A nice girl with swollen joints
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A nice girl with swollen joints

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A nice girl with swollen joints

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1. ?A nice girl with swollen joints? Andrea Ramirez, MD Pediatric Rheumatology Fellow September 3, 2008

2. Welcome interns! Congratulations on surviving your first 2 months of internship!

3. Goals Discuss pediatric rheumatology opportunities Discuss 2 clinical cases Review rheumatology labs that are commonly ordered ? and you thought we were just blood suckers! What to think about when you are on call

5. Opportunities for those interested 1 month elective Excellent examination skills Learn how properly examine a joint Superb history taking skills Expand your differential Improve your board scores Become a better doctor 3 year fellowship exists

6. Case 1 11 year old African American girl with a FHX of aunt with systemic lupus erythematosus (SLE) Patient evaluated in PCP?s office during well child visit and had serologic testing performed, including an Anti-nuclear antibody (ANA)

7. Case 1 Patient?s exam and history is benign Questions to the audience: What is this PCP and/or family worried about? Would you send an ANA in this patient? What are the percentages of a positive ANA in the general population?

8. ANA Can be positive in : - normal person ? up to 30 % - other autoimmune diseases - chronic liver disease - neoplasms - TB, malaria, SBE, EBV, HIV

9. Case 2 13 year old Hispanic female, post-menarche Previously well until 2 months ago when she started developing pain and swelling in bilateral wrists and fingers She is unable to open bottled water and having difficulty holding the jug of orange juice in the morning

10. What symptom is this patient having? ARTHRITIS

11. Arthritis as Sign and Symptom Metabolic Infectious/post-infectious Neoplastic Trauma Collagen Vascular/ rheumatology Autoimmune Neurology/ nephro, GI Drugs/ meds Y- other, misc

12. Case 2 If only it were the joints ? 10 lb weight loss (unintentional) Low grade fevers over the last 2 weeks up 100.6 Fatigue Sleeping with an extra pillow for the previous 2 nights

13. Case 2 What is your differential? What other questions might be important? What would you look for on physical exam?

14. Case 2 PE: Vital signs: T 99 degrees F, HR 130, BP 138/70, RR 28, W 109 lbs, H 5?2 Pale and tired appearing Thin hair frontally with a few patches of hair missing which patient has gracefully been covering up

16. Case 2 No malar rash Large palatal ulcer (non-painful) Mildly tachypneic, no nasal flaring except when lying flat Tachycardic, distant heart sounds, S1, S2 present, gallop?, no rub Hepatosplenomegaly Bilateral wrist swelling with pain on range of motion

17. OK doctor what next? Labs?

18. Labs CBC Cytopenias (lymphopenia, anemia, thrombocytopenia) Chem 7 Bun/Cr UA with micro Hematuria/proteinuria Liver panel Liver involvement (AIH), muscle involvement, hypoalbuminemia LDH Inflammatory markers (ESR, CRP)

19. More labs ANA profile: RNP, SM antibody, PM-1, SSA/Ro, SSB/La, SCL 70, dsDNA, FANA C3, C4 Coomb?s (direct) Antiphospholipid antibodies Lupus anticoagulant RPR Quantitative immunoglobulins Rheumatoid factor

20. Imaging?

21. CXR shows ?

22. Criteria for SLE (4/11 required) A RASH POINts MD Arthritis (non erosive) Renal disease (proteinuria, cellular casts) ANA (positive antinuclear antibody) Serositis (pleurisy or pericarditis) H aematological disorders (haemolytic anaemia/leucopenia/lymphopenia or thrombocytopenia) Photosensitivity Oral ulcers (also nasal ulcers) Immunological disorder (anti-DNA, anti-Sm,antiphospholipid antibody based on a false positive serological test for syphilis/lupus anticoagulant, or IgM or IgG anti-cardiolipin antibody) Neurological disorders (seizures or psychosis) Malar rash Discoid rash

23. Malar rash The rashes are extremely varied. At onset 50-80%, up to 85% during the course. Isolated discoid rash is rare. ~10% can turn to systemic disease. Erythematous ?butterfly? rash : 30-60% of children at onset, 80% during the course. Not pathognomonic Symmetric, spares nasolabial folds, quite well demarcated, may be slightly raised. Some follicular plugging. May be precipitated by sun exposure. Often heralds a disease flare The rashes are extremely varied. At onset 50-80%, up to 85% during the course. Isolated discoid rash is rare. ~10% can turn to systemic disease. Erythematous ?butterfly? rash : 30-60% of children at onset, 80% during the course. Not pathognomonic Symmetric, spares nasolabial folds, quite well demarcated, may be slightly raised. Some follicular plugging. May be precipitated by sun exposure. Often heralds a disease flare

