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SYSTEMIC LUPUS ERYTHEMATOSUS

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Title: SYSTEMIC LUPUS ERYTHEMATOSUS


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,DR3
    SCLE-
    HLA-B8,DR3 DEFICIENCES OF C2,C3,C4
    DLE -
    HLA B-7 DR2,DR3,DQ
  • ENVIRONMENT-
  • 1. UV LIGHT gt70 CASES HAS PHOTOSENSITIVITY
  • 2. DRUGS - PROCAINAMIDE, HYDRALAZINE, INH,
    PHENYTOIN, MINOCYCLINE

    3.. 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,HODGKINS 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 - 90 dsDNA - 60 Sm - 25 rRNp - 10 - Native DNA Ribonucleoprotein Ribosomal P protein SLE, LE Nephritis CNS LE
LOW DISEASE SPECIFICITY FOR SLE ssDNA -60 Histones - 50 Ro/SS-A - 25 La/Ss-B - 25 Denatured DNA Histones Ribonucleoproteins 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
  • RAYNAUDS 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
20
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21
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22
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23
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24
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25
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26
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27
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28
CICATRICAL ALOPECIA
29
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30
ORAL LESION
31
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32
HISTOPATHOLOGY
33
IMMUNOHISTOLOGY
34
TREATMENT
  • LOCAL
  • SUN SCREENS
  • TOPICAL GLUCOCORTICOCOIDS
  • SYSTAMIC
  • ANTIMALARIALS-
  • CHLOROQUIN
  • HYDROXYCHOLROQUIN
  • DAPSONE
  • RETINOIDS
  • CLOFAZIMINE
  • SYSTAMIC GLUCOCORTICOIDS
  • AZATHIOPRINE
  • CYCLOPHOSPHAMIDE
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