pulmonary fibrosis

introduction

pulmonary fibrosis occurs due to a variety of diffuse parenchymal lung diseases (DPLDs)
generally causes a restrictive pattern of lung disease

idiopathic pulmonary fibrosis
- most present with a gradual onset, often greater than 6 months, of dyspnea and/or a nonproductive cough
- mainly affects persons aged 50 years or older, 2/3rds are diagnosed at age > 60yrs
- affects ~1 in 2,000 over age 50 years
- mean survival from diagnosis is 2-5yrs and ~60% die from the condition
sarcoidosis
connective tissue (CT) disorders
- account for 15% of cases of interstitial lung disease
- eg. systemic lupus erythematosus (SLE), rheumatoid arthritis, dermatomyositis, vasculitis, Sjögren's syndrome (SS)
hypersensitivity pneumonitis
occupational exposures
drugs
radiation exposure
rarely:
- pulmonary Langerhans cell histiocytosis (PLCH)
- tuberous sclerosis
- lymphangioleiomyomatosis (LAM)
- Hermansky-Pudlak syndrome