Interstitial Lung Diseases

Curriculum

Learning Objectives:

 

  1. Approach to the patient with diffuse parenchymal lung disease
    1. Key historical points (symptoms on presentation, exposures, screening for systemic illness, R heart failure)
    2. Differential of dyspnea
    3. Who needs biopsy?
  1. Transbronchial vs. surgical lung biopsy
  2. Algorithm for referral for biopsy in UIP
  3. Lymphangioleimyomatosus – epidemiology, presentations, treatment
  4. Pulmonary Langerhans Cell Histiocytosis
  1. Idiopathic Interstitial Pneumonia
    1. Differential based on radiographic pattern (subpleural predominance, peribronchovascular distribution, ground glass, mosaic attenuation, cyst formation, nodules, honeycombing, and reticular patterns)
    2. Epidemiology, classic clinical presentation, classic radiograph patterns and pathologic differences of the 7 IIPs
    3. Idiopathic Pulmonary Fibrosis – diagnostic algorithm, followup, prognostic indicators, comorbidities
    4. Treatment options for IIPs (and failed ones for IPF)
    5. Mechanisms and potential complications of immunosuppressive agents
    6. Indications for referral and listing for lung transplant
  2. Other entities
    1. Hypersensitivity pneumonitis – epidemiology, common exposures, acute -> chronic differences in presentation and radiographs, typical pathology
    2. Drug-induced ILD – common culprits and presentations
    3. Cystic
  1. Pulmonary manifestations of systemic disease
    1. Systemic Lupus Erythematosus
    2. Rheumatoid Arthritis
    3. Systemic sclerosis – review treatment trials, how staging of ILD relates to prognosis
    4. Dermatomysositis/polymyositis
    5. Other: IBD, ankylosing spondylitis, etc
  2. Vasculitis
    1. Specific entities (Necrotizing Granulomatous polyangitis, Churg Strauss, Microscopic polyangitis, Goodpasture’s) – epidemiology, classic presentations, natural history
    2. When should vasculitis be included in the differential?
    3. Workup, treatment (remission and maintenance), followup indicators

 

Expectations of the Fellow:

  • Volume: 1-2 new consults per clinic, 1-2 followups (based on clinic volume, complexity of patients)
  • Preparation prior to clinic – chart review when available, basic knowledge of followup disease entity (epidemiology, typical treatment, natural history)
  • Workup of new patients: Adequate history (including exposure history, screening for systemic illness), summary of prior workup/films if pertinent, identify major radiographic pattern and its differential, initial plan of diagnostic workup/treatment (if applicable)
  • Followup patients: Identify the key issues in monitoring of progresss, immunosuppressive course, complications
  • Presentations: 2 during a 2 week period
    1. Article review pertinent to a patient seen (ex intervention trial in vasculitis on maintenance therapy)
    2. Overview of an entity not seen (2nd week) – review of epidemiology, natural history, presentation

 

Reading List

Articles are available on the pulmonary server

 

  • Diagnosis (including path review)
  • Idiopathic Interstitial Pneumonia
    1. Idiopathic Pulmonary Fibrosis
    2. Other
  • Connective tissue disease
  • Immunosuppression
  • Vasculitis
  • Drug-induced
  • Hypersensitivity pneumonitis
  • Other entities