rheumatology peds continued

Question 1

what are the most common ages for lupus

Answer 1

very rare before 10. often seen in adolescents, esp. around menarche

Question 2

what complication is seen in neonates born to moms with lupus

Answer 2

congenital heart block due to transplacental passage of maternal antibodies

Question 3

SLE diagnostic criteria

Answer 3

4/11:

1. serositis (pleuritis or pericardial effusion)
2. oral or nasal ulcers
3. arthritis (nonerosive)
4. photosensitivity
5. blood cytopenias
6. renal disease
7. ANA positive
8. anti-dsDNA antibodies or anti-Smith antibodies (smith is even more specific)
9. neuro symptoms (encephalopathy, seizures, psychosis)
10. malar rash
11. discoid lupus

Question 4

How is SLE managed?

Answer 4

1. immunosuppression. glucocortocoids are mainstay of treatment for kids. cyclophosphamide may also be useful, though it can cause hemorrhagic cystitis, infertility, and secondary malignancies.
2. treat complications:
   - anti-coagulation if thrombosis
   - renal failure txs

Question 5

major causes of death in SLE?

Answer 5

infection, renal failure, CNS complications

Question 6

dermatomyositis: epi and definition

Answer 6

• inflammatory condition of muscule that causes progressive muscle weakness with skin findings
• seen in 5-14 yos most commonly

Question 7

clinical features of dermatomyositis

Answer 7

constitutional symptoms
skin
-heliotrope rash, which crosses the nasal bridge
-gottron’s papules
proximal muscle weakness (esp. hip girdle/legs). gower’s sign.
may also have other manifestions (neck weakness, calcinosis, telangiectasias, constipation, dysphagia, cardiac issues)
NOT associated with malignancy in kids

Question 8

treatment of dermatomyositis

Answer 8

steroids

vitamin D and Ca to repair osteopenia

Question 9

what are the main complications of dermatomyositis?

Answer 9

• aspiration PNA
• intestinal perforation
• osteopenia (often d/t steroids)

Question 10

clinical features of rheumatic fever

Answer 10

cardiac involvement: endocarditis (most common), often of left sided heart valves; myocarditis (tachy out of proportion to fever), pericarditis (rare)
polyarthritis: migratory, asymmetric. no long-term jt disease
syndeham’s chorea
-erythema marginatum: pink-red macules which coalesce and spread centripetally with central clearing
officially diagnosed with jones criteria

Question 11

labs in rheumatic fever

Answer 11

antistreptolysin-o titers usually high- suggest recent strep infection
anti-DNase and anti-hyaluronidase

Question 12

tx rheumatic fever

Answer 12

• penicillin for strep
• NSAIDs for joint pain AFTER diagnosis has been established
• steroids if cardiac disease is severe
• haldol for sydenham’s chorea

Question 13

treatment of lyme disease

Answer 13

early disease or late disease with arthritis only is treated with doxycycline if >9, or amoxicillin
carditis and menigitis require IV ceftriaxone or penicillin