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SLE

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yogesh chiniah

on 23 May 2009

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Transcript of SLE

Systemic Lupus
Erythematosus Yogesh Chiniah
Roll No 21
Semester IX
Systemic Lupus
Erythematosus Yogesh Chiniah
Roll No 21
Semester IX SLE Autoimmune disease in which organs, tissues, and cells undergo damage mediated by tissue-binding antibodies and immune complexes
Most common multisystem connective tissue disease
SLE 30/100,000 Caucasians
200/100,000 Afro-Carribeans
90% women, peak in 2nd,3rd decades
Aetiology & Pathogenesis Aetiology & Pathogenesis Aetiology & Pathogenesis Aetiology & Pathogenesis Diverse spectrum of autoantibody production
Polyclonal B and T cell activation
Ineffective regulation of antigen availability and of ongoing antibody responses
Many autoantibodies are directed against DNA/protein or RNA/protein complexes
Many autoantigens are components of the intracellular and intranuclear machinery
Expression of novel antigens on the cell surface during apoptosis
Environmental factors that cause flares of lupus increase oxidative stress and subsequent apoptosis e.g. sunlight, UV, pregnancy, infections Multigenic disease
Some predisposing genes are located in the HLA region (particularly HLA class II DR/DQ genes and HLA class III genes encoding C’2 and C’4)
Homozygous deficiencies of C1q, C’2, C’4
Female gender is permissive
Higher antibody responses
Estradiol binds to receptors on T and B cells
OCP, HRT
Diagnosis Revised American Rheumatism Association Criteria for SLE 1. Malar rash 2. Discoid rash 3. Photosensitivity 4. Oral ulcers 5. Arthritis 6. Serositis Fixed erythema, flat or raised, sparing the nasolabial folds
Erythematous raised patches with adherent keratotic scarring and follicular plugging Skin rash as a result of unusual reaction to sunlight
Oral and nasopharyngeal ulceration, may be painless Non-erosive, involving 2 or more peripheral joints Pleuritis – convincing history of pleuritic pain, rub or pleural effusion
Pericarditis – rub, ECG evidence or effusion
7. Renal disorder 8. Neurological disorder 9. Haematological disorder 10. Immunologic disorder 11. ANA disorder Abnormal titre of ANA by immunofluorescence Anti-DNA antibodies in abnormal titre
Presence of antibodies to Sm antigen
Positive antiphospholipid antibodies Haemolytic anaemia
Leucopenia <4000/µL
Lymphopenia <1500/µL
Thrombocytopenia <100,000/µL in the absence of offending drugs
Seizures or psychosis, in the absence of offending drugs or metabolic derangement Persistent proteinuria > 0.5 g/day
Cellular casts (red cell, granular, or tubular)
Clinical features Cutaneous Biopsies of affected skin show deposition of Ig at the DEJ, injury to basal keratinocytes, and inflammation dominated by T lymphocytes in the DEJ and around the blood vessels and dermal appendages Discoid lupus erythematosus
Systemic rash
Subactue cutaneous lupus erythematosus (SCLE)
Discoid lesions are roughly circular with slightly raised, scaly hyperpigmented erythematous rims and depigmented, atrophic centres in which all dermal appendages are permanently destroyed
Lesions can be disfiguring particularly on the face and scalp
Local glucocorticoids + systemic antimalarials

Most common SLE rash is a photosensitive, slightly raised erythema, occasionally scaly, on the face (particularly the cheeks and nose – the “butterfly” rash), ears, chin, V region of the neck, upper back, and extensor surfaces of the arms SCLE consists of scaly red patches similar to psoriasis or attacks of circular red-rimmed lesions Musculoskeletal Migratory arthralgia with mild morning stiffness, tenosynovitis and small joint synovitis Nephritis
Usually asymptomatic

Investigations:
Urinalysis
Renal biopsy Renal Renal I no histological changes
II proliferative changes confined to mesangium
III proliferative changes in tufts of 10 – 50% of glomeruli
IV diffuse proliferative GN
V predominantly membranous changes with various degrees of proliferation
VI end-stage, scarred glomeruli Patients with dangerous proliferative forms of glomerular damage have microscopic haematuria and proteinuria (>500mg/24h)
Half develop NS
If DPGN is untreated, ESRD develops within 2 years of diagnosis
Renal Nervous System Cognitive dysfunction, difficulties with memory and reasoning
Headaches
Seizures
Psychosis TIA, strokes, MI
Accelerated atherosclerosis
Long-term anticoagulation
Pericarditis
Myocarditis, fibrinous endocarditis of Libman-Sachs Cardiovascular Haematology Anaemia – normocytic, normochromic
Leukopenia
Thrombocytopenia
Nausea, vomiting, diarrhoea
Vasculitis involving the intestine – perforations, ischemia, bleeding, sepsis GIT Management Avoid sun and UV light exposure
Use of high-factor sun blocks (SPF 25-50)
Mild disease – intermittent analgesics or NSAIDs
Hydroxychloroquine (200-400 mg/day) for cutaneous and joint symptoms
Short courses of oral steroids for mild to moderate disease activity (e.g. rashes, synovitis, pleuropericarditis)
Prognosis 5-year survival > 90%
Early mortality within 5 years of diagnosis is usually due to organ failure or overwhelming sepsis
Fivefold increased late mortality mainly resulting from premature cardiovascular disease
Late morbidity
Acute or life-threatening disease (i.e. renal, cerebral) requires high-dose steroids (e.g. oral prednisolone 40-60 mg daily or i.v. methyl prednisolone 500 mg – 1 g) in combination with pulse i.v. cyclophosphamide Other immunosuppressive drugs (azathioprine, methotrexate, ciclosporin, tacrolimus, mycophenolate mofetil) are useful either alone or in combination with steroids for severe but non-life-threatening manifestations or as step-down therapy after cyclophosphamide Patients with antiphospholipid antibody syndrome who have had previous thrombosis will require life-long warfarin
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