CASE 5 CLINICAL PATHOLOGY Palana, C Palma, S Panganiban, A Paredes, C Pasana, C
Dec 19, 2015
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
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