Curriculum standards

Knowledge guides

LG17: Systemic lupus erythematosus and related conditions

Key presentations and conditions

Advanced Trainees will have a comprehensive depth of knowledge of these presentations and conditions.

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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.

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Epidemiology, pathophysiology, and clinical sciences

Advanced Trainees will have a comprehensive depth of knowledge of the principles of the foundational sciences.

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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.

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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.

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Presentations

Arthralgia
Chest pain
Fatigue
Fever
Haemolysis
Lymphadenopathy
Oedema
Pleural / Pericardial effusion
Proteinuria
Rash
Raynaud phenomenon
Shortness of breath
Ulcers

Conditions

Antiphospholipid syndrome
Cutaneous lupus
Sjögren syndrome
Systemic lupus erythematosus (SLE):
- lupus nephritis – types I–V
- mixed connective tissue disease

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

Complete heart block – fetus / neonate
Neurological or neuropsychiatric symptoms
Secondary hemophagocytic lymphohistiocytosis (HLH)
Sicca symptoms

Conditions

Drug-related lupus
Kikuchi–Fujimoto disease
Neonatal lupus
Undifferentiated connective tissue disease

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

Difference between ethnic groups, with particular reference to Aboriginal and Torres Strait Islander peoples and Māori
Incidence and prevalence
Male to female ratio, highlighting the female predominance in autoimmune disease

Pathophysiology

Potential genetic and environmental triggers in the pathophysiology of SLE

Pharmacological treatment strategies in SLE

Drug interactions, induction versus maintenance therapy, safety monitoring, and side effect profiles for medications:
- anticoagulant drugs
- antimalarial – hydroxychloroquine
- corticosteroids
- cytotoxic drugs, such as cyclophosphamide
- disease-modifying antirheumatic drugs (DMARDs), including:
  - biological DMARDs (bDMARDs):
    - B cell targeting therapies
    - interferon-targeting
  - conventional DMARDs (cDMARDs)
- non-steroidal anti-inflammatory drugs
- other treatment strategies:
  - antihypertensive agents
  - anti-infective prophylaxis
  - lipid-lowering agents
- treatment strategies for Raynaud syndrome:
  - non-pharmacological measures
  - pharmacological measures

Clinical assessment

Diagnostic and classification criteria
Disease activity measures
Disease damage measures

Imaging and other investigations

Autoantibodies:
- antinuclear antibodies (ANA)
- antiphospholipid antibodies:
  - anticardiolipin
  - anti-B2-glycoprotien I
  - lupus anticoagulant
- antithyroid antibodies
- anti-C1q antibodies
- anti-double-stranded DNA (dsDNA)
- anti-histone antibodies
- extractable nuclear antibodies, such as:
  - La
  - RNP
  - Ro
  - Sm
- rheumatoid factor (RhF)
Biochemical analysis:
- complement studies:
  - C1q
  - C3
  - C4
  - CH50
  - CH100
- creatinine
- electrolytes
- ferritin
- immunoglobulins:
  - IgA
  - IgG
  - IgM
- liver function tests
- muscle enzymes:
  - CK
  - LDH
- thyroid function tests
- urea
Haematological markers:
- clotting studies, including lupus anti-coagulant
- complete blood count
- Coombs test
Imaging:
- angiography:
  - conventional
  - CT
  - MRI
- CT
- echocardiography
- MRI
- PET
- radionucleotide studies
- ultrasound
- x-ray
Inflammatory markers:
- C-reactive protein (CRP)
- erythrocyte sedimentation rate (ESR)
Kidney histopathology result interpretation
Lung function testing
Macrophage activation syndrome testing
Pre-immunosuppression screening
Urinalysis:
- B2-microglobulin
- protein and albumin – creatinine ratio
- spun urine microscopy
- urine ratios

Procedures

Consideration of biopsy of specific organs, such as:
- kidney
- lymph node
- skin

Outcomes and long-term monitoring in SLE

Cardiovascular outcomes in SLE
Differences between adult- and childhood-onset SLE
Mortality rates
Other organ-specific outcomes:
- central nervous system (CNS)
- eyes
- kidney
- lung
- skin
Potential triggers of flare
Role of dsDNA, complement, and inflammatory markers in monitoring disease activity