CASE 5CLINICAL PATHOLOGYPalana, CPalma, SPanganiban, AParedes, CPasana, C

Objectives To establish a definition of SLETo introduce some background epidemiologyTo recognise the clinical features of SLE & the clinical features of Lupus NephritisTo develop an awareness of the drug therapy related to SLE

LupusLupus is an auto-immune disease in which body creates antigens that attack different body tissuesThere are three types of Lupus DiscoidSystemicDrug-induced

Types of LupusSystemic Lupus most common, it affects major organs, and can be fatal

Discoid Lupus affects only the skin. It is not fatal, but can cause severe scarring, and may develop into Systemic Lupus if not treated

Drug-induced Lupus systemic lupus caused by certain medications. When the medicine is stopped, the disease goes away

Systemic Lupus Erythematosus - SLEAn inflammatory multi-system disease of unk etiology w/ variable clinical and lab featuresConnective tissue disease that mainly affects the blood, joints, skin, and kidneysOccurs predominantly in women of childbearing age

Who gets SLE?80 90% are women of child bearing agePeople of African, Native American, Hispanic, and Asian descent are more likely to get Lupus

Classification for SLE SOAP BRAIN MD

LUPUS CRITERIAFor the purpose of identifying patients in clinical studies, a person has SLE if 4 or more of the 11 criteria are present, serially or simultaneously, during any interval of observationIt is important to remember that a patient may have SLE and not have 4 criteria

Raynauds PhenomenonReversible skin color change (white blue red)Induced by cold or stressEpisodes of transient digital ischemiaRepresent vasoconstriction of digital vessels Often, the associated disease is CTDSource: http://www.nlm.nih.gov/medlineplus/ency/images/ency

MALAR RASHManifested reddish discoloration over her cheeks and bridge on the noseMalar rash Ig and complement a the dermal-epidermal junction: lupus band test

CUTANEOUS Oral ulcersAlopecia common yet less specific feature of SLE that often affects the temporal regions or creates a patchlike pattern of hair loss Photosensitivity a skin rash as a result of unusual reaction to sunlight

RENALRenal involvement is commonAbnormal urinalysisProteinuriaHematuria

Clinical FeaturesClassical photosensitive butterfly rashRaynauds phenomenon Profound tirednessArthralgia or even arthritis? AlopeciaOral ulcers less common than in adults

PATHOLOGYHyperreactivity and Hypersensitivity of immune systemPredisposing genesHLA II DR (DR2, DR3) and DQHLA III C2 and C4HLA-B8Polyclonal B-cell activation, sustained estrogen activity, environmental triggers (e.g. sun, procainamide)

CLINICAL FINDINGSMusculoskeletal earliest manifestationSmall-joint inflammation (e.g. hands) with absence of joint deformityMorning stiffness last minutes Hematologic Autoimmune hemolytic anemiaThrombocytopeniaLeukopeniaLymphatic Generalized painful lymphadenopathy Splenomegaly

CLINICAL FINDINGSSkinImmunocomplex deposition along basement membrane Produces liquefactive degeneration Malar butterfly rashRenal Diffuse proliferative glomerulonephritis most common glomerulonephritis CardiovascularFibrinous pericarditis with or without effusionLibman-Sacks endocarditis (sterile vegetations on mitral valve)

CLINICAL FINDINGS Respiratory Interstitial fibrosis of lungsPleural effusion with friction rubPregnancy-relatedComplete heart block in newbornsCaused by IgG anti-SS-A (Ro) antibodies crossing the placenta Recurrent spontaneous abortionsCaused by antiphospholipid antibodies

Drug-induced lupus erythematosus Associated drugsProcainamideHydralazine Features that distinguish drug-induced lupus from SLEAnti-histone antibodiesLow incidence of renal and CNS involvementDisappearance of symptoms when the drug is discontinued

BLOOD ABNORMALITIESLeukocytopenia (

AUTO ANTIBODIESThe ANA test is the best screening test for SLE and should be performed whenever SLE is suspectedThe ANA is positive in significant titer (usually 1:160 or higher) in virtually all patients with SLE

Immune AbnormalitiesSmith antibodyAnti-ds DNA antibodyAnti-phospholipid antibodyAnti-cardiolipin antibody (IgG or IgM)Biologic fallse positive VDRL (> 6 months)Lupus anticoagulant

Significant of Autoantibodies in SLE

TreatmentNSAIDSAntimalarials hydroxycholoquineCorticosteroidsImmunosuppressive agentsCyclophosphamideMethotrexateAzathiopreneMycophenolate

TreatmentHematopoietic stem cell transplantationImmunoablation aloneRituximab cyclophosphamide glucocorticoids DHEAIV Immunoglobulin

CASE 5-THANK YOU-Palana, CPalma, SPanganiban, AParedes, CPasana, C