Does my patient have Lupus?

Does my patient have Lupus?Jammie Barnes, MDAssistant professorDepartment of Medicine, Division of Rheumatology

LBJ referral: +ANA with aches and painsDr. Barnes: Its LupusDr. Warner: WrongAnother referral: same storyDr. Barnes: Its LupusDr. Warner: WrongA retrospective chart review at LBJ (1yr)104 +ANA referrals. ONLY 6 cases of confirmed SLE

Dr. House or Dr. WarnerUnderstand the limitations of sensitivity and specificity of ANADetermine who needs to be evaluated for SLEDescribe the systemic signs and symptoms of SLEApply the American College of Rheumatology criteria for SLEApply to cases

ObjectivesANA is 100% sensitive

Diabetes LupusSensitivity & SpecificitySnNout: high sensitivity negative test is good at ruling out the diseaseNegative ANA very unlikely to have SLESpPin: high specificity positive test good at ruling in diseaseSensitivity 100%Specificity 60%

Prevalence = 10/1000 = 1%Yes- SLENo- SLE+ test10390400- test0600600109901000Prevalence = 500/1000 = 50%Yes DzNo - Dz+ test500200700- test03003005005001000PPV: 10/400 = 2.5%PPV: 500/700 = 71.4%The nomogramLR: 2.5

Reminder: +LR= sens/(1-spec)Consider prevalenceClinical scenario in your patientIf you order a test expect a resultPretest probability

Positive ANA, now what!!

Autoabs directed against DNA or snRNPPositive test: >1:80Best to order test by immunofluorescence (IF)ELISA enzyme linked assays are cheaper but have 80-98% agreement with IF ACR recommends ordering ANA by IFANA

1/3 of healthy people have an ANA 1:405% of healthy people have ANA 1:1603.3% of healthy people have ANA 1:320Healthy 1st degree relatives can have + ANAHealthy older people increased + ANAANA linked to thyroid dz, hepatitis, environmental exposure, cancer, infections and drugsOther problems with ANASouthern Medical Journal. Vol 105, no 2, Feb 20122 possibilitiesRaise the threshold of positive testHigh titers do warrant more investigation > 1:1280Couple the test with more specific signs and symptoms of rheumatic diseaseHigh risk - low occurrence Making the ANA better

When to order an ANA

Criteria seizures and psychosisBoth in absence of offending drugsQuestion: Have you ever had a seizure or convulsion?

CNS/PNS

Orphanet Journal of Rare Disease 2006 1:64 criterion for skin: malar rash, discoid rash, photosensitivity and oral ulcersDo you get sores in your mouth or nose for more than 2 weeks at a time Rash on your cheek for more than a monthSkin breakout (rash) after being in the sun (not a sunburn)Others:AlopeciaHave you had rapid loss of hairRaynaudsHave your fingers ever shown unusual color changes in the coldPurpura, urticaria and vasculitis

Skin/MucocutaneousHemolytic anemiaLeukopenia 2times or lymphopenia 2 timesThrombocytopenia 0.5gm per day or 3+ on dipstick or cellular castHave you have been told you have protein in your urineClass 1-6 of lupus nephritisMicroangiopathic glomerular diseaseRenal vein thrombosis

RenalNo criteria for diagnosisNone specific abd pain, nausea and vomittingRare mesenteric vasculitisGINot a criteria LADHSMReticuloendothelialCriteria: Arthritis tenderness, swelling or effusion in 2 or more joints witnessedTypically non-erosive Jacouds arthopathyOthers:MyositisMSKNot a criteriaProfound fatigue (disabling fatigue) in absence of depressionFever (no signs of infection)Weight lossConstitutional Criteria: Positive ANA >1:80Positive anti-dsDNA OR Anti-Smith OR antiphospholipid antibodyAbnl IgG or IgM cardiolipin, + lupus anticoagulant, false positive RPROthers:SSA/B (anti-Ro and La), RNP

Immunologic Upon screening: Two or more organs systems involved order CBC, CMP, UA to evaluate for systemic diseaseIf above reveals possible systemic disease then order an ANA and possible other antibodiesIf 4 or more criteria by ACR or suspect SLE refer to RheumatologyApplying Signs and Sxs

21 y/o college student with two months of joint pain worse in AMNotices faint rash on face for last monthVery tired and finds it difficult to concentrate in classDenies fevers, abd pain, chest pain, diarrhea or constipationOn exam: malar rash, decreased breath sounds at bases, no murmurs, diffuse cervical LAD and mild synovitis in the MCPs and PIPsCaseOrder labs/studies: CBC, UA, CMP, CXRWhat other labs do you want?ANA, RF, CCP and TSHWBC count 3.2, nl Hgb and platelets, neg RF and CCP, UA 2+ proteinuria, no cast or red cells, UPC 0.3, ANA 1:640, +dsDNA, +smith and chest xray with effusionsDoes she meet criteria? YES! What next36 y/o stay at home Mom presents with joint pains for 3 monthsShe has no swelling, but she has tenderness all over in the upper and lower bodyShe tells you she has anemia, severe fatigue but she can still take care of her childrenShe has occasional HA, some weight gain, but other ROS is negativeOn exam she is overweight with BMI of 32, multiple tender points but no synovitisCaseOrder CMP,CBC, UA and TSHHer labs are normal with exception of HGB of 10.2 and MCV of 76What next: Iron studiesLow ferritin, smear: hypochromic RBCs, low iron and high TIBCDo you need to do more?Treat IDAWhat next32 y/o man with long standing history of epilepsy. He has been on anti-seizure medication for many years. Initially he was on phenytoin and now on oxcarbazepineHe has developed a photosensitive rash and joint painIn ROS he also has pleuritic chest painOn exam he has a erythematous rash on the face and upper chest, synovitis of the bilateral wrist but rest of exam is normalCaseCBC, CMP, UA, CXR and ANAHe has positive ANA, nl CMP, CMP, UA and chest xrayWhat does he have?Drug induced lupusDo you need histone antibodies?NoHow do you proceed?Discuss changing anti-convulsant medication, may add NSAIDs, steroid cream for rash and hydroxychloroquine

What nextRemember ANA does not equal lupusNeed careful history and physicalLupus is RARE disease but high morbidity and mortality if missed

Please remember your packet!!I need to contact you again in 3months for post test!!!Thank you for time