Curriculum standards
Knowledge guides
LG17: 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
- Altered sensation
- Erection difficulty
- Fever of unknown origin
- Haemoptysis
- Joint swelling
- Mucosal ulceration
- Myalgia
-
Pain:
- abdominal
- claudication symptoms
- eye
- joint
- testicular
- Rash
- Rectal bleeding
- Shortness of breath
- Sinusitis
- Supraglottic stenosis
- Urine change
Conditions
-
Antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitis, such as:
- eosinophilic granulomatosis with polyangiitis (EGPA)
- granulomatosis with polyangiitis (GPA)
- microscopic polyangiitis (MPA)
- Behçet disease
- Polyarteritis nodosum (PAN)
- Polymyalgia rheumatica (PMR)
- Thromboangiitis obliterans (Buerger disease)
-
Vasculitis:
- cutaneous leukocytoclastic
- immunoglobulin (Ig)-associated:
- IgA / Henoch–Schoenlein purpura
- IgG4
- large vessel:
- giant cell arteritis (GCA):
- extra-cranial GCA
- Takayasu arteritis
- giant cell arteritis (GCA):
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
- Coronary artery disease due to vasculitis
- Myocarditis
- Pericarditis
- Peripheral neuropathy and mononeuritis multiplex
Conditions
- Cogan syndrome
- Cryoglobulinemia
- Hypereosinophilic syndrome
- Kawasaki disease
- Periaortitis – Ormond's disease
- Primary angiitis of the central nervous system
- Sweet syndrome
- Vasculitis:
- cerebral
- drug-induced
- necrotising
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
- Anatomy of arteries, capillaries, and veins
- Classification and epidemiology of various systemic vasculitis
- Mechanisms of blood vessel damage / inflammation, such as:
- altered / damaged endothelial cell function due to infection, toxin, or tumour
- ANCA-mediated immune changes
- cellular and molecular immune responses involving cytokines and adhesion molecules in endothelial cells
- granuloma formation
- immune complex formation and deposition
- Pattern of arterial involvement and associated organ manifestation in different types of vasculitis
Therapeutics and management principles
- Established and emerging biologic therapy for large vessel vasculitis and PMR, such as alternative IL-6 agents and IL-17 blockage, and Janus kinase inhibitors
- Immunosuppression according to:
- disease severity
- organ involvement
- patient factors
- the type of vasculitis
- Induction and maintenance therapy based on disease activity and relapse risk
- Intravenous immunoglobulin
- Optimisation of coexisting vasculopathy
- Prevention and monitoring of potential treatment complications, such as:
- bone health and prevention / management of osteoporosis
- cardiovascular disease
- general lifestyle, and maintenance of healthy weight
- malignancy screening
- prophylactic antibiotics and antivirals
- safety bloodwork and monitoring of treatments, including disease-modifying antirheumatic drug (DMARD) side effects
- smoking cessation
- stress (peptic) ulcers and gastrointestinal effects of management
- vaccination
- Recommendation for vascular intervention, including stenting
- Selective use of apheresis therapy / plasma exchange in complex or severe cases of vasculitis
- Supportive, non-pharmacological therapy, such as compression stockings
- The role of, and preference for, prednisone, and the expanding role of steroid-sparing agents to minimise long-term prednisone complications
- Use of other anticytokine therapy, such as mepolizumab for EGPA
Clinical assessment
- Ophthalmologic examination
Imaging and other investigations
- Bronchoscopy
- CT, such as:
- angiography
- Echocardiogram
- MRA
- MRI
- Nerve conduction studies
- PET-CT
- Ultrasound – vascular for arteritis
Laboratory studies
- ANCA
- Anti-glomerular basement membrane (GBM)
- Antinuclear antibodies (ANA):
- double-stranded DNA (dsDNA)
- extractable nuclear antigen (ENA)
- Biopsy, such as:
- kidney
- lymph node
- muscle
- nasal
- skin
- temporal artery
- Complement, such as:
- C3
- C4
- C-reactive protein (CRP)
- Cryoglobulins
- Erythrocyte sedimentation (ESR)
- Full blood count (FBC)
- Hepatitis serology and QuantiFERON Gold (pre-immunosuppression)
- HLA-B51
- Immunoglobulin levels:
- IgA
- IgG4
- Kidney function test (EUC)
- Liver function test (LFT)
- Myeloperoxidase (MPO)
- Protein electrophoresis, flow cytometry, and free light chains
- Proteinase 3 (PR-3)
- Rheumatoid factor
- Streptococcal serology
- Urine:
- analysis, including 24 hour-urine collection
- immunofixation
- Choice and duration of immunotherapy, minimisation of disease relapse, and subsequent treatment of relapse disease
- Holistic care and lifestyle measures that optimise overall patient health and wellbeing
-
Immunosuppression in specific patient groups, such as:
- advanced age, or who are frail and comorbid
- allergy or intolerance to medications used
- child-bearing age
- coexisting malignancy
- lactating
- liver disease or kidney failure
- pregnant
- Management of non-inflammatory vascular complications and complications relating to immunomodulation
- Medicolegal considerations:
- appreciation of roles in the medicolegal environment, such as expert witness in the courts
- independent examiner
- preparation of documents, including how requirements may vary depending on laws of the local jurisdiction, for:
- Commonwealth agencies
- courts
- other mandatory reporting systems, such as the National Disability Insurance Scheme (NDIS)
- workers compensation authorities