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):
- Takayasu arteritis
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:
- 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:
- C-reactive protein (CRP)
- Cryoglobulins
- Erythrocyte sedimentation (ESR)
- Full blood count (FBC)
- Hepatitis serology and QuantiFERON Gold (pre-immunosuppression)
- HLA-B51
- Immunoglobulin levels:
- 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