SYSTEMIC LUPUS ERYTHEMATOSUS

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SYSTEMIC LUPUSERYTHEMATOSUS
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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

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SPECTRUM OF DISEASE
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• ACLE ACUTE CUTANEOUS LUPUS ERYTHEMATOSUS
• SCLE SUB ACUTE CUTANEOUS LUPUS ERYTHEMATOSUS
• CCLE CHRONIC CUTANEOUS LUPUS ERYTHEMATOSUS

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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

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• 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

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PATHOGENESIS
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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

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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

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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
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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

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ACUTE LE

• BUTTER FLY
• LESION

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ERYTHEMA AT DORSA OF HAND
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SKIN LESION OF SCLE
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SCLE
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DLE
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DLE
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DLE LESION AT PINNA
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DLE LESION
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CICATRICAL ALOPECIA
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ORAL LESION
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HISTOPATHOLOGY
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IMMUNOHISTOLOGY
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TREATMENT

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