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:
- 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
- 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
- other treatment strategies, including:
- antihypertensive agents
- antiplatelet agents
- anti-infective prophylaxis
- lipid-lowering agents
- 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:
- 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
- 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
