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SYSTEMIC LUPUS ERYTHEMATOSUSBy Maureen Charagu

Facilitator Dr. Daniel Zuriel-DermatopathologistUniversity of Nairobisystemic lupus erythematosus by Maureen Charagu1

Case presentationHistory of X28 years female1 month history of intermittent fever joint pains puffiness of her face, hands and feet malar rash which was more severe on exposure to sunlight. intermittent abdominal pain chest pain. bleeding of her gums after brushing her teeth bruising of her skinsystemic lupus erythematosus by Maureen Charagu2Physical examinationenlarged non-tender lymph nodes and spleen. A faint pericardial friction rub was present.Patient was on Prednisolone for treatment.systemic lupus erythematosus by Maureen Charagu3Lab investigations urine revealed moderate to severe protenuria &red and white cells in the sedimentserum hypoprotenemia, a reversed albumin/ globulin ratio, hypocomplementemia, and antinuclear antibodies (ANAs) to double stranded DNACBC- low numbers platelet, neutrophil and normochromic normocytic anaemia. LE cells were seen in a special preparationsystemic lupus erythematosus by Maureen Charagu4systemic lupus erythematosus by Maureen Charagu5

OBJECTIVES Definition of SLE- autoimmune dx Causes and pathogenesisMorphology and Clinical PictureInvestigationsTreatment

6systemic lupus erythematosus by Maureen CharaguDefinition a multi-system auto-immune disease that is caused by tissue damage resulting from autoantibodies against selfantigens and formation of immunecomplexes7systemic lupus erythematosus by Maureen CharaguPredisposing factorsGeography. Race. Sex. Affects 1 in 700 women of the child bearing age.Age- high incidence 20-29 yearsFamily historyBlood group studies by Baart De La Faille

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Its a disease of the young and oldWHAT CAUSES LUPUS? Infectious agentsDrugsUVTraumaToxins and dietImmune regulation dysfunction tolerance $ ANAsHormonal imbalanceGenetic susceptibility- HLA-DQ locus9systemic lupus erythematosus by Maureen Charagu

9Lupus is an extremely complex disease, and although scientists are making progress in understanding the causes of lupus, there is still no single known cause. It is thought that a combination of genetics, environment, and possibly hormones act together to trigger the disease. Genetics: There is considerable evidence indicating that genes play a major role in the disease process. Researchers believe that there may be as many as 100 genes, which contribute to the genetic predisposition and development of SLE, and they have recently discovered a single gene that causes a lupus-like illness in mice.Hormones: The effect of hormones in humans with lupus is not clear. However, because the majority of lupus patients are women in their childbearing years, it seemed a logical aspect to study. Female hormones tend to stimulate the immune system or promote an immune response, (remember that having lupus means having an overactive immune system), whereas male hormones have the opposite effect and are more immunosuppressive. There does not seem to be any evidence that men or women with lupus produce abnormal levels of hormones, however, there may be differences in the way people with lupus process these hormones.Environment: Although it has not yet been fully proven, there may be certain environmental factors that play a role in initiating or triggering lupus in a genetically predisposed person. Maureen Charagu H31/36132/20101/18/2015systemic lupus erythematosus by Maureen Charagu10

pathogenesisDIAGNOSIS of SLEACR cRITERIASkin: Malar Rash Discoid rashOral ulcersPhoto sensitive rash

Systemic CriteriaArthritisSerositisKidney DisorderNeurologic DisorderHematolic Disorder

Laboratory CriteriaImmunologic DisorderPositive ANA Test

systemic lupus erythematosus by Maureen Charagu11

systemic lupus erythematosus by Maureen Charagu12

photosensitivityOral ulcer

Bulous eruption

LE cella neutrophil or macrophage that has phagocytized (engulfed) the denatured nuclear material of another cellmorphologyKidney WHO ClassificationClass Normal on LMMesangial lupus GNFocal proliferative GNDiffuse Proliferative GNMebranous GN

systemic lupus erythematosus by Maureen Charagu13Mesangial lupusSlight increase in mesangial matrix and cellularity

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Focal proliferative GNDiffuse proliferative GNMembranous GNEntire glomerulus has hypercellularityIncreased deposition of basement membraneLocalised proliferation of the endothelium Characterised by hematuria and protenuria- severity depends on classCVSEndocardial Libmanns and sacks verrucous vegetations on both surfaces of any of the valves, May be superimposed with bacterial endocarditisMyocarditisPericardium fibrinoid deposition with LE bodiessystemic lupus erythematosus by Maureen Charagu1515Maureen Charagu H31/36132/20101/18/2015

16systemic lupus erythematosus by Maureen Charagu

systemic lupus erythematosus by Maureen Charagu17

Superficial and deep perivascular lymphocyte infiltrateInjury to the blood vessels that supply the retina. Retinal hemorrhages= reduced blood supply in:Macula= loss of central visionOptic nerve= blindness

Cont..CNS- Vascular lesion= ischaemia/ multifocal cerebral microinfarctsLung- pleuritis & Pleural EffussionSpleen moderately enlargedLymph nodes enlarged(edema &sinus hyperplasia) are nontenderJoints- synovitisGIT- Ulceration & arteritis (bleeding gums, Abd pain)Acute necritizing vasculitis

systemic lupus erythematosus by Maureen Charagu18investigationsScreening- ANAs (antibodies to DNA,histones etc)CBC (RBC, WBC, platelets)UrinalysisSedimentation Rate (ESR)Rheumatoid FactorSkin biopsyKidney Biopsy

systemic lupus erythematosus by Maureen Charagu19EFFECT OF LABORATORY TESTS WITH INCREASED LUPUS ACTIVITYh C reactive protein (CRP)h Sedimentation rate (ESR)h Anti DNAh Liver and Kidney Function tests h CPKh Urine protein or cell casts i CBC (WBC, RBC, platelets)i Complementi Serum albumin 20systemic lupus erythematosus by Maureen Charagu20An increase in lupus activity usually causes the following test results to riseCRP, sed rate, anti DNA, liver and kidney function tests (AST, ALT, BUN, Creatinine- with kidney or liver involvement), CPK (if muscle involvement is present), urine protein or cellular casts.An increase in lupus activity usually causes the following test results to fall: CBC (WBC, RBC, platelets), complement, serum albumin. COMMON LUPUS MEDICATIONS NSAIDs-ASA, ibuprofen, indomethacin Antimalarials-Chloroquine Corticosteroids- Hydrocortisone, prednosolone, Prednisone, Methylprednisolone Immunosuppressantscyclphosphamide, Azathioprine 21systemic lupus erythematosus by Maureen Charagu21Medications play an important role in the care of people with SLE. For most people with lupus, effective treatment can minimize symptoms, reduce inflammation, and maintain normal body functions. Treatment approaches are based on the specific needs and symptoms of each person. The medication prescribed usually depends on which organ(s) are involved and the severity of the involvement. The choice of drugs is highly individualized and typically changes often during the course of the disease. The medications used in the management of lupus include: NSAIDs Antimalarials Corticosteroids Immunosuppressants Investigational (research)

Maureen Charagu H31/36132/20101/18/2015did you know? systemic lupus erythematosus by Maureen Charagu22

Toni BraxtonNick CanonMichael JacksonLady Gaga has borberline LupusPeople living with lupus lets find a cure.systemic lupus erythematosus by Maureen Charagu23

systemic lupus erythematosus by Maureen Charagu24reference: Dubois lupus erythematosus by- daniel j. Wallacebevra hannahs hahn