SYSTEMIC LUPUSERYTHEMATOSUS

DEFINATIONDEFINATION

SYSTAMIC LUPUS ERYTHEMATOSUS IS A SYSTAMIC LUPUS ERYTHEMATOSUS IS A DISEASE OF UNKNOWN ETIOLOGY IN DISEASE OF UNKNOWN ETIOLOGY IN WHICH TISSUES AND CELLS ARE DAMAGED WHICH TISSUES AND CELLS ARE DAMAGED BY PATHOGENIC AUTOANTIBODIES AND BY PATHOGENIC AUTOANTIBODIES AND IMMUNE COMPLEXESIMMUNE COMPLEXES

• 90% OF CASES ARE WOMEN USUSALLY 90% OF CASES ARE WOMEN USUSALLY CHID-BEARING AGE BUT CHILDREN, MEN CHID-BEARING AGE BUT CHILDREN, MEN AND ELDERLY PERSON CAN BE AFECTEDAND ELDERLY PERSON CAN BE AFECTED

• PRAVELENCE RATE IS APPROX.15 TO 50 PRAVELENCE RATE IS APPROX.15 TO 50 CASES PER 100,000 POPULATIONCASES PER 100,000 POPULATION

SPECTRUM OF DISEASESPECTRUM OF DISEASE

• ACLE – ACUTE CUTANEOUS LUPUS ACLE – ACUTE CUTANEOUS LUPUS ERYTHEMATOSUSERYTHEMATOSUS

• SCLE – SUB ACUTE CUTANEOUS SCLE – SUB ACUTE CUTANEOUS LUPUS ERYTHEMATOSUSLUPUS ERYTHEMATOSUS

• CCLE – CHRONIC CUTANEOUS LUPUS CCLE – CHRONIC CUTANEOUS LUPUS ERYTHEMATOSUSERYTHEMATOSUS

PATHOGENESISPATHOGENESIS• TISSUE DAMAGE CAUSED BY TISSUE DAMAGE CAUSED BY

• AUTOANTIBODIES AUTOANTIBODIES

• IMMUNE COMPLEXESIMMUNE COMPLEXES

• ABNORMAL IMMUNE RESPONSES ARE ABNORMAL IMMUNE RESPONSES ARE

• 1. POLYCLONAL ANTIGEN SPECIFIC T & B 1. POLYCLONAL ANTIGEN SPECIFIC T & B CELL HYPERREACTIVITY CELL HYPERREACTIVITY

• 2. INADEQUATE REGULATION OF 2. INADEQUATE REGULATION OF HYPERREACTIVITY HYPERREACTIVITY

• ABNORMAL IMMUNE RESPONSES DEPEND ABNORMAL IMMUNE RESPONSES DEPEND UPON INTERACTION BETWEEN UPON INTERACTION BETWEEN

• SUSCEPTIBILITY GENES- SUSCEPTIBILITY GENES- ACLE - DR2,DR3 ACLE - DR2,DR3 SCLE- HLA-B8,DR3 & DEFICIENCES OF C2,C3,C4 SCLE- HLA-B8,DR3 & DEFICIENCES OF C2,C3,C4 DLE - HLA B-7 DLE - HLA B-7 DR2,DR3,DQ DR2,DR3,DQ

• ENVIRONMENT- ENVIRONMENT- • 1. 1. UV LIGHT >70 % CASES HAS PHOTOSENSITIVITY UV LIGHT >70 % CASES HAS PHOTOSENSITIVITY

• 2. DRUGS - PROCAINAMIDE, HYDRALAZINE, INH, 2. DRUGS - PROCAINAMIDE, HYDRALAZINE, INH, PHENYTOIN, MINOCYCLINE PHENYTOIN, MINOCYCLINE 3.. VIRUSES CMV, EPSTEIN- 3.. VIRUSES CMV, EPSTEIN-BARR VIRUSESBARR VIRUSES

PATHOGENESISPATHOGENESIS

CLINICAL MANIFESTATIONCLINICAL MANIFESTATION• ACLE ACLE – – SKIN LESIONS WAX & WANE IN PARALLEL WITH SKIN LESIONS WAX & WANE IN PARALLEL WITH

UNDERLYING DISEASE ACTIVITY, UNDERLYING DISEASE ACTIVITY, NO SCARRINGNO SCARRING

• LOCALISED LOCALISED - - SYMETIRCAL ERYTHEMA & EDEMA AT MALAR SYMETIRCAL ERYTHEMA & EDEMA AT MALAR EMINENCESEMINENCES

• GENERALISED - MORBILLIFORM/ GENERALISED - MORBILLIFORM/ EXANTHEMATOUS ERUOTIONSEXANTHEMATOUS ERUOTIONS

• SCLE SCLE - - PHOTOSENSITIVITY PHOTOSENSITIVITY

• ANNULAR ERYTHEMA, PSORIASIFORM , EM. ANNULAR ERYTHEMA, PSORIASIFORM , EM. EYRTHRODERMA.&EYRTHRODERMA.&

• NO SCARRING NO SCARRING

• ASSOCOATION WITH RO/SS-A ANTIBODIES & ASSOCOATION WITH RO/SS-A ANTIBODIES & MALIGNANCIES-BREAST,LUNGS,GI,HODGKIN’S MALIGNANCIES-BREAST,LUNGS,GI,HODGKIN’S DISEASE. AOTOIMMUNE DISEASESDISEASE. AOTOIMMUNE DISEASES

