ILD Interstitial Lung Disease or Diffuse Parenchymal Lung Disease
- related: Pulmonology
- tags: #literature #pulmonology
Overview
- ILD is a wide group of diseases that's characterized by either inflammation or fibrosis
- They can be reversible but are often progressive
- Symptoms are usually dyspnea or cough
- Some of them can progress to end stage lung disease
- PFT findings are typically restrictive from decreased lung elasticity and thickened interstitium. Some ILD will have mixed obstruction and restriction.
Classification
Links to this note
- Pulmonology
- HP hypersensitivity pneumonitis
- LIP lymphoid interstitial pneumonia
- combined pulmonary fibrosis emphysema CPFE
- NSIP nonspecific interstitial pneumonitis
- lymphangioleiomyomatosis LAM
- NSIP has shared imaging features with UIP
- follicular bronchiolitis
- Diffuse Parenchymal Lung Disease
- mulch pneumonitis is hypersensitivity in CGD patients
- criteria for radiographic diagnosis of UIP includes lower lobe honeycombing and bronchiectasis
- LAM vs PLCH vs LIP vs PBML image, histology, PFT
- pulmonary alveolar proteinosis
- RB-ILD
- cryoptogenic organizing pneumonia chest imaging
- cryptogenic organizing pneumonia COP histology shows Masson bodies
- AIP acute interstitial pneumonia
- DIP desquamative interstitial pneumonia
- drug induced pneumonitis
- ILD associated with MDA-5 dermatomyositis
- IPF idiopathic pulmonary fibrosis
- Monitoring of Nonsteroidal Immunosuppressive Drugs in Patients With Lung Disease and Lung Transplant Recipients
- mycophenolate as primary treatment for sjogren associated ILD
- nintedanib can be used to treat fibrotic HP and CTD ILD
- UIP usual interstitial pneumonia
- LAM has cysts in normal lungs whereas Langerhan has abnormal lungs
Categorization of ILD are difficult because they can be classified based on etiology or radiographic/histopathology.
Histopathology and Radiologic Classification
- There are 6 major histopathologic pattern. They are diagnosed based on biopsy:
- UIP usual interstitial pneumonia
- NSIP nonspecific interstitial pneumonitis
- respiratory bronchiolitis
- DIP desquamative interstitial pneumonia
- organizing pneumonia
- diffuse alveolar damage
- It's important to know that some histopathologic features will also correlate with radiologic patterns (UIP on pathology with UIP pattern on CT)
- Radiologic pattern are broadly categorized as fibrotic or nonfibrotic. Common patterns include following:
- UIP pattern (criteria for radiographic diagnosis of UIP includes lower lobe honeycombing and bronchiectasis)
- fibrotic or nonfibrotic NSIP pattern (NSIP has shared imaging features with UIP)
- fibrotic or nonfibrotic (HP hypersensitivity pneumonitis)
- combined pulmonary fibrosis emphysema CPFE
- cystic lung diseases
- crazy paving pattern: pulmonary alveolar proteinosis
Clinical and Etiology Classification
You can categorize ILDs based on etiology:
- idiopathic interstitial pneumonia
- idiopathic chronic fibrosing: IPF idiopathic pulmonary fibrosis, idiopathic NSIP
- idiopathic acute/subacute: MKSAP cryoptogenic organizing pneumonia, AIP acute interstitial pneumonia
- autoimmune
- CT-ILD
- IPAF Interstitial Pneumonia with Autoimmune Feature
- ILD associated with MDA-5 dermatomyositis
- follicular bronchiolitis
- LIP lymphoid interstitial pneumonia
- CLAD chronic lung allograft dysfunction
- HP hypersensitivity pneumonitis
- CT-ILD
- granulomaous
- exposure
Treatment
- use pirfenidone over nintedanib for less CYP interactions for pulmonary fibrosis
- nintedanib can be used to treat fibrotic HP and CTD ILD
- mycophenolate as primary treatment for sjogren associated ILD
Literature Notes
- Monitoring of Nonsteroidal Immunosuppressive Drugs in Patients With Lung Disease and Lung Transplant Recipients
- Diagnostic Classification of Interstitial Lung Disease in Clinical Practice?
- Panther-IPF trial: IPF has higher mortality with prednisone + NAC + azathioprine