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Systemic Sclerosis (Systemic Scleroderma), Pediatric

Reviewed 10/2018
 


BASICS

DESCRIPTION

  • Systemic sclerosis (SSc) is an autoimmune vasculopathic disease causing fibrosis of skin and internal organs.

    • SSc is a type of scleroderma (meaning “hard skin”) but should not be conf...

DIAGNOSIS

HISTORY

  • Onset is almost always insidious.

  • Initial symptoms in SSc

    • Reflection of vasculopathy in microvasculature

      • RP

      • Edematous fingers

      • Nailfold capillary changes and distorted/thickened cuticles

    • RP ...

TREATMENT

GENERAL MEASURES

  • Collaborative care with subspecialists (pulmonary, GI, cardiology), school, social work, physical therapy

  • Supportive care: Avoid skin trauma; wound care for digital and other ...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

  • Physical exam for digital or other skin ulcerations, joint mobility, muscle bulk, weight loss, poor growth

  • Pulmonary function tests and echocardi...

ADDITIONAL READING

  • Foeldvari I. Update on juvenile systemic sclerosis. Curr Opin Rheumatol.  2015;17(3):18. [View Abstract on OvidInsights]

  • Martini G, Foeldvari I, Russo R, et al; for Juvenile Scl...

CODES

ICD9

710.1 Systemic sclerosis 

ICD10

  • M34.9 Systemic sclerosis, unspecified

  • M34.89 Other systemic sclerosis

SNOMED

  • 128461001 Systemic sclerosis sine scleroderma

  • 89155008 Systemic sclerosis

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