Conditions
Diffuse lung disease
- Disorders of infancy:
- acinar dysplasia
- alveolar capillary dysplasia with misalignment of pulmonary veins
- congenital alveolar dysplasia
- diffuse developmental disorders
- Growth abnormalities:
- associated with chromosomal abnormalities (e.g., Trisomy 21)
- associated with congenital heart disease in chromosomally normal children
- chronic lung disease of prematurity / bronchopulmonary dysplasia (BPD)
- pulmonary hypoplasia
- Surfactant dysfunction disorders and related abnormalities:
- neuroendocrine cell hyperplasia of infancy (NEHI)
- pulmonary alveolar proteinosis
- pulmonary interstitial glycogenosis
- specific conditions of less well understood aetiology
- surfactant dysfunction disorders:
- ABCA3 genetic mutations
- Sp-B genetic mutations
- Sp-C genetic mutations
Disorders of the normal host
- Acute interstitial pneumonia / idiopathic diffuse alveolar damage
- Disorders related to environmental agents:
- hypersensitivity pneumonia
- toxic inhalation (including vaping)
- Eosinophilic pneumonias
- Idiopathic pulmonary haemosiderosis
- Infectious and postinfectious processes:
- postinfectious airway injury, ranging from mild airway fibrosis to constrictive / obliterative bronchiolitis with and without preceding history of viral respiratory infection
- Nonspecific interstitial pneumonia
Other systemic diseases that influence the respiratory system
- Acquired immunodeficiencies
- Congenital immunodeficiencies
- Drug-induced disease
- Graft versus host disease
- Haematological disease (including sickle cell disease)
- Post-lung transplant management
- Post-stem cell transplantation immunodeficiency
- Rheumatoid and connective tissue disorders
Vasculitides
- Pulmonary embolism
- Vasculitis (including capillaritis, small, medium, and large vessel disease)
- Veno-occlusive disease
For each presentation and condition, Advanced Trainees will know how to:
Synthesise
- recognise the clinical presentation
- identify relevant epidemiology, prevalence, pathophysiology, and clinical science
- take a comprehensive clinical history
- conduct an appropriate examination
- establish a differential diagnosis
- plan and arrange appropriate investigations
- consider the impact of illness and disease on patients and their quality of life when developing a management plan
Manage
- provide evidence-based management
- prescribe therapies tailored to patients’ needs and conditions
- recognise potential complications of disease and its management, and initiate preventative strategies
- involve multidisciplinary teams
Consider other factors
- identify individual and social factors and the impact of these on diagnosis and management
Disorders masquerading as interstitial disease
- Alpha 1 antitrypsin deficiency
- Arterial hypertensive vasculopathy
- Congestive vasculopathy, including veno-occlusive disease
- Lymphatic disorders (lymphangiectasis and lymphangiomatosis)
- Thromboembolic disease
Disorders of the immunocompromised host
- Disorders related to solid organ, lung, and stem cell transplantation, and rejection syndromes:
- graft versus host disease
- post-transplant lymphoproliferative disorder
- Disorders related to therapeutic intervention:
- chemotherapeutic and/or radiation injury
- drug hypersensitivity
- Lymphoid infiltrates related to immune compromise
- Nonspecific lymphoproliferation:
- malignant
- with lymphoid hyperplasia
- with poorly formed granulomas
- Opportunistic infections (e.g., pneumocystis)
Disorders related to systemic disease processes
- Immune-mediated disorders:
- acquired pulmonary alveolar proteinosis / autoantibody to GMCSF
- Goodpasture syndrome
- lymphoproliferative disease
- nonspecific airway changes, including:
- lymphocytic bronchiolitis
- lymphoid hyperplasia
- mild constrictive changes
- nonspecific interstitial pneumonia
- organising pneumonia
- other manifestations of collagen-vascular disease
- pulmonary hemorrhage syndromes
- pulmonary vasculitis syndromes
- Nonimmune-mediated systemic disorders:
- Langerhans cell histiocytosis
- malignant infiltrates
- sarcoidosis
- storage disease
For each presentation and condition, Advanced Trainees will know how to:
Synthesise
- recognise the clinical presentation
- identify relevant epidemiology, prevalence, pathophysiology, and clinical science
- take a comprehensive clinical history
- conduct an appropriate examination
- establish a differential diagnosis
- plan and arrange appropriate investigations
- consider the impact of illness and disease on patients and their quality of life when developing a management plan
Manage
- provide evidence-based management
- prescribe therapies tailored to patients’ needs and conditions
- recognise potential complications of disease and its management, and initiate preventative strategies
- involve multidisciplinary teams
Consider other factors
- identify individual and social factors and the impact of these on diagnosis and management
- Basic histopathology of:
- chronic pneumonitis of infancy
- desquamative interstitial pneumonitis
- nonspecific interstitial pneumonia
- pulmonary alveolar proteinosis
Investigations
- Basic histopathology of interstitial and diffuse lung disease of childhood (chILD)
- Blood tests for diagnosis of immunodeficiency / immune mediated disorder / non-immune mediated systemic disorder
- Diagnostic imaging including chest x-ray, CT, and MRI
- Genetic testing where appropriate, e.g., chILD
- Clear communication between the surgeon, physician, and pathologist in relation to lung biopsy
- Differential diagnosis of infection versus rejection in the post-transplant setting
- Genetic diagnostic technique, the role of the geneticist, and the importance of genetic counselling
- Informed consent required from patients, families and carers
- The role of the international community of practice when managing rare diseases
- The role of the multidisciplinary team when managing rare disease
- Timing of lung biopsy in the diagnosis of chILD
- Transbronchial biopsy techniques
