Interstitial lung disease

Learning resources

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CPD framework

The interstitial lung disease CPD framework provides an outline of topics to guide your learning and development.

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1. Immunology and defence mechanisms

  1. Anatomical barriers
  2. Reflex mechanisms (sneezing, cough and dyspnoea)
  3. Mucociliary clearance and fluid homeostasis
  4. Innate defence molecules
  5. Professional phagocytes/antigen-presenting cells
  6. Adaptive immunity and cytokine/chemokine production
  7. Granuloma formation
  8. Basics of fibrogenesis

2. Control of breathing

  1. Control systems
  2. Peripheral chemoreceptors
  3. Central chemoreceptors
  4. Testing the control system
  5. Ventilatory responses to CO2
  6. The hypoxic ventilatory response
  7. Interaction between hypoxic and hypercapnic responses
  8. Disturbances in the control of breathing
  9. Control of breathing in pulmonary diseases
  10. Type 1 and type 2 respiratory failure
  11. Respiratory stimulants

3. Control of ventilation

  1. Ventilatory response to inhaled carbon dioxide
  2. Estimation of the ventilatory response to hypoxia
  3. Respiratory muscles

4. Respiratory mechanics

  1. Airway resistance
  2. Body plethysmography
  3. Interrupter technique
  4. Forced oscillation technique
  5. Lung compliance
  6. Measurement of respiratory mechanics (total lung capacity with He (TLC He), total lung capacity with plethysmography (TLC pleth) and total lung capacity with N2 (TLC N2)) and the usefulness of the alveolar volume (AV)/total lung capacity (TLC) ratio
  7. Respiratory muscle strength: maximum inspiratory power, maximum expiratory power and sniff nasal inspiratory power

5. Gas exchange

  1. Transfer factor of the lung for carbon monoxide (TLCO)
  2. Definition
  3. Technique
  4. Calculation of TLCO and measurement of the carbon monoxide transfer coefficient (KCO)
  5. Transfer factor of the lung for nitric oxide (TLNO) and TLCO/TLNO measurement

6. Arterial blood gas (ABG) and acid-base status assessment

  1. Step 1: evaluation of the utility of ABG and capillary blood gas
  2. Step 2: diagnosis of A-B disorders: Henderson-Hasselbalch equation and the relationship between partial pressure of oxygen (PO2), partial pressure of carbon dioxide (PCO2) and pH
  3. Step 3: more on A-B disorders: importance of the D(A-a) difference, fraction of inspired oxygen (FiO2) the alveolar gas equation and measuring oxygen shunts

7. Exercise testing

  1. Exercise protocols
  2. Maximal incremental text
  3. Constant work rate tests
  4. Walking tests
  5. Indications for and basic interpretation of cardiopulmonary exercise testing
  6. Exercise variables and indexes

8. Lung function tests

  1. Interpreting lung volume
  2. Grading the severity of airflow obstruction or restriction after adoption of Z scores

9. Symptoms

  1. General symptoms of interstitial lung disease (ILD) and extrapulmonary involvement in some ILDs such as sarcoidosis and connective tissue disease (CTD)-associated ILD (CTD-ILD)
  2. Vasculitides, extrapulmonary involvement and ILDs
  3. CTD
  4. Rare lung diseases such as lymphangioleiomyomatosis (LAM)

10. Signs

  1. Velcro and extrapulmonary signs/symptoms
  2. Signs of right heart failure and pulmonary hypertension

11. Syndrome-based approach

  1. Diagnosis and differential diagnosis (i.e. Hepato-pulmonary syndrome, telomeropathies, sarcoidosis aspects, Hermansky-Pudlak syndrome, Niemann-Pick disease and Gaucher disease)
  2. CTD features
  3. Haematological diseases
  4. Occupational disease
  5. Pulmo-renal syndromes
  6. Genetic testing in ILDs

12. Endoscopic technique items

  1. Bronchoalveolar lavage/bronchial biopsy and forceps transbronchial lung biopsy
  2. Cryobiopsy technique including complications of transbronchial cryobiopsy

