pulmonary fibrosis

see also:

• respiratory medicine

• 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