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Systemic Lupus Erythematosis

Systemic Lupus Erythematosis. Definition: Inflammatory multisystem disease of unknown cause, with diverse clinical and laboratory manifestations and variable course and prognosis….. ???. Aetiology. Unknown Genetics - 10% Infective Hormonal Sunlight

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Systemic Lupus Erythematosis

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  1. Systemic Lupus Erythematosis • Definition: Inflammatory multisystem disease of unknown cause, with diverse clinical and laboratory manifestations and variable course and prognosis…..???

  2. Aetiology • Unknown • Genetics - 10% • Infective • Hormonal • Sunlight • Drugs

  3. Who gets Lupus? • UK 12/100 000 • Sweden 39/100 000 • Cape Town 50/100 000 • Women in reproductive years: 16 - 45 • female: male 9:1

  4. constitutional 73% arthralgia 77% skin 57% arthritis 56% kidney 44% Raynaud’s 33% lymph nodes 25% CNS 24% pleurisy 23% pericarditis 20% mouth ulcers 18% vasculitis 10% myositis 7% Symptoms

  5. Organ involvement • Skin: butterfly rash, diffuse rash, vasculitis • Joints: hands, wrists, knees • Serositis: pleural/ pericardial • Kidney: mild inflammation to severe destruction • Lung: pneumonitis, haemorrhage, hypertension • Cardiac: myocarditis, endocarditis, CAD • Haematologic: anaemia, low WCC, low platelets

  6. Neuropsychiatric lupus • CNS • Epilepsy, CVA, cranial N, myelitis • Peripheral • Peripheral neuropathy, mononeuritis • Psychiatric • Psychosis • Organic brain syndrome • Depression

  7. Malar rash Discoid rash Photosensitivity Oral ulcers Arthritis Serositis Renal Neurology: seizures or psychosis Haematology: anaemia, WCC <4000, plt <100 Immunology:ds DNA (+) ANF Criteria for classification (1982)

  8. Useful antibody tests • Antinuclear Factor (ANF) • Anti-DNA Antibody (ADA) • Anti-centromere Antibody • Anti-topoisomerase1 Antibody • Extractable Nuclear Antigens • Sm • RNP • Ro/ La • Antiphospholipid Antibody(ACLA)

  9. Reasons for referral to a Rheumatologist • To confirm a diagnosis • To assess disease activity and severity • To provide a general management plan • To manage uncontrolled disease • To manage life-threatening/ organ disease • To manage/ prevent treatment toxicities • Special circumstances, eg. Pregnancy + APL

  10. Connective Tissue Diseases • Systemic Lupus Erythematosis • Lupus Variants • Progressive Systemic Sclerosis • Polymyositis/ Dermatomyositis • Vasculitis • Primary • Secondary

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