Lupus erythematosus

1. Lupus erythematosus

2. Definition Lupus erythematosus 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.

3. Etiology genetic factors the findings of 100 LE family surveys : • Family history positive : 12 % • The prevalence of SLE in first-degree relatives is 1.84 % • The degree of heritability was 56 % In addition, the concordance rate in monozygotic twins comes to over 50% suggestion : LE is multifactorial inheritance Environmental influence high

4. Etiology Enviornmental influence • Virus infection-- other bacterial infections are seldom • Physical factor -- sunlight ,coolness, wetness, ray • Emotional factor ---stress, worry • Endocrine factor –high estrogen ,prolactin • drugs -- more than 20 drugs such as penicillin, phenytoin may induce symptomatic LE

5. Pathogenesis Environmental influence (infection, sunlight, drug) Genetic factor Immuno-modulatory function disorder Auto-antigen formed Sensitivitied T- lymphocytes Various auto antibody Immune-complexes precipitation Blood cell injured Organs injured Alterations in organs

6. Clinical manifestation • Discoid LE ←→★ Systemic LE

7. Discoid lupus erythematosus,DLE • Skin lesions are predominantly manifested • Eruptions may occur singly or be multiple • localized DLE Lesions localized on face, dorsa of the hand • generalized DLE Lesions 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

10. 录象片断

11. skin lesions in discoid LEFavorite sites are malar areas, bridge of nose, lower lip, ear, scalp and dorsa of the hand

12. 录象片断

13. Skin lesions in discoidLEatrophy 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 blister • Specific eruption: malar erythema erythema on finger pulp or toe pulp erythema 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

17. 录象片断

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 azotemia • Heart—myocarditis, pericarditis and endocarditis • Pulmo —pleural effusions, interstitial lung disease • locomotorsystem -Myositis, arthritis • Digestive system —gastroenteritis, peritonitishepatitis • Nervous system —mental disorder, central and peripheral neuritis or vascular lesions • Eye--Retina neural degeneration, conjunctivitis, subhyaloid hemorrhage • Others—Raynaud’s phenomenon, fatigue and fever, lymphadenectasis

22. Laboratory findings • Hypocytosis • Proteinuria (erythrocyturia \leukocyturia\ cylindruria) • Elevated sedimentation rate • Positive rheumatoid factor • Elevated immunoglobulin • Low level of serum complement

23. laboratory findings injured nuclear Anti-nucleoprotein antibody homogeneous body Be phagocytized by PMN Attract PMN groups ofcell LE cell

24. laboratory findings • Special test（1）LE cell( cell smear)

25. laboratory findings • Special test （2）ANA (indirect immuno-fluorescence ) （3）anti ds-DNA antibody （4）ENA(-Sm、RNP、Ro、La antibodies） (immuno-blot) （5）LBT lupus band test (direct immuno-fluorescence )

27. histology • There is hyperkeratosis in epidermis follicular orifice dilat, keratinous plug in it spinous layer atrophy liquifaction degeneration of basal cells • lymphocytes 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 scales Horny plugs Centrally atrophy Dyspigmentation Telangiectasia

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)

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.

31. Treatment • Avoid exposure to strong sunlight, to excessive cold, heat, tension , tiredness and infection • For predominant skin lesion, hydroxychloroquine, thalidomide, tripterygium wilfordii are recommended , topical application of carticosteroid • For patients whose organs involved, carticosteroid should be administered, and combinations of steroid with immunosuppressive drugs are recommended when necessary • Others : nonsteroidal anti-inflammatory agents, immuno-modulatory drugs and other supportive treatments