CCLECCLE

• DISCOID ERYTHEMATOUS PLAQUES WITH DISCOID ERYTHEMATOUS PLAQUES WITH ADHERENT SCALE & FOLLICULAR PLUGS-CARPET ADHERENT SCALE & FOLLICULAR PLUGS-CARPET TACK SIGN TACK SIGN

• HYPERPIGMENTATION AT PERIPHERY, AROPHIC HYPERPIGMENTATION AT PERIPHERY, AROPHIC CENTRAL SCARING, CENTRAL SCARING,

• TELENGIEACTASIA, TELENGIEACTASIA, • HYPOPIGMENTATION, HYPOPIGMENTATION, • SCARING ALOPECIAS, SCARING ALOPECIAS, • OCCURS AT SUN EXPOSED AREASOCCURS AT SUN EXPOSED AREAS• HYPERTROPHIC DLEHYPERTROPHIC DLE• BULLOUS LESIONSBULLOUS LESIONS• MUCOSAL – 25% OF CASESMUCOSAL – 25% OF CASES• LUPUS PANNICULITISLUPUS PANNICULITIS

AUTOANTIBODIES ASSOCATIONAUTOANTIBODIES ASSOCATIONANTIGENANTIGEN MOLECULAR MOLECULAR

SPECIFICITYSPECIFICITYCLINICAL CLINICAL ASSOCIATIONASSOCIATION

HIGH DISEASE HIGH DISEASE SPECIFICITY FOR SPECIFICITY FOR SLESLE

ANA - ANA - 90%90%

dsDNA - dsDNA - 60%60%

Sm - Sm - 25% 25%

rRNp - rRNp - 10%10%

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Native DNANative DNA

RibonucleoproteinRibonucleoprotein

Ribosomal P proteinRibosomal P protein

SLE,SLE,

LE NephritisLE Nephritis

CNS LECNS LE

LOW DISEASE LOW DISEASE SPECIFICITY FOR SPECIFICITY FOR SLESLE

ssDNA -60%ssDNA -60%

Histones - Histones - 50%50%

Ro/SS-A - Ro/SS-A - 25%25%

La/Ss-B - La/Ss-B - 25%25%

Denatured DNADenatured DNA

HistonesHistones

RibonucleoproteinsRibonucleoproteins

RibonucleoproteinsRibonucleoproteins

Risk for SLE in DLERisk for SLE in DLE

Drug induced SLEDrug induced SLE

SCLE, neonatal LE, SCLE, neonatal LE, SSjSSj

SSJ, SCLESSJ, SCLE

RISK FACTORS FOR DEVELOPMENT OF RISK FACTORS FOR DEVELOPMENT OF SLE IN PAITENT OF DLESLE IN PAITENT OF DLE

• DIFFUSE NONSARRING ALOPECIADIFFUSE NONSARRING ALOPECIA• GENERELISED LYMPHADENOPATHYGENERELISED LYMPHADENOPATHY• PERIUNGAL NAIL FOLD TELENGIACTASIAPERIUNGAL NAIL FOLD TELENGIACTASIA• RAYNAUD’S PHENOMENONRAYNAUD’S PHENOMENON• UNEXPLANED ANEMIAUNEXPLANED ANEMIA• LEUCOPENIALEUCOPENIA• FALSE POSITIVE TEST FOR SYPHILSFALSE POSITIVE TEST FOR SYPHILS• HIGH TITER OF ANAHIGH TITER OF ANA• ANTI ssDNA ANTIBODIESANTI ssDNA ANTIBODIES• ELEVETED ESRELEVETED ESR

ACUTE LEACUTE LE

BUTTER FLY BUTTER FLY

LESIONLESION

ERYTHEMA AT DORSA OF ERYTHEMA AT DORSA OF HANDHAND

SKIN LESION OF SCLESKIN LESION OF SCLE

SCLESCLE

DLEDLE

DLEDLE

DLE LESION AT PINNADLE LESION AT PINNA

DLE LESION DLE LESION

CICATRICAL ALOPECIACICATRICAL ALOPECIA

ORAL LESIONORAL LESION

HISTOPATHOLOGYHISTOPATHOLOGY

IMMUNOHISTOLOGYIMMUNOHISTOLOGY

TREATMENTTREATMENT

• LOCALLOCAL – – • SUN SCREENSSUN SCREENS• TOPICAL GLUCOCORTICOCOIDSTOPICAL GLUCOCORTICOCOIDS

• SYSTAMICSYSTAMIC• ANTIMALARIALS- ANTIMALARIALS- • CHLOROQUINCHLOROQUIN• HYDROXYCHOLROQUINHYDROXYCHOLROQUIN• DAPSONEDAPSONE• RETINOIDSRETINOIDS• CLOFAZIMINECLOFAZIMINE• SYSTAMIC GLUCOCORTICOIDSSYSTAMIC GLUCOCORTICOIDS• AZATHIOPRINEAZATHIOPRINE• CYCLOPHOSPHAMIDECYCLOPHOSPHAMIDE