Curriculum standards

Knowledge guides

LG14: Foundations of paediatric rheumatology

Epidemiology, pathophysiology, and clinical sciences

Advanced Trainees will have in-depth knowledge of the topics listed under each clinical sciences heading.

For the statistical and epidemiological concepts listed, trainees should be able to describe the underlying rationale, the indications for using one test or method over another, and the calculations required to generate descriptive statistics.

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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 will identify important specialty-specific issues and the impact of these on diagnosis, management and outcomes.

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Anatomy

Normal growth and development, including puberty
Structure and function of:
- blood vessels
- bone
- connective tissue
- joints
- muscle
- nerves
- tendons

Associated therapies

Complementary or non-evidence-based medicine
Dietician support
Occupational therapy:
- adaptive equipment and assistive devices
- home, school, and workplace assessment
Pain management strategies
Patient education and self-management
Physiotherapy:
- exercise therapy:
  - conditioning
  - hydrotherapy
  - joint protection and energy conservation techniques
  - range of motion
  - rest and splinting
  - strengthening
  - stretching
Psychology

Immunology

Basic structure and function of:
- cellular and molecular components of the immune system
- central and peripheral lymphoid organs

Pathophysiology

Classification of rheumatic diseases of childhood
Pathophysiology of pain
Relevant adult musculoskeletal and connective tissue conditions and problems
Rheumatological emergencies

Pharmacology

Clinical use of medications, including:
- analgesics for pain
- antibiotic regimens for the prophylaxis and treatment of infectious complications in immune-suppressed children
- anticoagulants in the prophylaxis and management of thrombotic diseases
- bisphosphonates for chronic non-infectious osteomyelitis, and primary or iatrogenic osteoporosis
- corticosteroids, including the role of calcium and vitamin D supplementation:
  - intra-articular
  - intramuscular
  - intravenous
  - oral
Combination drug therapy
Cytotoxic drugs as used for organ- or life-threatening disease, such as cyclophosphamide
Disease-modifying antirheumatic drugs (DMARDs):
- biologic DMARDs (bDMARDs), including, but not limited to, inhibitors of:
  - B cells
  - co-stimulatory effects
  - interferon
  - interleukin-1 (IL-1)
  - interleukin-6 (IL-6)
  - interleukin-17 (IL-17)
  - tumour-necrosis factor α
- conventional DMARDs (cDMARDs), including, but not limited to:
  - azathioprine
  - cyclosporine
  - leflunomide
  - methotrexate
  - mycophenolate mofetil
- small-molecule DMARDs (tsDMARDs), including, but not limited to:
  - C5a inhibitors
  - Janus kinase inhibitors
Interactions with other medications
Intravenous and oral therapies for severe Raynaud phenomenon and pulmonary arterial hypertension
Monitoring and management of drug toxicity
Non-immune-suppressing immunomodulatory drugs, such as:
- colchicine
- hydroxychloroquine
Non-steroidal anti-inflammatory drugs (NSAIDs)
Pharmacology of agents used in rheumatic disease
Prescribing for children with kidney and hepatic insufficiency
Risks of pregnancy with medications
Sedation and pain management techniques for children and young people
Use of medications under special circumstances, such as off-label

Physiology

As applicable to the understanding of the mechanisms and the treatment of musculoskeletal conditions, such as:
- biomechanics
- cellular and molecular biology
- genetics
- growth development and puberty
- immune mechanisms:
  - autoimmunity
  - autoinflammation
  - immune complexes
  - infectious agents

Clinical assessment tools

Measures of disease activity, functional status, cumulative damage, and quality of life appropriate to the child’s condition. These include:
- 36-Item Short Form Survey (SF-36)
- American College of Rheumatology Pediatric 30 (ACR-Pedi 30)
- BAS Disease Activity Index (BASDAI)
- BAS Metrology Index (BASMI) for juvenile ankylosing spondylitis
- Bath Ankylosing Spondylitis Functional Index (BASFI)
- Child Health Questionnaire (CHQ-50)
- Childhood Health Assessment Questionnaire (CHAQ)
- Systemic Lupus Erythematosus Disease Activity Index (SLEDAI)
- Systemic Lupus International Collaborating Clinics (SLICC) Damage Index
Screening tools, including, but not limited to:
- paediatric gait, arms, legs and spine (pGALS)
- patient-reported experience measures (PREMS)

Diagnostic imaging techniques

Angiography:
- conventional
- imaging-based
Bone densitometry dual-energy x-ray absorptiometry (DEXA)
CT
MRI
PET
Plain x-ray
Radionucleotide scans, such as bone scan
Ultrasound

Investigations (optional)

Clinician-performed musculoskeletal ultrasound

Investigations (required)

Capillaroscopy
Interpretation of joint fluid results:
- cell counts
- culture
Interpretation of relevant laboratory and imaging results
Tissue biopsy (awareness of when it is required)

Laboratory investigations

Autoantibodies:
- antibodies to form blood elements, such as:
  - antiplatelet antibodies
  - Coombs testing:
    - direct
    - indirect
- antineutrophil cytoplasmic antibodies (ANCA), including specificities for neutrophil granule constituents, such as myeloperoxidase (MPO) and proteinase-3 (PR-3)
- antinuclear antibody (ANA)
- antiphospholipid antibodies, such as:
  - anti-beta-2-glycoprotein I antibodies
  - anti-cardiolipin
  - lupus anticoagulant
- anti-double-stranded DNA (dsDNA)
- anti-histone
- extractable nuclear antigen (ENA), including:
  - Jo-1
  - La
  - RnP
  - Ro
  - Scl-70
  - Sm
- rheumatoid factor and anticyclic citrullinated peptide (CCP)
Clinical chemistry:
- creatinine kinase and lactate dehydrogenase
- liver function tests
- serum electrolytes
- thyroid function tests
- urea and creatinine
Genetic testing:
- monogenic auto-inflammatory diseases
- storage disorders and other inborn errors of metabolism
- whole exome and parental studies, and applicability to rheumatic diseases
Haematology:
- coagulation studies
- full blood count, including white-cell differential
Inflammatory markers:
- C-reactive protein
- erythrocyte sedimentation rate
- ferritin
- serum amyloid A
Other immunology:
- complement studies C1q, C3, C4, CH50, and components of the complement cascade
- flow cytometry studies for analysis of lymphocyte subsets and function
- human leukocyte antigens (HLA) typing
- immunofixation electrophoresis, serum immunoglobulin levels, and serum protein electrophoresis
Serologic and polymerase chain reaction (PCR) tests for common infections

Neuroelectrophysiological tests

Electromyography
Nerve conduction

Procedures

Arthrocentesis of large and small joints
Intra-articular steroid injection on small and large joints

Synovial fluid analysis

Cell count and differentia
Culture and sensitivity

Assistance for families and patients, such as:
- cultural
- emotional
- psychological
Disability determination, such as:
- career advice and planning
- carer allowance and payments
- disability pension – adolescents
- educational supports
Factors affecting adherence to treatment regimens:
- access to advice and support systems
- distance from specialist help
- educational
- emotional and psychosocial
- socioeconomic
Pharmaceutical Benefits Scheme requirements for commencing certain medications, such as bDMARDs
Transitional care of adolescents, and transfer to adult services:
- adolescent-specific issues:
  - body image
  - emotional development and independence
  - risk-taking behaviours
  - social media
- local resources for transition of care to adult services
- medication-specific issues
- specific counselling regarding contraception and pregnancy
- transfer of adolescents taking off-label or compassionate-access medication
- transition guidelines for paediatric and adult rheumatology services