Presentations
- Calcinosis
- Cutaneous manifestations of juvenile dermatomyositis (JDM), such as Gottron papules
- Generalised weakness
For each presentation and condition, Advanced Trainees will know how to:
Synthesise
- recognise the clinical presentation
- identify relevant epidemiology, pathophysiology, and clinical science
- take a comprehensive clinical history
- conduct an appropriate examination
- establish a differential diagnosis
- plan and arrange appropriate investigation
- consider the impact of illness and disease on patients and families, and their quality of life
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
Presentations
- Dysphagia / Dysphonia
- Dyspnoea, decreased exercise tolerance, and other respiratory complaints (in the setting of weakness)
- Gastrointestinal symptoms, including haemorrhage
- Lipodystrophy
Conditions
- Antisynthetase syndrome
- Amyopathic dermatomyositis
- Calcinosis cutis
- Immune-mediated necrotising myopathy
- Interstitial lung disease (in the setting of JDM)
- Mixed connective tissue disease (MCTD) and undifferentiated connective tissue disease (UCTD)
For each presentation and condition, Advanced Trainees will know how to:
Synthesise
- recognise the clinical presentation
- identify relevant epidemiology, pathophysiology, and clinical science
- take a comprehensive clinical history
- conduct an appropriate examination
- establish a differential diagnosis
- plan and arrange appropriate investigation
- consider the impact of illness and disease on patients and families, and their quality of life
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
Epidemiology
- Annual incidence and prevalence
- Male to female ratio
Pathophysiology
- JDM pathogenesis:
- environmental factors
- genetic factors
- relevant immunological mechanisms
- Pathophysiology of juvenile dermatomyositis, as it effects each organ system
Treatment strategies
- Drug interactions, mechanism of action, safety monitoring, and side effect profiles for medications used to treat inflammatory myositis, including:
- corticosteroids:
- cytotoxic drugs
- disease-modifying antirheumatic drugs (DMARDs):
- biological DMARDs
- conventional DMARDs
- targeted synthetic DMARDs
- non-steroidal anti-inflammatory drugs
- treatment considerations:
- antihypertensive agents
- anti-infective prophylaxis
- bone health
- intravenous immunoglobulin
- Induction and remission treatment, and general organ-specific escalation pathways
- Management of calcinosis
- Non-pharmacological measures:
- allied health input, including, but not limited to:
- occupational therapy
- physiotherapy
- psychology
- speech therapy
- sun avoidance
Clinical assessment and disease scoring tools
- Assessment of nailfold capillaries
- Diagnostic and classification criteria:
- differences between adult and juvenile dermatomyositis
- Disease activity measures, including:
- myositis assessment scores, such as Childhood Myositis Assessment Scale (CMAS)
- skin assessment scores
- Use of myositis-specific antibodies to guide expected phenotype, prognosis, and treatment or monitoring strategies
Investigations
- Baseline investigations to detect inflammation, particularly muscle enzymes, such as:
- alanine transaminase (ALT)
- aspartate aminotransferase (AST)
- creatine kinase (CK)
- lactate dehydrogenase (LDH)
- Complication screening, such as:
- barium swallow
- CT – chest
- echocardiography
- lung function testing
- Imaging modalities, especially MRI of proximal muscles of the lower limbs
- Increasingly historical role of electromyography in JDM
- Indications for skin or muscle biopsies
- Utility and pitfalls of autoantibodies, including:
- antinuclear antibodies (ANA)
- extractable nuclear antigen (ENA)
- myositis-specific and associated antibodies (MSA) and methylmalonic acid (MAA)
Procedures
- Muscle biopsy, including utility and risks
- Semi-permanent intravenous catheters
- Skin biopsy
Outcomes and long-term monitoring
- Cardiovascular outcomes, including stroke risk and coronary artery disease
- Mortality rates in juvenile and adult-onset dermatomyositis
- Other organ-specific outcomes:
- central nervous system (CNS)
- eyes
- gastrointestinal tract (GIT)
- lung
- skin
- Risk of malignancy in juvenile and adult-onset dermatomyositis
