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Morning Report

September 24, 2010. Morning Report. SLE. 20% diagnosed in childhood Mostly in adolescence F:M ratio Prior to puberty - 3:1 After puberty - 9:1 Native Americans > African Americans > Hispanics > Chinese > Filipinos More severe disease in AA and Hispanics Multigenic.

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Morning Report

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  1. September 24, 2010 Morning Report

  2. SLE • 20% diagnosed in childhood • Mostly in adolescence • F:M ratio • Prior to puberty - 3:1 • After puberty - 9:1 • Native Americans > African Americans > Hispanics > Chinese > Filipinos • More severe disease in AA and Hispanics • Multigenic

  3. Clinical Manifestations • Most common symptoms in teenagers • Fever • Rash • Mucositis • Arthritis • Constitutional • Weight loss • Malaise

  4. Mucocutaneous • Butterfly rash • Malarerythema • Photosensitive • Sparing of nasolabial folds • Discoid lesions • Scalp or extremities • Photosensitive • Maculopapular lesions • Occur anywhere

  5. Mucocutaneous • Alopecia • Begins frontal • Spreads diffusely • Rarely permanent • Vasculitic • Palmarerythema • Mucosal lesions • Ulcers • Sometimes painless

  6. CNS • 2nd leading cause of morbidity and mortality • Psychiatric • Psychosis • Seizures • HA • Lupus cerebritis • Also difficulty with concentration, memory, depression or decline in school performance • Less commonly • Chorea, neuropathies, transverse myelitis

  7. Cardiovascular • Any layer of the heart affected • Pericardium • Most common • Myocarditis • CHF, arrhythmia • Libman-Sacks endocarditis • At risk for SBE

  8. Cardiovascular • Raynaud phenomenon • Very common in adolescents • Cold, emotional stress, caffeine, cigarette smoke • At risk for infarction • Premature atherosclerosis

  9. Pulmonary • Pleuropulmonary disease • Pleuritic chest pain • Effusion • 60% of adolescent patients will have abnormal PFTs • Other • Pneumonitis • Pulmonary Hemorrhage • Pulmonary HTN

  10. Gastrointestinal • Abdominal pain is common • Pancreatitis • Mesenteric vasculitis • Peritonitis • Hepatitis • Question #2

  11. Renal • #1 cause of morbidity • 75% of children within first 2 years • Signs • Microscopic hematuriaand proteinuria • HTN • Decreased GFR • Elevated BUN or Cr

  12. Renal • Renal Biopsy

  13. Musculoskeltal • Arthralgia • Arthritis • Nonerosive, nondeforming • Small and large joints • Myalgia • Proximal muscle weakness • Myositis • Possible MCTD

  14. Hematologic • Cytopenia • Any cell line • Anemia 50% • Normocyticnormochromic • Anemia of chronic disease or autoimmune hemolytic • ITP

  15. Lab Eval • ANA • Best screening test • Positive in almost all that have active disease • Not specific • More specific if ≥ 1:1,280 • Other diseases • JIA, Dermatomyositis, scleroderma, thyroid disease

  16. Lab Eval

  17. Lab Eval • Complement (C3, C4) • Reduced levels • May indicate need for aggressive therapy

  18. Lab Eval • Antiphospholipid antibodies • CBC • Electrolytes • BUN and Cr • Hepatic Enzymes • Acute-phase reactants • UA

  19. Treatment • Consult with Rhematology • Treatment depends on clinical manifestations • Emerging morbidity is associated with treatment • Close monitoring

  20. Prognosis • Improving • Juvenile-onset • 92% - 5 year survival rate • 85% - 10 year survival rate • Major causes of death • Renal disease • Infection • CNS disease

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