Presentations
- Calcinosis
- Contracture
- Digital ulceration
- Dysphagia
- Muscle weakness
- Raynaud phenomenon
- Sclerodactyly
- Shortness of breath / Declining exercise tolerance
- Sicca
- Skin thickening
Conditions
- Diffuse systemic sclerosis (dSSc)
- Limited systemic sclerosis (lSSc)
- Linear scleroderma
- Localised scleroderma / Morphoea
- Mixed connective tissue disease (MCTD) / Overlap syndromes
- Morphoea en coup de Sabre
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
Conditions
- Chemical- / Drug-related scleroderma
- Graft-versus-host disease (GVHD) with sclerosis
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
- Age of onset
- Genetic factors
- Incidence and prevalence
- Male to female ratio
Pathophysiology
- Contemporary understanding of pathophysiology of disease as it effects different organ systems
- Diagnostic criteria:
- American College of Rheumatology (ACR)
- European Alliance of Associations for Rheumatology
Pharmacological treatment strategies
- Drug interactions, safety monitoring, and side effect profiles for medications used to treat scleroderma, such as:
- corticosteroids:
- cytotoxic drugs, such as cyclophosphamide
- disease-modifying antirheumatic drugs (DMARDs):
- biological DMARDs (bDMARDs):
- IL-6-cell targeting therapies
- Janus kinase inhibitors
- tumour necrosis factor (TNF)-targeting
- conventional DMARDs (cDMARDs):
- cyclosporine
- methotrexate
- mycophenolate mofetil
- other treatment strategies:
- antifibrotic agents
- antihypertensive agents
- anti-infective prophylaxis
- haemopoetic stem cell transplantation
- lipid-lowering agents
- vasodilatory agents
Clinical assessment and disease scoring tools
- Diagnostic criteria and disease activity measurement
Investigations
- Assessment of:
- cardiac involvement:
- echocardiogram
- electrocardiogram (ECG)
- gastrointestinal involvement, such as barium swallow
- respiratory involvement:
- high-resolution CT
- lung function testing
- Baseline blood and urine investigations assessing for organ dysfunction, such as:
- kidney function
- liver function
- muscle enzymes
- Baseline investigations looking for inflammation
- Monitoring blood tests for patients on treatment
- Pre-immunosuppression screening, such as latent tuberculosis screening
- Role of different imaging modalities in:
- assessing for underlying structural damage
- diagnosis of scleroderma and consideration of differentials
- surveillance of disease activity, and assessing damage over time
- Skin biopsy
- Specific immune testing in scleroderma, such as:
- antinuclear antibody (ANA)
- extractable nuclear antibodies, including:
- anticentromere
- La
- RNP
- Ro
- Scl-70
- Sm
- scleroderma-associated antibodies, including:
- myositis specific antibodies
- RNA-polymerase III
Outcomes and long-term monitoring
- Cardiovascular outcomes, including stroke risk and coronary artery disease
- Guidelines for monitoring for disease progression, such as for interstitial lung disease (ILD)
- Mortality rates in juvenile scleroderma
- Other organ-specific outcomes:
- central nervous system (CNS)
- eyes
- gastrointestinal tract (GIT)
- lung
- skin
