系统性红斑狼疮.ppt

1、Curriculum Vitae,E-mail: EDUCATION 2011-2012:The University of Melbourne, Australia. Visiting Academic Researcher 2004-2007: Peking University, PhD. MD 1996-1999: Guangxi Medical University, M.S. degree 1987-1992: Guangxi Medical University, B.Sc. degree PROFESSIONAL EXPERIENCE 2009-present: Profess

2、or, Department of Dermatology, the First Affiliated Hospital of Guangxi Medical University 2004-2009: Associate Professor, Department of Dermatology, the First Affiliated Hospital of Guangxi Medical University 1997-2004: Attending physician, Department of Dermatology, the First Affiliated Hospital o

3、f Guangxi Medical University 1992-1997: Resident, Department of Dermatology, the First Affiliated Hospital of Guangxi Medical University MAJOR INTEREST Medical Mycology, Therapy and Diagnosis,Lupus Erythematosus,Cunwei Cao Professor,Dermatology and Venereolog DepartmentGuangxi Medical University Fir

4、st Hospital,What is Lupus Erythematosus?,Lupus Erythematosus (LE) is a chronic inflammatory, immune mediated collagen vascular disorder with multisystem involvement. The cutaneous presentation in LE may be the primary expression or part of this multisystemic disease. LE is perceived to form a spectr

5、um with discoid lupus erythematosus (DLE) and systemic lupus erythematosus (SLE) as its polar expressions. Sub acute cuntaneous lupus erythematosus (SCLE) is intermediate in severity between DLE and SLE. Can affect any part of the body, skin, joints, kidneys, lungs, blood vessels, heart, liver, and

6、nervous system.,Topics of Discussion,Pathogenesis Clinical features Diagnosis Treatment Other types of Lupus,PATHOGENESIS,Specific cause of LE is unknown. Multiple associated factors proposed - genetic, racial, hormonal and immune interact, drug and environmental factors.,Discoid Lupus Erythematosus

7、,Discoid lupus erythematosus (DLE) is the name of an eruption. DLE generally occurs in young adults, with women outnumbering men 2:1. DLE can be localized or disseminated distribution. Distribution: the sun-exposed area, usually localized above the neck. Favored sites are the scalp, bridge of the no

8、se, malar areas, lower lip,and ears.,Flare up during spring and summer. In may patients, the skin is the only organ involved. However, patients with SLE may have DLE eruption. Skin lesions are discrete, erythematous plaques with well formed adherent scales that extend into the dilated, patulous,hair

9、 follicles. Removal of the overlying scales, could see the characteristic “carpet tack” or langue de chat (cats tongue) sign undersurface of the scale. The lesions heal with atrophy, scarring, telangiectasia and central depigmentation.,Patients may complain of mild pruritus or occasional pain within

10、 the lesions, but most patients are asymptomatic. Arthralgia or arthritis may occur. Approximately 5% or fewer DLE patients have accompanying systemic involvement. Patients may manifest any symptom of SLE; therefore, the history should include an assessment for symptoms.,Discoid Rashes,DLE,Diagnosis

11、 for DLE,Affects skin, hair or mucus membranes Identified by rash or lesions Diagnosed by biopsy of rash 10% will evolve into SLE Treatment involves topical or intra-lesional steroids, antimalarials.,Sub-acute cutaneous lupus erythematosus (SCLE),Not uniformly associated with SLE 50% of affected pat

12、ients have SLE , 10% of SLE patients have this type of skin lesion Present as two types of skin lesions : Annular polycyclic erythema with tendency to the color degradation in the center or psoriasiform eruptions, with less scales, mild follicular plugging, healing without scarring and recurrent. Ha

13、ve a high incidence of photosensitivity, most frequently affected areas : Shoulders, forearm, neck and upper thorax. the face is usually spared. Strongly associated with Anti-Ro (SS-A) & Anti-La (SS-B) Abs,What is SLE?,SLE is a systemic autoimmune disease in which the immune system attacks the body

14、cells and tissues which results in inflammation and tissue damage. Type 3 hypersensitivity reaction in which antibody-immune complexes participate and cause further immune responses. Charecterized by remissions and exacerbations. Can affect any part of the body, most often harms the heart, joints, s

15、kin, lungs, blood vessels, liver , kidneys and nervous system.,PREVALENCE,Male : Female ratio 1:9 Higher prevalence among Asian, Afro-Americans, Afro-caribbeans and Hispanic Americans. Age of onset : 30 (females), 40 (men) Increased frequency among women has been attributed in part to an estrogen ho

