Interstitial Lung Disease ILD

• related: Pulmonology

---

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

• cryoptogenic organizing pneumonia chest imaging
• MKSAP cryoptogenic organizing pneumonia

UIP

• criteria for diagnosis of UIP includes lower lobe honeycombing and bronchiectasis
• NSIP has shared imaging features with UIP

Combined pulmonary fibrosis emphysema

• combined pulmonary fibrosis emphysema CPFE

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

• langerhans histiocytosis

Literature Notes

• Monitoring of Nonsteroidal Immunosuppressive Drugs in Patients With Lung Disease and Lung Transplant Recipients

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
  • HIV
  • LAM, HX
  • hypersensitivity pneumonitis

IIP

First do HR CT scan. Types:

• UIP
  • criteria for diagnosis of UIP includes lower lobe honeycombing and bronchiectasis
• NSIP
  • NSIP has shared imaging features with UIP
  • no honeycombing
  • ground glass
  • diffused, subpleural sparing
• BR-ILD
  • smoking hx
  • central lobular nodules
  • traction bronchiectasis
• Misc
  • desquamative interstitial pneumonia (DIP)
    • end stage, severe BR-ILD
  • acute interstitial pneumonia (AIP)
    • acute/subacute, rapid few months
    • ARDS looking
    • atoll sign or reverse halo sign
    • really sick
    • treat with steroids
  • cryptogenic organizing pneumonia (COP)
    • cryoptogenic organizing pneumonia chest imaging
    • migratory
    • consolidative ground glass

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]

Links to this note

• Pulmonology
  • Interstitial Lung Disease ILD
• combined pulmonary fibrosis emphysema CPFE
  • related: Interstitial Lung Disease ILD
• hypersensitivity pneumonitis
  • related: Interstitial Lung Disease ILD
• criteria for diagnosis of UIP includes lower lobe honeycombing and bronchiectasis
  • related: Interstitial Lung Disease ILD
• Diffuse Parenchymal Lung Disease
  • related: Pulmonology, Interstitial Lung Disease ILD
• mulch pneumonitis is hypersensitivity in CGD patients
  • related: Interstitial Lung Disease ILD
• NSIP has shared imaging features with UIP
  • related: Interstitial Lung Disease ILD
• cryoptogenic organizing pneumonia chest imaging
  • related: Interstitial Lung Disease ILD
• Monitoring of Nonsteroidal Immunosuppressive Drugs in Patients With Lung Disease and Lung Transplant Recipients
  • related: Interstitial Lung Disease ILD, Pulmonology
• UIP
  • related: Interstitial Lung Disease ILD