of 31/31
Lupus erythem Lupus erythem atosus atosus

Lupus erythematosus. Definition Lupus erythematosus Lupus erythematosus LE is classified as connective tissue disease. It occurs predominantly in females

  • View
    220

  • Download
    4

Embed Size (px)

Text of Lupus erythematosus. Definition Lupus erythematosus Lupus erythematosus LE is classified as...

  • Slide 1

Lupus erythematosus Slide 2 Definition Lupus erythematosus Lupus erythematosus LE is classified as connective tissue disease. It occurs predominantly in females in the reproductive years. Multiple abnormal immune responses are present. LE 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. It may involved any organ of the body and is manifested in many forms. It may be life-threatening when it becomes severe. Slide 3 Etiology genetic factors genetic factors the findings of 100 LE family surveys : l Family history positive : 12 % l The prevalence of SLE in first-degree relatives is 1.84 % l The degree of heritability was 56 % In addition, the concordance rate in monozygotic twins comes to over 50% In addition, the concordance rate in monozygotic twins comes to over 50% suggestion : LE is multifactorial inheritance LE is multifactorial inheritance Environmental influence high Environmental influence high Slide 4 Etiology Etiology Enviornmental influence l Virus infection-- other bacterial infections are seldom l Physical factor -- sunlight,coolness, wetness, ray l Emotional factor ---stress, worry l Endocrine factor high estrogen,prolactin l drugs -- more than 20 drugs such as penicillin, phenytoin may induce symptomatic LE Slide 5 Pathogenesis Genetic factor Immuno-modulatory function disorder Various auto antibody Blood cell injured Immune-complexes precipitation Environmental influence (infection, sunlight, drug) Auto-antigen formed Organs injured Alterations in organs Sensitivitied T- lymphocytes Slide 6 Clinical manifestation l Discoid LE Systemic LE Slide 7 Discoid lupus erythematosus, DLE Discoid lupus erythematosus, DLE l Skin lesions are predominantly manifested l Eruptions may occur singly or be multiple l localized DLE Lesions localized on face, dorsa of the hand Lesions localized on face, dorsa of the hand l generalized DLE Lesions widespread on trunk,extremities Lesions widespread on trunk,extremities Slide 8 Skin lesions in discoid LE Red macules or papules associated with edema discoid eyrthematosus with adherent scales, horny plugs -centrally atrophy, dyspigmentation, telangiectasia Red macules or papules associated with edema discoid eyrthematosus with adherent scales, horny plugs -centrally atrophy, dyspigmentation, telangiectasia Slide 9 Slide 10 F Slide 11 skin lesions in discoid LE Favorite sites are malar areas, bridge of nose, lower lip, ear, scalp and dorsa of the hand Slide 12 F Slide 13 Skin lesions in discoid LE atrophy in the scalp false alopecia areata lesions on low lip may be eroded Slide 14 Systemic lupus erythematosus l SLE may involved any organ of the body and is manifested in many forms. It will be life-threatening when severely. l Basic pathology alteration shows necrotizing vasculitis Slide 15 Skin and mucous membrane lesions Skin involvement occurs in 80% of cases. Skin involvement occurs in 80% of cases. It is often the earliest abnormality and predominant symptom. It is often the earliest abnormality and predominant symptom. Sunlight may develop the facial eruption and a severe relapse. Sunlight may develop the facial eruption and a severe relapse. Mulity form : erythema, papule, blood blister Mulity form : erythema, papule, blood blister Specific eruption: Specific eruption: malar erythema malar erythema erythema on finger pulp or toe pulp erythema on finger pulp or toe pulp erythema around the nail fold erythema around the nail fold Slide 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 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 Slide 17 F Slide 18 Finger pulp or toe pulp show prunosus macule associated with edema, telangiectases or h, atrophy,necrosis. they may also occur on palms, soles Finger pulp or toe pulp show prunosus macule associated with edema, telangiectases or h emorrhagic, atrophy,necrosis. they may also occur on palms, soles Slide 19 Skin and mucous membrane lesions Eruptions widespread the body may be vesicle, petechia, nodule and livedo reticularis, erythema multiform-liked eruptions Eruptions widespread the body may be vesicle, petechia, nodule and livedo reticularis, erythema multiform-liked eruptions Slide 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. Oral mucosal erythema--erosions, shallow ulcerations occur commonly on lip, buccal mucosa, the tongue, palatine mucosa,, gums of the teeth. Slide 21 Other internal organs involvement l Renal nephritic or nephroticchronic renal insufficiency with azotemia l Heart myocarditis, pericarditis and endocarditis l Pulmo pleural effusions, interstitial lung disease l system -Myositis, arthritis l locomotor system -Myositis, arthritis l Digestive system hepatitis l Digestive system gastroenteritis, peritonitis hepatitis l Nervous system mental disorder, central and peripheral neuritis or vascular lesions l l Eye--Retina neural degeneration, conjunctivitis, subhyaloid hemorrhage l OthersRaynauds phenomenon, fatigue and fever, l OthersRaynauds phenomenon, fatigue and fever, lymphadenectasis Slide 22 Laboratory findings l Hypocytosis l Proteinuria (erythrocyturia \leukocyturia\ cylindruria) l Elevated sedimentation rate l Positive rheumatoid factor l Elevated immunoglobulin l Low level of serum complement Slide 23 laboratory findings injured nuclear injured nuclear Anti- nucleoprotein antibody Attract PMN groups of cell LE cell Be phagocytized by PMN homogeneous body Slide 24 laboratory findings Special test 1 LE cell( cell smear) Special test 1 LE cell( cell smear) Slide 25 laboratory findings Special test Special test 2 ANA (indirect immuno-fluorescence ) (indirect immuno-fluorescence ) 3 anti ds-DNA antibody 4 ENA(-Sm RNP Ro La antibodies (immuno-blot) (immuno-blot) 5 LBT lupus band test (direct immuno-fluorescence ) (direct immuno-fluorescence ) Slide 26 Slide 27 histology l There is hyperkeratosis in epidermis plug in it follicular orifice dilat, keratinous plug in it spinous layer atrophy liquifaction degeneration of basal cells l lymphocytes and plasma cell infiltrate in dermis specially around the and vascellum l lymphocytes and plasma cell infiltrate in dermis specially around the appendages of the skin and vascellum Slide 28 Diagnose l DLE depended on typical eruptions depended on typical eruptions Discoid erythematosus Discoid erythematosus Adherent scales Adherent scales Horny plugs Horny plugs Centrally atrophy Centrally atrophy Dyspigmentation Dyspigmentation Telangiectasia Telangiectasia Slide 29 1982 Revised American Rheumatism Association Criteria for the diagnosis of SLE 1 1)Malar erythema 2)discoid LE 3)Photosesitivity 4)Oral ulcer 5)Nonerosive arthritis 6)Serositis (pericarditis or pleurisy) 7)Nephropathy (albuminuria or cellular casts) 8)CNS disorder (unexplained seizures or psychosis) Slide 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 STS 11)Antinuclear antibody in abnormal titer, unexplained A patient may be said to have SLE if four or more criteria are satisfied. Slide 31 Treatment l Avoid exposure to strong sunlight, to excessive cold, heat, tension, tiredness and infection l For predominant skin lesion, hydroxychloroquine, thalidomide, tripterygium wilfordii are recommended, topical application of carticosteroid l For patients whose organs involved, carticosteroid should be administered, and combinations of steroid with immunosuppressive drugs are recommended when necessary l Others : nonsteroidal anti-inflammatory agents, immuno-modulatory drugs and other supportive treatments