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Juvenile Systemic Lupus Erythematosus Morning Report: Symptoms, Lab Evaluation, Treatment, and Prognosis

Learn about juvenile systemic lupus erythematosus (SLE) diagnosis, symptoms, lab tests, and treatment. Prognosis and survival rates for pediatric patients. SLE manifestations vary across different ethnic groups. Multigenic clinical features impact disease severity.

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Juvenile Systemic Lupus Erythematosus Morning Report: Symptoms, Lab Evaluation, Treatment, and Prognosis

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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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