16、rmonal effect.,PRECIPITATING FACTORS,Family history (5%) Sunlight (UVR) - most effective precipitating factor Drugs - Hydralazine, procainamide and anti-convulsants. Note : Rash is a relatively uncommon feature of drug induced SLE.,CLINICAL FEATURES,How do patients present?,90% malaise and fatigue 9

17、0% arthralgia and myalgias 70% photosensitivity/rashes 57% fever 50% arthritis 44% pleuritis 40% alopecia 25% raynauds 25% hypertension 20% oral ulcerations,SKIN FINDINGS,The “butterfly” rash,Classic Malar rash with sparing of nasolabial folds. Important differential diagnosis : Rosacea, Seborrheic

18、dermatitis,SLE,Acute Cutaneous: Malar Rash Note Sparing of Nasolabial Folds,Chronic Cutaneous: Discoid Note Scarring, Hyperpigmentation,Red to violaceous, well demarcated papules and plaques on the dorsa of fingers , charecteristically sparing the skin overlying the joints. This is an important diag

19、nostic sign when considering DERMATOMYOSITIS, which charecteristically involves the skin over the joints.,Nailfold telangiectasias, microthrombi, erythema and mild edema of periungual skin,PALMAR ERYTHEMA,Oral Lesions of SLE,Erythema of hard and soft palate, papules ,vesicles and petechiae Erythemat

20、ous rash of the tongue.,Classically Painless ulcerations, occuring on the hard palate,Raynauds phenomenon,Lupus Hair,Thin hair that easily fractures Usually occurs along the frontal hairline Is associated with disease activity Grows back normally as disease subsides.,Before therapy,After therapy,SLE

21、 patient with Alopecia,NOTE : Scarring Alopecia is a complication in DLE.,LIVIDO RETICULARIS,Diagnosing Lupus,Medical History (Including family history) Complete physical examination Laboratory tests Skin or kidney biopsy,REMEMBER DIAGNOSTIC CRITERIA : “ SOAP BRAIN MD”,ACR DIAGNOSTIC CRITERIA FOR SL

22、E,A person shall be said to have SLE if any 4 or more of the 11 criteria are present , serially or simultaneously , during any interval of observation.,High specificity for SLE,dsDNA - Useful in monitoring the disease activity in SLE Sm Antibody rRNP,Low specificity for SLE,ANA ssDNA - Possible risk

23、 for SLE in DLE pts , Also seen in RA, DM, MCTD, SS, SjS , localized scleroderma C1q - Severe SLE , Urticarial vasculitis U1RNP - Overlapping features with other CTD , MCTD (100%) Ro (SS-A) Cardiolipin Histones Beta 2 glycoprotein 1 RA La (SS-B),Investigations,General investigations: TC, DC, Hb, PLT

24、, ESR Urea, creatinine URE for protein , RBC and cellular casts 24 hours urine test for creatinine clearance and protein excretion,Tests of SLE Disease activity:,Anti-dsDNA, Complement determinations (C3,C4) , CRP Anti-nucleosome antibodies, anti-C1q antibodies,ECHOCARDIOGRAPHY,Noninfective thrombot

25、ic endocarditis involving mitral valve in SLE. Note nodular vegetations along line of closure and extending onto chordae tendineae (libman_sacks syndrome).,Other Tests,Radiography CT scan - To rule out interstitial lung disease, pneumonitis, pulmonary emboli, alveolar hemorrhage MRI Brain - CNS lupu

26、s white matter changes, vasculitis, stroke. Lumbar puncture & CSF study Skin biopsy,LUPUS BAND TEST,Microphotograph of histologic section of human skin prepared for Direct immunofluorescence using an Anti-IgG antibody,Treatment : Patient Education,1. Avoidance sun 2. Use of SPF 35 sunblocks UVA and

27、UVB 3. Sun-protective clothing 4. Promote exercise 5. Healthy diet (low chol, low sugar, low salt) 6. Smoking cessation,Treatment : Anti-malarials,Hydroxychloroquine, quinacrine (available compounded), chloroquine Prevent activation of toll like receptors 7 & 9 Used in lupus over 50 years FDA approved indication Mildest immunosuppressant Very safe, risk of retinal toxicity low Eye exam once yearly,Hydroxychloroqine,Takes 6 weeks to kick in, up to 6 months for maximal effect Reduces intensity of flares Increases time t