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SLE / fatigue / pain and swelling in the hands

Clinical Discipline(s)/Organ System(s)
Musculoskeletal System, Renal Medicine, Rheumatology, Immunology
Progress Test Topic(s)
Musculoskeletal
Description
A 19 year old Māori woman presents to her GP with painful swollen hands and lethargy. Examination reveals a malar (butterfly) rash. Further investigation demonstrates lymphopenia, an antinuclear antibody (ANA) titre 1:160 (homogenous pattern) and mild proteinuria.
Progress Test-Type Questions:   Question 1 | Question 2 | Question 3
Applied Science for Medicine 
   - Genetic basis of autoimmune disease, including relevance of HLA groups
   - Role of environmental factors in aetiology of systemic lupus erythematosus (SLE)
   - Immunopathology of SLE
   - Pathogenesis of glomerular injury seen in immune complex-mediated glomerular disease including SLE
Clinical and Communication Skills 
   - History from a patient with multisystem chronic inflammatory disease
   - Perform a full multisystem physical examination
   - Differential diagnosis of autoimmune disease
   - The clinical manifestations common to SLE (criteria for classification of SLE)
   - Differential diagnosis of a facial rash
   - The use of autoantibody and complement tests in diagnosing SLE
   - Interpretation of lung function tests with regard to incipient interstitial lung disease
   - Indications for renal biopsy in lupus nephritis/acute nephritis
   - Clear communication regarding explanation/discussion of a complex diagnosis (including investigation and management)
   - General measures advised in treatment of SLE, such as use of sun protection including sunscreens and control of cardiovascular risk factors
   - Commonly used treatments including antimalarials, glucocorticoids and immunosuppressive agents
   - Advice regarding pregnancy and contraception in women with SLE
   - The use of angiotensin converting enzyme (ACE) inhibitors for prophylaxis against major organ involvement, hypertension and renal crisis
   - The effect of SLE on life expectancy, especially in relation to cardiovascular disease
Personal and Professional Skills 
   - What to include in a primary care focussed history and examination and time-management of the consultation
   - Ability to communicate with the patient and family/whānau in a way that ensures they have sufficient knowledge and understanding to be able to make informed decisions and give informed consent
   - Identify patient's anxiety regarding immunosuppression
   - Impact of chronic disease in a young patient group regarding employment, social life, fertility, body image
Hauora Māori 
   - Importance of whānau for adolescent wellbeing
   - Ability to cater for differential health literacy needs of Māori patients and whānau
   - Awareness of differing risk profiles for Māori (and other disadvantaged populations) compared with non-Māori in New Zealand
   - Understanding of inequities in access to health services and quality of medical care for Māori, and application of this to clinical decision making and management
   - Acknowledgement of the disproportionate impact of illness on whānau employment/income, and therefore availability and models of caregiving
   - Māori patients with SLE have a markedly higher risk of developing lupus nephritis
Population Health 
   - Cost-effectiveness of disease-modifying antirheumatic drugs
   - Management, audit and monitoring of chronic autoimmune disease
   - Epidemiology of SLE: age, gender, prevalence
Conditions to be considered relating to this scenario
Common
systemic lupus erythematosus (SLE), fibromyalgia, sarcoidosis, glandular fever (EBV and CMV mononucleosis), systemic sclerosis, rheumatoid arthritis, drug-induced lupus erythematosus
Uncommon
rheumatic fever, polymyositis, tuberculosis, lymphoma, HIV