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SYSTEMIC LUPUS ERYTHEMATOSUS

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Presentation on theme: "SYSTEMIC LUPUS ERYTHEMATOSUS"— Presentation transcript:

1 SYSTEMIC LUPUS ERYTHEMATOSUS

2 DEFINATION SYSTAMIC LUPUS ERYTHEMATOSUS IS A DISEASE OF UNKNOWN ETIOLOGY IN WHICH TISSUES AND CELLS ARE DAMAGED BY PATHOGENIC AUTOANTIBODIES AND IMMUNE COMPLEXES 90% OF CASES ARE WOMEN USUSALLY CHID-BEARING AGE BUT CHILDREN, MEN AND ELDERLY PERSON CAN BE AFECTED PRAVELENCE RATE IS APPROX.15 TO 50 CASES PER 100,000 POPULATION

3 SPECTRUM OF DISEASE

4 ACLE – ACUTE CUTANEOUS LUPUS ERYTHEMATOSUS
SCLE – SUB ACUTE CUTANEOUS LUPUS ERYTHEMATOSUS CCLE – CHRONIC CUTANEOUS LUPUS ERYTHEMATOSUS

5 PATHOGENESIS TISSUE DAMAGE CAUSED BY AUTOANTIBODIES IMMUNE COMPLEXES
ABNORMAL IMMUNE RESPONSES ARE 1. POLYCLONAL ANTIGEN SPECIFIC T & B CELL HYPERREACTIVITY 2. INADEQUATE REGULATION OF HYPERREACTIVITY

6 ABNORMAL IMMUNE RESPONSES DEPEND UPON INTERACTION BETWEEN
SUSCEPTIBILITY GENES ACLE - DR2,DR SCLE- HLA-B8,DR3 & DEFICIENCES OF C2,C3,C DLE - HLA B-7 DR2,DR3,DQ ENVIRONMENT- 1. UV LIGHT >70 % CASES HAS PHOTOSENSITIVITY 2. DRUGS - PROCAINAMIDE, HYDRALAZINE, INH, PHENYTOIN, MINOCYCLINE VIRUSES CMV, EPSTEIN-BARR VIRUSES

7 PATHOGENESIS

8 CLINICAL MANIFESTATION
ACLE – SKIN LESIONS WAX & WANE IN PARALLEL WITH UNDERLYING DISEASE ACTIVITY, NO SCARRING LOCALISED SYMETIRCAL ERYTHEMA & EDEMA AT MALAR EMINENCES GENERALISED - MORBILLIFORM/ EXANTHEMATOUS ERUOTIONS SCLE - PHOTOSENSITIVITY ANNULAR ERYTHEMA, PSORIASIFORM , EM. EYRTHRODERMA.& NO SCARRING ASSOCOATION WITH RO/SS-A ANTIBODIES & MALIGNANCIES-BREAST,LUNGS,GI,HODGKIN’S DISEASE. AOTOIMMUNE DISEASES

9 CCLE DISCOID ERYTHEMATOUS PLAQUES WITH ADHERENT SCALE & FOLLICULAR PLUGS-CARPET TACK SIGN HYPERPIGMENTATION AT PERIPHERY, AROPHIC CENTRAL SCARING, TELENGIEACTASIA, HYPOPIGMENTATION, SCARING ALOPECIAS, OCCURS AT SUN EXPOSED AREAS HYPERTROPHIC DLE BULLOUS LESIONS MUCOSAL – 25% OF CASES LUPUS PANNICULITIS

10 AUTOANTIBODIES ASSOCATION
ANTIGEN MOLECULAR SPECIFICITY CLINICAL ASSOCIATION HIGH DISEASE SPECIFICITY FOR SLE ANA % dsDNA % Sm % rRNp % - Native DNA Ribonucleoprotein Ribosomal P protein SLE, LE Nephritis CNS LE LOW DISEASE SPECIFICITY FOR SLE ssDNA % Histones % Ro/SS-A % La/Ss-B % Denatured DNA Histones Ribonucleoproteins Risk for SLE in DLE Drug induced SLE SCLE, neonatal LE, SSj SSJ, SCLE

11 RISK FACTORS FOR DEVELOPMENT OF SLE IN PAITENT OF DLE
DIFFUSE NONSARRING ALOPECIA GENERELISED LYMPHADENOPATHY PERIUNGAL NAIL FOLD TELENGIACTASIA RAYNAUD’S PHENOMENON UNEXPLANED ANEMIA LEUCOPENIA FALSE POSITIVE TEST FOR SYPHILS HIGH TITER OF ANA ANTI ssDNA ANTIBODIES ELEVETED ESR

12 ACUTE LE BUTTER FLY LESION

13 ERYTHEMA AT DORSA OF HAND

14 SKIN LESION OF SCLE

15 SCLE

16 DLE

17 DLE

18 DLE LESION AT PINNA

19 DLE LESION

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28 CICATRICAL ALOPECIA

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30 ORAL LESION

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32 HISTOPATHOLOGY

33 IMMUNOHISTOLOGY

34 TREATMENT LOCAL – SYSTAMIC SUN SCREENS TOPICAL GLUCOCORTICOCOIDS
ANTIMALARIALS- CHLOROQUIN HYDROXYCHOLROQUIN DAPSONE RETINOIDS CLOFAZIMINE SYSTAMIC GLUCOCORTICOIDS AZATHIOPRINE CYCLOPHOSPHAMIDE


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