SLE

(Neonatal Lupus)

11 criteria. Need 4 to make the dx.
5 in the head, 4 in the body, 2 serologic

  1. Neuro/Psych: seizure/psychosis: Choreais most common; Also seizures, psychosis, ataxia, pseudotumor cerebri…
  2. Photosensitivity
  3. malar rash Occurs in 30% of pts. Spares nasolabial folds; can be red/purple with follicular projections into skin; can get atrophy
  4. discoid rash: rare, can see in neonatal lupus
  5. oral/nasal ulcers: non-painful
  6. serositis: Pericarditis most common; Liebman-Sacks endocarditis* (classic for SLE and other anti-phospholipid antibodies)
  7. arthritis:>2 joints, usually non-erosive; Arthralgiasare more common, but not enough to meet this criteria; Most common presenting symptom.
  8. nephritis: 75% get some renal involvement; Look for persistent proteinuriaor casts
  9. cytopenia (WBC/HB/and/or PLT); Antibody mediated; Coombs (+) anemia; Can affect all cell lines
  10. +ANA
  11. one of these 4: +VDRL, +dsDNA*, +LE prep, +smith nuclear Ag.*

*fairly specific for SLE

Can also get: alopecia, increased PTT due to antiphospholipid Ab, decreased c3, c4
In active disease, C3, C4, and CH50 are decreased

*Sterile vegetations of the heart valves, or Liebman Sacks’ endocarditis, is a less common cardiac manifestation of SLE. It was recently suggested that Liebman Sacks’ endocarditis is often a part of the antiphospholipid syndrome. The prevalence of Liebman Sacks’ endocarditis has decreased since treatment with corticosteroids was introduced in the 1950s.

Discussion

  • M=F until adolescence, then F>M by 5x

  • Rare <5 y/o

  • Drug induced Lupus

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  • Common Drugs: hydralazine, INH, chlorpromazine, OCP

  • Sx stop 6 mos after stopping meds

  • Get Anti-Histoneantibodies

  • Represents 10% of cases, so look for offending agents

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    Treatment

    1. STEROIDS
    2. Sunblock (at least SPF 40)
    3. Cytotoxicagents (azathioprine, cyclophosphamide)
    4. Target organ specific tx (anti-coagulants, anti-psychotics)

    CHLA Board Review 2005