fibrosis mainly affecting the walls of the ………

Answer 1

alveoli

Question 2

IPF is believed to result from

At risk epithelium:
Age
Genetics:

Answer 2

repeated injury and defective repair of alveolar epithelium, often in a genetically predisposed individual

Telomerase mutations
Surfactant mutations
MUC5B variant

Question 3

Microscopic features of IPF

Answer 3

patchy interstitial fibrosis /The hallmark

The earliest lesions /exuberant fibroblastic proliferation (fibroblastic foci)

cystic spaces lined by hyperplastic type II pneumocytes or bronchiolar epithelium (honeycomb fibrosis).

Question 4

Clinical features of IPF

Answer 4

gradual onset of a nonproductive cough
progressive dyspnea
characteristic “dry” or “Velcrolike” crackles during inspiration.
Cyanosis,
cor pulmonale,
peripheral edema may develop in later stages

The characteristic clinical and radiologic findings (subpleural and basilar fibrosis, reticular abnormalities, and “honeycombing”) often are diagnostic.

Question 5

Honeycomb

Answer 5

IPF
Silicosis
Asbestosis