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
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