Interstitial Lung Disease ILD

IPF

  • No data to support use of prednisone for treatment of IPF
  • OFEV slows progression and improves survival for IPF
    • Monitor liver function when starting OFEV with monthly CMP for 3 months

Cryptogenic organizing pneumonia

UIP

Combined pulmonary fibrosis emphysema

NSIP

  • 40-50 year old, subacute, fever in 1/3 pts, lack of male predominance, absence of clubbinga

AIP

  • abrupt onset rapid progrsesive respiratory failure with diffuse opacities on CXR

Langerhans

Literature Notes

ILD aka Diffuse Parenchymal Lung Disease

major types:

  • idiopathic interstitial pneumonia (IIP)
  • Interstitial Pneumonia with Autoimmune Features (IPAF)
  • connective tissue disease related (CTD-ILD)
    • scleroderma (can also have UIP)
    • RA
    • sjogren
    • myositis
  • drug related
  • granulomatous: sarcoidosis
  • others

IIP

First do HR CT scan. Types:

subpleural sparing:

reverse halo sign:

graph LR
id1[Diffuse Parenchymal Lung Disease]-->id2["DPLD of known cause (drugs) or association (collagen, vascular disease)"]
id1-->id3[Idiopathic interstial pneumonias]
id1-->id4[Granulomatous DPLD, eg sarcoidosis]
id1-->id5[Other forms, eg LAM, HX]

graph TB
id3[Idiopathic interstial pneumonias]-->id6[Idiopathic pulmonary fibrosis] & id7[IIP other than IPF]
id7-->id8[DIP] & id9[AIP] & id10[NSIP] & id11[Cryptogenic] & id12[LIP] & id13[respiratory bronchiolitis ILD]

Idiopathic interstitial Pneumonias