Transcript SYSTEMIC LUPUS ERYTHEMATOSUS

SYSTEMIC LUPUS
ERYTHEMATOSUS
DEFINATION
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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 CHIDBEARING AGE BUT CHILDREN, MEN AND
ELDERLY PERSON CAN BE AFECTED
PRAVELENCE RATE IS APPROX.15 TO 50 CASES
PER 100,000 POPULATION
SPECTRUM OF DISEASE
• ACLE – ACUTE CUTANEOUS LUPUS
ERYTHEMATOSUS
• SCLE – SUB ACUTE CUTANEOUS LUPUS
ERYTHEMATOSUS
• CCLE – CHRONIC CUTANEOUS LUPUS
ERYTHEMATOSUS
PATHOGENESIS
• TISSUE DAMAGE CAUSED BY
• AUTOANTIBODIES
• IMMUNE COMPLEXES
• ABNORMAL IMMUNE RESPONSES ARE
• 1. POLYCLONAL ANTIGEN SPECIFIC T & B
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CELL HYPERREACTIVITY
2. INADEQUATE REGULATION OF
HYPERREACTIVITY
• 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 >70 % CASES HAS PHOTOSENSITIVITY
• 2. DRUGS
- PROCAINAMIDE, HYDRALAZINE, INH,
PHENYTOIN, MINOCYCLINE
3.. VIRUSES CMV, EPSTEIN-BARR VIRUSES
PATHOGENESIS
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.
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EYRTHRODERMA.&
NO SCARRING
ASSOCOATION WITH RO/SS-A ANTIBODIES &
MALIGNANCIES-BREAST,LUNGS,GI,HODGKIN’S
DISEASE. AOTOIMMUNE DISEASES
CCLE
• DISCOID ERYTHEMATOUS PLAQUES WITH ADHERENT
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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
AUTOANTIBODIES ASSOCATION
ANTIGEN
HIGH DISEASE
SPECIFICITY FOR
SLE
ANA
- 90%
dsDNA
- 60%
Sm
- 25%
rRNp
- 10%
LOW DISEASE
SPECIFICITY FOR
SLE
ssDNA
-60%
Histones
- 50%
Ro/SS-A
- 25%
MOLECULAR
SPECIFICITY
CLINICAL
ASSOCIATION
SLE,
LE Nephritis
Native DNA
Ribonucleoprotein
Ribosomal P protein
CNS LE
Denatured DNA
Histones
Ribonucleoproteins
Ribonucleoproteins
Risk for SLE in DLE
Drug induced SLE
SCLE, neonatal LE, SSj
SSJ, SCLE
RISK FACTORS FOR DEVELOPMENT OF SLE
IN PAITENT OF DLE
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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
ACUTE LE
BUTTER FLY
LESION
ERYTHEMA AT DORSA OF HAND
SKIN LESION OF SCLE
SCLE
DLE
DLE
DLE LESION AT PINNA
DLE LESION
CICATRICAL ALOPECIA
ORAL LESION
HISTOPATHOLOGY
IMMUNOHISTOLOGY
TREATMENT
• LOCAL –
• SUN SCREENS
• TOPICAL GLUCOCORTICOCOIDS
• SYSTAMIC
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ANTIMALARIALSCHLOROQUIN
HYDROXYCHOLROQUIN
DAPSONE
RETINOIDS
CLOFAZIMINE
SYSTAMIC GLUCOCORTICOIDS
AZATHIOPRINE
CYCLOPHOSPHAMIDE