2 DefinitionLupus erythematosusLE is classified as connective tissue disease. It occurs predominantly in females in the reproductive years. Multiple abnormal immune responses are present.It may involved any organ of the body and is manifested in many forms. It may be life-threatening when it becomes severe.
3 Etiology suggestion : the findings of 100 LE family surveys : genetic factorsthe findings of 100 LE family surveys :Family history positive : %The prevalence of SLE in first-degree relatives is %The degree of heritability was 56 %In addition, the concordance rate in monozygotic twins comes to over 50%suggestion :LE is multifactorial inheritanceEnvironmental influence high
4 Etiology Enviornmental influence Virus infection-- other bacterial infections are seldomPhysical factor -- sunlight ,coolness, wetness, rayEmotional factor ---stress, worryEndocrine factor –high estrogen ,prolactindrugs -- more than 20 drugs such as penicillin, phenytoin may induce symptomatic LE
5 Pathogenesis Environmental influence (infection, sunlight, drug) Genetic factorImmuno-modulatory function disorderAuto-antigen formedSensitivitied T- lymphocytesVarious auto antibodyImmune-complexes precipitationBlood cell injuredOrgans injuredAlterations in organs
6 Clinical manifestation Discoid LE ←→★ Systemic LE
7 Discoid lupus erythematosus, DLE Skin lesions are predominantly manifestedEruptions may occur singly or be multiplelocalized DLELesions localized on face, dorsa of the handgeneralized DLELesions widespread on trunk ,extremities
8 Skin lesions in discoid LE Red macules or papules associated with edema→→ discoid eyrthematosus with adherent scales, horny plugs—-centrally atrophy, dyspigmentation, telangiectasia
13 Skin lesions in discoid LE atrophy in the scalp → false alopecia areata lesions on low lip may be eroded
14 Systemic lupus erythematosus SLE may involved any organ of the body and is manifested in many forms. It will be life-threatening when severely.Basic pathology alteration shows necrotizing vasculitis
15 Skin and mucous membrane lesions Skin involvement occurs in 80% of cases.It is often the earliest abnormality and predominant symptom.Sunlight may develop the facial eruption and a severe relapse.Mulity form : erythema, papule, blood blisterSpecific eruption:malar erythemaerythema on finger pulp or toe pulperythema around the nail fold
16 Skin and mucous membrane lesions Malar erythema --red macules associated with edema, begin on the malar areas and bridge of the nose, then spread on the ear, tip of the nose, upper eyelids
18 Finger pulp or toe pulp show prunosus macule associated with edema, telangiectases or hemorrhagic, atrophy,necrosis. they may also occur on palms, soles
19 Skin and mucous membrane lesions Eruptions widespread the body may be vesicle, petechia, nodule and livedo reticularis, erythema multiform-liked eruptions
20 Skin and mucous membrane lesions Oral mucosal erythema--erosions, shallow ulcerations occur commonly on lip, buccal mucosa, the tongue, palatine mucosa,, gums of the teeth .
21 Other internal organs involvement Renal –nephritic or nephrotic—chronic renal insufficiency with azotemiaHeart —myocarditis, pericarditis and endocarditisPulmo —pleural effusions, interstitial lung diseaselocomotor system -Myositis, arthritisDigestive system —gastroenteritis, peritonitis hepatitisNervous system —mental disorder, central and peripheral neuritis or vascular lesionsEye--Retina neural degeneration, conjunctivitis, subhyaloid hemorrhageOthers—Raynaud’s phenomenon, fatigue and fever, lymphadenectasis
27 histology There is hyperkeratosis in epidermis follicular orifice dilat, keratinous plug in itspinous layer atrophyliquifaction degeneration of basal cellslymphocytes and plasma cell infiltrate in dermis specially around the appendages of the skin and vascellum
28 Diagnose DLE depended on typical eruptions Discoid erythematosus Adherent scalesHorny plugsCentrally atrophyDyspigmentationTelangiectasia
29 1982 Revised American Rheumatism Association Criteria for the diagnosis of SLE（1） 1)Malar erythema2)discoid LE3)Photosesitivity4)Oral ulcer5)Nonerosive arthritis6)Serositis (pericarditis or pleurisy)7)Nephropathy (albuminuria or cellular casts)8)CNS disorder (unexplained seizures or psychosis)
30 1982 Revised American Rheumatism Association Criteria for the diagnosis of SLE （2） 9)Hematologic disorder (hemolytic anemia with reticulosis, or leucopenia below 4000 on two occasions, or lymphopenia below 1500 on tow occasions)10)Immunologic disorder: positive LE-cell preparation, or antibody to native DNA or SM antigen, or false positive STS11)Antinuclear antibody in abnormal titer, unexplainedA patient may be said to have SLE if four or more criteria are satisfied.
31 TreatmentAvoid exposure to strong sunlight, to excessive cold, heat, tension , tiredness and infectionFor predominant skin lesion, hydroxychloroquine, thalidomide, tripterygium wilfordii are recommended , topical application of carticosteroidFor patients whose organs involved, carticosteroid should be administered, and combinations of steroid with immunosuppressive drugs are recommended when necessaryOthers : nonsteroidal anti-inflammatory agents, immuno-modulatory drugs and other supportive treatments