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Sarcoidosis, Pediatric

Peter Weiser, MD and Randy Q. Cron, MD, PhD Reviewed 10/2018
 


BASICS

DESCRIPTION

A chronic granulomatous disease (CGD) with noncaseating epithelioid giant cell granulomas in multiple organs that has two distinct variants often differentiated by age of onset 

EPIDEMIOLOGY

DIAGNOSIS

  • EOS/Blau—patients have more typical clinical triad presentation; gene mutation testing can confirm diagnosis.

  • ATD—presentation is more vague; symptoms are less specific and biopsy confirmatio...

TREATMENT

Medications are used to treat active disease with clinical symptoms. 
  • Pitfalls include overtreating asymptomatic lymphadenopathy and not detecting hypercalciuria.

MEDICATION

  • Corticosteroids oft...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

  • Referral to rheumatologist indicated

  • Regular ophthalmologic assessment

  • Signs to watch for:

    • Rising creatinine levels

    • Shortness of breath

    • Persistent uv...

ADDITIONAL READING

  • Baumann RJ, Robertson WC Jr. Neurosarcoid presents differently in children than in adults. Pediatrics.  2003;112(6, Pt 1):e480–e486. [View Abstract on OvidInsights]

  • Blau EB. Fam...

CODES

ICD9

  • 135 Sarcoidosis

  • 517.8 Lung involvement in other diseases classified elsewhere

  • 713.7 Other general diseases with articular involvement

  • 695.2 Erythema nodosum

ICD10

  • D86.9 Sarcoidosis, unspecified

  • D8...

FAQ

  • Q: Why is therapy in childhood sarcoidosis more aggressive compared with adults?

  • A: These may be two distinct granulomatous diseases. EOS/Blau is a very aggressive and destructive disease requiring...

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