24. Discoid lesions and other rashes

25. Other findings in SLE True vasculitis skin rash ? 10-20%, can result in digital infarct. Oral ulcer ? commonly at hard palate, painless ? disease flare !!! Alopecia ? up to 50%, occurs during active disease or after flare. Raynaud?s phenomenon ? 10-20% True photosentive rash : - ~ one third of pediatric SLE. - can occur in any sun expose area, other than the face - may be maculopapular or papulosquamous - may be associated with anti-Ro and anti-La - sun exposure may not only exacerbate skin flare, but may cause systemic flare !!! True vasculitis skin rash ? 10-20%, can result in digital infarct. Oral ulcer ? commonly at hard palate, painless ? disease flare !!! Alopecia ? up to 50%, occurs during active disease or after flare. Raynaud?s phenomenon ? 10-20% True photosentive rash : - ~ one third of pediatric SLE. - can occur in any sun expose area, other than the face - may be maculopapular or papulosquamous - may be associated with anti-Ro and anti-La - sun exposure may not only exacerbate skin flare, but may cause systemic flare !!!

26. For our patient Admit to the hospital Large pericardial effusion w/concern for tamponade physiology Cardiology consult PPD in preparation of steroid therapy? 24 hour urine to futher evaluate our patient?s proteinuria Ophthalmology consult to further evaluate for disease activity (vasculitis, baseline eye exam for impending Plaquenil therapy)

27. SLE Prototype of immune complex (imcpx) mediated disorder Defect in imcpx clearance by mononuclear phagocytic system is well documented. Estimated that 15-17% of lupus pts present before age 16. 10-20 per 100,000 children and adolescents (10-20:10,000) higher in hispanic, black and oriental people More severe organ involvement than in adults

28. SLE : etiology & pathogenesis... Organ injury is secondary to either direct binding of autoAbs to self-Ag or to deposition of imcpx in vessels or tissues. Abnormal immune responses?hyperactivated B- and T-cells, abn of monocyte-macrophages, abn of immunoregulation. Multifactorial factors: interaction of genetic susceptibility and environmental factors.

29. SLE : evidence for genetic predisposition in humans... A 3- to 10-folds increase in clinical disease in monozygotic vs dizygotic twins An 8-fold greater RR for SLE in 1st degree relatives, with 10 to 16% of pts with SLE having an affected 1st- or 2nd-degree relative

30. SLE : clinical manifestation? Ranges from an insidious, chronic illness with long history of intermittent signs and symptoms to an acute, rapidly fatal disease. Constitutional S/S are common at onset or exacerbation. Intermittent or sustained fever, fatigue, wt loss, anorexia are manifestations of active dz, or as presenting S/S. At onset: single or more common ? multisystem disease The earlier diagnosis and treatment are made, the easier the control and the better the outcome are the rule. Usually delay until 6-12 mths has elapsed in children (83%).

31. ?Lupus is a branch of internal medicine? - Virginia Pascual MD, Pediatric Rheumatologist and Researcher Scientist

32. SLE ?Mimics? Lymphoma (NHL, HL) Crohn?s disease/ IBD Endocarditis Tuberculosis EBV, CMV, HIV, ParvoB19 RA or JIA Systemic vasculitides

33. SLE can ? CVS Pericardial effusion ? tamponade physiology Myocardial infarction Libman Sacks Lung Pleural effusions Pulmonary hemorrhage Pulmonary embolism

34. SLE can ? Kidney ARF HTN Loss of AT3 Thrombotic manifestations Including TTP-HUS Antiphospholipid antibody syndrome

35. SLE can ?. Infection Can rapidly evolve into sepsis Hematologic abnormalities Hemolytic anemia Thrombocytopenia Leukopenia/neutropenia

36. What to do? Airway, breathing, and circulation If patient is decompensating call RRT or code Collect as much information as you can Brief history, ROS Physical exam Call your upper level Call rheumatology for help

37. Things to remember? History and physical exam findings are key A low-titer ANA test may be seen in unaffected individuals and family members Hematologic manifestations of SLE An increasing anti-DNA titer and decreasing C3 and C4 concentrations as signs of active SLE (renal) A serious infection can occur in a patient with SLE receiving immunosuppressive therapy


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