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Does my patient have Lupus?

Does my patient have Lupus?. Jammie Barnes, MD Assistant professor Department of Medicine, Division of Rheumatology. It’s Lupus. http://www.youtube.com/watch?v=bueW1i9kQao. Dr. House or Dr. Warner. LBJ referral: +ANA with aches and pains Dr. Barnes: It’s Lupus Dr. Warner: Wrong

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Does my patient have Lupus?

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  1. Does my patient have Lupus? Jammie Barnes, MD Assistant professor Department of Medicine, Division of Rheumatology

  2. It’s Lupus • http://www.youtube.com/watch?v=bueW1i9kQao

  3. Dr. House or Dr. Warner • LBJ referral: +ANA with aches and pains • Dr. Barnes: It’s Lupus • Dr. Warner: Wrong • Another referral: same story • Dr. Barnes: It’s Lupus • Dr. Warner: Wrong • A retrospective chart review at LBJ (1yr) • 104 +ANA referrals…. ONLY 6 cases of confirmed SLE

  4. Objectives • Understand the limitations of sensitivity and specificity of ANA • Determine who needs to be evaluated for SLE • Describe the systemic signs and symptoms of SLE • Apply the American College of Rheumatology criteria for SLE • Apply to cases

  5. ANA is 100% sensitive Lupus Diabetes

  6. Sensitivity & Specificity PPV: 10/400 = 2.5% SnNout: high sensitivity – negative test is good at ruling out the disease Negative ANA – very unlikely to have SLE SpPin: high specificity –positive test good at ruling in disease Sensitivity – 100% Specificity – 60% PPV: 500/700 = 71.4%

  7. The nomogram Reminder: +LR= sens/(1-spec) LR: 2.5

  8. Pretest probability • Consider prevalence • Clinical scenario in your patient • If you order a test – expect a result Positive ANA, now what!!

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

  10. Other problems with ANA • 1/3 of healthy people have an ANA 1:40 • 5% of healthy people have ANA 1:160 • 3.3% of healthy people have ANA 1:320 • Healthy 1st degree relatives can have + ANA • Healthy older people increased + ANA • ANA linked to thyroid dz, hepatitis, environmental exposure, cancer, infections and drugs Southern Medical Journal. Vol 105, no 2, Feb 2012

  11. Making the ANA better • 2 possibilities • Raise the threshold of positive test • High titers do warrant more investigation > 1:1280 • Couple the test with more specific signs and symptoms of rheumatic disease • High risk - low occurrence

  12. When to order an ANA

  13. CNS/PNS • Criteria – seizures and psychosis • Both in absence of offending drugs • Question: Have you ever had a seizure or convulsion? Orphanet Journal of Rare Disease 2006 1:6

  14. Skin/Mucocutaneous • 4 criterion for skin: malar rash, discoid rash, photosensitivity and oral ulcers • Do you get sores in your mouth or nose for more than 2 weeks at a time • Rash on your cheek for more than a month • Skin breakout (rash) after being in the sun (not a sunburn) • Others: • Alopecia • Have you had rapid loss of hair • Raynauds • Have your fingers ever shown unusual color changes in the cold • Purpura, urticaria and vasculitis

  15. Hematologic • Hemolytic anemia • Leukopenia <4000 on > 2times or lymphopenia <1500 on > 2 times • Thrombocytopenia <100k in absence of drugs • All meet hematologic criteria (only get 1 point) • Questions: Have you ever been told that you have anemia, low blood count, low platelet count

  16. Cardio/Pulm • Criteria: • Pericarditis – documented by ECG, rub or pericardial effusion • Pleuritis – convincing h/o pleuritic chest pain, rub or pleural effusion • Question: Do you get chest pain with deep breath? • 1 point • Others: • Endo and myocarditis, pulmonary arterial hypertension, valvular, CAD • Chronic interstitial pneumonitis, acute lupus pneumonitis, acute alveolar hemorrhage, acute reversible hypoxemia, PE, shrinking lung syndrome

  17. Renal • Criteria: • Persistent proteinuria >0.5gm per day or 3+ on dipstick or cellular cast • Have you have been told you have protein in your urine • Class 1-6 of lupus nephritis • Microangiopathic glomerular disease • Renal vein thrombosis

  18. GI • No criteria for diagnosis • None specific abd pain, nausea and vomitting • Rare mesenteric vasculitis

  19. Reticuloendothelial • Not a criteria • LAD • HSM

  20. MSK • Criteria: • Arthritis – tenderness, swelling or effusion in 2 or more joints witnessed • Typically non-erosive • Jacoudsarthopathy • Others: • Myositis

  21. Constitutional • Not a criteria • Profound fatigue (disabling fatigue) – in absence of depression • Fever (no signs of infection) • Weight loss

  22. Immunologic • Criteria: • Positive ANA >1:80 • Positive anti-dsDNAOR Anti-Smith OR antiphospholipid antibody • AbnlIgG or IgMcardiolipin, + lupus anticoagulant, false positive RPR • Others: • SSA/B (anti-Ro and La), RNP

  23. Applying Signs and Sxs • Upon screening: • Two or more organs systems involved – order CBC, CMP, UA to evaluate for systemic disease • If above reveals possible systemic disease then order an ANA and possible other antibodies • If 4 or more criteria by ACR or suspect SLE refer to Rheumatology

  24. Case • 21 y/o college student with two months of joint pain worse in AM • Notices faint rash on face for last month • Very tired and finds it difficult to concentrate in class • Denies fevers, abd pain, chest pain, diarrhea or constipation • On exam: malar rash, decreased breath sounds at bases, no murmurs, diffuse cervical LAD and mild synovitis in the MCPs and PIPs

  25. What next • Order labs/studies: CBC, UA, CMP, CXR • What other labs do you want? • ANA, RF, CCP and TSH • WBC count 3.2, nlHgband 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 effusions • Does she meet criteria? • YES!

  26. Case • 36 y/o stay at home Mom presents with joint pains for 3 months • She has no swelling, but she has tenderness all over in the upper and lower body • She tells you she has anemia, severe fatigue but she can still take care of her children • She has occasional HA, some weight gain, but other ROS is negative • On exam she is overweight with BMI of 32, multiple tender points but no synovitis

  27. What next • Order CMP,CBC, UA and TSH • Her labs are normal with exception of HGB of 10.2 and MCV of 76 • What next: • Iron studies • Low ferritin, smear: hypochromic RBCs, low iron and high TIBC • Do you need to do more? • Treat IDA

  28. Case • 32 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 oxcarbazepine • He has developed a photosensitive rash and joint pain • In ROS he also has pleuritic chest pain • On exam he has a erythematous rash on the face and upper chest, synovitis of the bilateral wrist but rest of exam is normal

  29. What next • CBC, CMP, UA, CXR and ANA • He has positive ANA, nl CMP, CMP, UA and chest xray • What does he have? • Drug induced lupus • Do you need histone antibodies? • No • How do you proceed? • Discuss changing anti-convulsant medication, may add NSAIDs, steroid cream for rash and hydroxychloroquine

  30. Thank you for time • Remember ANA does not equal lupus • Need careful history and physical • Lupus 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!!!

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