13. Endobronchial ultrasound (EBUS) and endoscopic ultrasound (EUS)

  1. Indications and contraindications for EBUS and EUS
  2. Sample processing and rapid on-site evaluation

14. Surgical lung biopsy

  1. Indications
  2. Contraindications
  3. Complications

15. Chest X-ray

  1. Limitations and indications of chest X-rays
  2. Basic interpretation of chest radiographs
  3. Radiological correlates of chest organs and bony chest structures
  4. Describing radiological findings of a chest radiograph
  5. Recognition of abnormal results and formulation of a diagnosis

16. Thoracic ultrasound


  1. Early detection
  2. Ultrasound appearance


17. Computed tomography (CT) scan

  1. Technique of multi-slice thickness CT (high resolution CT (HRCT)) for ILD diagnostics
  2. Patterns and differential diagnosis
  3. Computer-based quantification (CALIPER software)

18. Systemic pharmacotherapy

  1. Steroids and immunomodulators used to treat ILD
  2. Antifibrotic drugs for pulmonary fibrosis

19. Respiratory physiotherapy

Indications, limitations and role of respiratory physiotherapy in ILD

20. Pulmonary rehabilitation

Indications, limitations and role of pulmonary rehabilitation in ILD

21. Palliative care

  1. Principles of palliative care
  2. Early integration of palliative care, multidisciplinary care and communication

22. Oxygen therapy

  1. Prescription according to current statements and guidelines
  2. Oxygen toxicity
  3. Criteria for long-term oxygen therapy (LTOT) in patients with ILD
  4. Ambulatory oxygen

23. Preventative measures

  1. General aspects of preventative measures (vaccination, sports, )
  2. Dietary measure
  3. Exercise
  4. Influenza vaccination
  5. Pneumococcal vaccination
  6. SarsCoV2 vaccination
  7. Specific preventative management

24. Smoking cessation

  1. Smoking cessation
  2. ILD and emphysema
  3. ILD and lung cancer

25. Assisted ventilation

  1. Limitations of assisted ventilation in advanced cases
  2. High-flow oxygen
  3. Extracorporeal membrane oxygenation
  4. High-frequency oscillatory ventilation

26. Lung transplantation

  1. Indications and contraindications for lung transplantation
  2. Indications for early referral
  3. Indications for lung transplantation in patients with systemic diseases

27. Differential diagnosis

  1. Differential diagnosis of respiratory emergencies
  2. Acute exacerbation of fibrosing ILDs

28. Immediate management steps of respiratory emergencies

  1. Early referral to a specialist ILD centre
  2. Multidisciplinary diagnosis for ILD

29. Diffuse parenchymal lung disorders manifesting with acute lung injury

Diffuse parenchymal lung disorders manifesting with acute lung injury

30. Idiopathic pulmonary fibrosis (IPF)

  1. Clinical aspects
  2. Diagnostic aspects
  3. Treatment of IPF
  4. Acute exacerbation of IPF in patients already known to be affected by IPF
  5. Identification of patients with IPF and significant emphysema
    1. CT scan features
    2. Pulmonary function tests focusing on the discrepancy between volume preservation and significant reduction of carbon monoxide diffusing capacity (DLCO

31. COPD/emphysema and ILD

Co-existence of both diseases

32. Pulmonary TB including multidrug-resistant/extensively drug-resistant TB

High risk of TB in patients treated with biologicals

33. Lung cancer

  1. General aspects of lung cancer
  2. Lung cancer in the context of ILD
  3. Immunotherapy and ILD

34. Acute respiratory failure

  1. ILD appearing with acute respiratory failure
  2. Differential diagnosis
  3. Diagnostic approaches
  4. Treatment

35. Chronic respiratory failure

  1. Cor pulmonale in patients with advanced ILD
  2. Treatment of hypercapnia in patients with ILD
  3. Indications for O2 supplementation including LTOT and other forms of O2 delivery (e.g. non-invasive ventilation)

36. Sarcoidosis

  1. Clinical aspects
  2. Diagnostic modality
  3. Treatment of different subtypes, including life-threatening organ involvement (heart, central nervous system, hypercalcemia and others)

37. Idiopathic interstitial pneumonias

  1. Idiopathic interstitial pneumonias including cryptogenic organising pneumonia of unknown aetiology/bronchiolitis obliterans organising pneumonia
  2. Smoking-related idiopathic interstitial pneumonia
  3. Pleuro-parenchymal fibroelastosis
  4. Non-specific interstitial pneumonia
  5. Progressive fibrosing ILD
    1. Diseases with PF-ILD
    2. Treatment of PF-ILD

