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
- cryptogenic organizing pneumonia COP histology shows Masson bodies
UIP
- criteria for diagnosis of UIP includes lower lobe honeycombing and bronchiectasis
- NSIP has shared imaging features with UIP
Combined pulmonary fibrosis emphysema
NSIP
- 40-50 year old, subacute, fever in 1/3 pts, lack of male predominance, absence of clubbinga
- https://pmc.ncbi.nlm.nih.gov/articles/PMC3369453/: steroid dosing
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
- HIV
- LAM, HX
- HP hypersensitivity pneumonitis
IIP
First do HR CT scan. Types:
- UIP
- 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
- desquamative interstitial pneumonia (DIP)
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
- lymphoid interstitial pneumonia LIP
- LAM has cysts in normal lungs whereas Langerhan has abnormal lungs
- LAM vs PLCH vs LIP vs PBML image, histology, PFT
- lymphangioleiomyomatosis LAM
- pulmonary langerhans histiocytosis PLCH
- lymphoid interstitial pneumonia LIP
- pulmonary alveolar proteinosis
- follicular bronchiolitis
- use pirfenidone over nintedanib for less CYP interactions for pulmonary fibrosis
- nintedanib can be used to treat fibrotic HP and CTD ILD
- ILD associated with MDA-5 dermatomyositis
- mycophenolate as primary treatment for sjogren associated ILD
Links to this note
- Pulmonology
- HP hypersensitivity pneumonitis
- related: Interstitial Lung Disease ILD
- lymphangioleiomyomatosis LAM
- related: Interstitial Lung Disease ILD
- lymphoid interstitial pneumonia LIP
- related: Interstitial Lung Disease ILD
- combined pulmonary fibrosis emphysema CPFE
- related: Interstitial Lung Disease ILD
- Diffuse Parenchymal Lung Disease
- related: Pulmonology, Interstitial Lung Disease ILD
- follicular bronchiolitis
- related: Interstitial Lung Disease ILD
- LAM vs PLCH vs LIP vs PBML image, histology, PFT
- related: Interstitial Lung Disease ILD
- mulch pneumonitis is hypersensitivity in CGD patients
- related: Interstitial Lung Disease ILD
- criteria for diagnosis of UIP includes lower lobe honeycombing and bronchiectasis
- related: Interstitial Lung Disease ILD
- NSIP has shared imaging features with UIP
- related: Interstitial Lung Disease ILD
- pulmonary alveolar proteinosis
- related: Interstitial Lung Disease ILD
- LAM has cysts in normal lungs whereas Langerhan has abnormal lungs
- related: Interstitial Lung Disease ILD
- cryoptogenic organizing pneumonia chest imaging
- related: Interstitial Lung Disease ILD
- cryptogenic organizing pneumonia COP histology shows Masson bodies
- related: Interstitial Lung Disease ILD
- ILD associated with MDA-5 dermatomyositis
- 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
- mycophenolate as primary treatment for sjogren associated ILD
- related: Interstitial Lung Disease ILD
- UIP
- related: Interstitial Lung Disease ILD