Curriculum standards
Knowledge guides
LG20: Vasculitis
Key presentations and conditions
Advanced Trainees will have a comprehensive depth of knowledge of these presentations and conditions.
Less common or more complex presentations and conditions
Advanced Trainees will understand these presentations and conditions.
Advanced Trainees will understand the resources that should be used to help manage patients with these presentations and conditions.
Epidemiology, pathophysiology, and clinical sciences
Advanced Trainees will have a comprehensive depth of knowledge of the principles of the foundational sciences.
Investigations, procedures, and clinical assessment tools
Advanced Trainees will know the scientific foundation of each investigation and procedure, including relevant anatomy and physiology. They will be able to interpret the reported results of each investigation or procedure.
Advanced Trainees will know how to explain the investigation or procedure to patients, families, and carers, and be able to explain procedural risk and obtain informed consent where applicable.
Important specific issues
Advanced Trainees will identify important specialty-specific issues and the impact of these on diagnosis and management and integrate these into care.
Presentations
- Abdominal pain
- Arthritis
- Eye inflammation
- Fever
- Hypertension
- Ischaemia
- Myalgia / Myositis
- Neuropathy
- Proteinuria and/or haematuria
- Pulmonary haemorrhage
- Rash
- Recurrent sinus infection
- Signs of shock
- Stroke
Conditions
- Large vessel vasculitis:
- Takayasu arteritis
- Medium vessel vasculitis:
- Kawasaki disease
- paediatric inflammatory multisystem syndrome temporally related to SARS-CoV-2 (PIMS-TS)
- polyarteritis nodosa (PAN):
- classic PAN
- cutaneous PAN
- DADA2 deficiency
- Small vessel vasculitis:
- antineutrophil cytoplasmic antibodies (ANCA) associated:
- eosinophilic granulomatosis with polyangiitis
- granulomatosis with polyangiitis
- microscopic polyangiitis
- non-ANCA associated:
- hypocomplementaemic urticarial vasculitis
- immunoglobulin A (IgA) vasculitis
- isolated cutaneous vasculitis
- antineutrophil cytoplasmic antibodies (ANCA) associated:
- Variable vessel vasculitis:
- primary angiitis of the central nervous system (PaCNS)
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
- As previously listed, plus:
- hearing loss
- oral or genital ulceration
- vestibular dysfunction
Conditions
- Behcet disease
- Cogan syndrome
- Vasculitis secondary to infection, including:
- drugs
- hepatitis B
- malignancies
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
- Genetic factors
- Incidence and prevalence
- Male to female ratio
Pathophysiology
- Pathophysiology of the various vasculidities and their effect on different organ systems
Treatments
- Drug interactions, indications, safety monitoring, and side effect profiles for medications used in treatment of vasculitis:
- corticosteroids:
- intravenous
- intra-articular
- oral
- cytotoxic drugs
- intravenous immunoglobulin
- disease-modifying antirheumatic drugs (DMARDs):
- biological DMARDs, such as inhibitors of:
- B cells
- interleukin-1
- interleukin-6
- tumour necrosis factor α
- conventional DMARDs, such as azathioprine
- targeted synthetic DMARDs, such as Janus kinase inhibitors
- biological DMARDs, such as inhibitors of:
- other treatment strategies, including:
- antihypertensive agents
- antiplatelet agents
- anti-infective prophylaxis
- lipid-lowering agents
- corticosteroids:
- Induction versus maintenance versus refractory therapies
Clinical assessment and disease scoring tools
- Diagnostic criteria classification by:
- European Alliance of Associations for Rheumatology (EULAR)
- Paediatric Rheumatology European Society (PReS)
- Paediatric Rheumatology International Trials Organisation (PRINTO)
- Differences between adult and juvenile presentations of vasculitis
- Vasculitis-specific disease activity measures
Investigations
- Baseline blood and urine investigations assessing for involvement of specific organ systems, such as:
- kidney function
- liver function
- muscle enzymes
- Baseline investigations assessing for inflammation
- Biopsy of specific organs, as required, such as:
- kidney
- skin
- Echocardiography at baseline and for monitoring
- Imaging modalities:
- angiography, including:
- conventional
- CT
- medical radiation technologists (MRT)
- cross-sectional imaging, such as of:
- central nervous system (CNS)
- lungs
- sinuses
- radionucleotide studies
- ultrasound
- x-ray
- angiography, including:
- Immune function screening
- Indications for genetic testing
- Monitoring blood tests for patients on treatment
- Pre-immunosuppression screening, such as latent tuberculosis screening
- Respiratory function testing at baseline and for monitoring
- Specific immune testing in vasculitis, such as:
- anti-c1q antibodies
- antineutrophil cytoplasmic antibodies (ANCA), including:
- myeloperoxidase (MPO)
- proteinase-3 (PR-3)
- antinuclear antibodies (ANA)
- extractable nuclear antibodies (ENA)
- HLA-B51
- Testing of and interpretation of results for macrophage activation syndrome
Multidisciplinary team considerations
- Collaboration with other teams to make accurate diagnoses:
- infectious diseases
- nephrology
- neurology
- ophthalmology
- respiratory
Outcomes and long-term monitoring in systemic vasculitis
- Cardiovascular outcomes, including stroke risk and coronary artery disease
- Mortality rates
- Other organ-specific outcomes:
- blood vessels, such as aneurysm
- central nervous system (CNS)
- eyes
- gastrointestinal tract (GIT)
- lung
- skin