38. Bronchiolitis

  1. Of known causes
  2. Of unknown causes
  3. High-resolution computed tomography (HRCT) scan features
  4. Pulmonary function tests
  5. Diagnostic work-out
  6. Treatment

39. CTD

  1. CTD-ILD: a clinical overview
  2. Treatments of CTD-ILD

40. Langerhans cell histiocytosis

  1. General clinical overview
  2. Pathogenesis
  3. Treatment

41. LAM

  1. General clinical overview
  2. Pathogenesis
  3. Treatment

42. Pulmonary alveolar proteinosis

  1. General clinical overview
  2. Pathogenesis
  3. Treatment

43. Amyloidosis

  1. Knowledge of existence
  2. General clinical aspects
  3. Pathogenesis
  4. Treatment

44. Drug-induced disease

  1. General aspects of drug-induced disease
  2. Pathogenesis
  3. Treatment

45. Radiation-induced disease

Radiation-induced disease

46. Acute and chronic eosinophilic pneumonia

  1. Clinical aspects
  2. Pathogenesis
  3. Treatment

47. Hypereosinophilic syndrome

  1. Definition
  2. Clinical aspects
  3. Pathogenesis
  4. Treatment

48. Pulmonary hypertension

  1. Pathophysiology of pulmonary hypertension
  2. Pharmacological treatment of pulmonary hypertension according to the underlying disease
  3. Diagnosis of pulmonary hypertension
  4. Translating national and international management guidelines to an individual patient
  5. Appropriate decisions for referral and transfer to specialised referral centres

49. Vasculitis and diffuse pulmonary haemorrhage

  1. Definition of the main entities
  2. Granulomatosis with polyangiitis (GPA)
  3. Eosinophilic granulomatosis with polyangiitis (EGPA)
  4. Microscopic polyangiitis

50. Pleural effusion

Pleural disease may be associated with autoimmunity

51. Primary immunodeficiency syndromes

  1. Aspects of primary immunodeficiency syndromes
  2. Differential diagnosis

52. Secondary immunodeficiency syndromes/immunosuppression

Secondary immunodeficiency syndromes/immunosuppression

53. Cardiac disease

  1. General aspects of cardiac disease
  2. Differentiation between cardiac and pulmonary diseases as a cause of acute respiratory failure
  3. Differential diagnosis of the cardiac causes of acute respiratory failure
  4. Invasive cardiovascular monitoring (e.g. Swan-Ganz catheterisation)
  5. Cardiovascular effect of positive pressure ventilation

54. Gastrointestinal and liver diseases and renal failure

General aspects of lung damage related to gastrointestinal and liver diseases and renal failure in the context of ILD

55. Haematological disease

General aspects of haematological disease

56. Birt-Hogg-Dubé syndrome

  1. General aspects of Birt-Hogg-Dubé syndrome
  2. Clinical, radiological and histopathological features
  3. Genetic background and biology
  4. Detection of tumours during follow-up
  5. Treatment modality, including participation in clinical trials, and genetic counselling

Learning Activities

57. Respiratory hazards associated with occupational factors

General aspects of respiratory hazards associated with occupational factors

58. Acute inhalation injuries and their possible sequelae

  1. General aspects of acute inhalation injuries and their possible sequelae
  2. Identification of smoke inhalation and burns as causes of respiratory failure
  3. Assessment of the degree of severity of pulmonary involvement
  4. Optimal treatment of inhalation injury, including systemic effects

59. Occupational respiratory infections including those affecting healthcare workers

General aspects of occupational respiratory infections including those affecting healthcare workers

60. ILD caused by dusts of biologic origin (including extrinsic allergic alveolitis)

General aspects of ILD caused by dusts of biologic origin

61. Asbestos-related conditions

General aspects of asbestos-related conditions other than bronchopulmonary cancer (but including mesothelioma)

62. Epidemiological and statistical methods for critical appraisal

General aspects of epidemiological and statistical methods for critical appraisal

Guidelines on interstitial lung diseases

Access the catalogue of ERS guidelines, statements and technical standards relating to the topic of interstitial lung diseases.

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