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Kids and Scleroderma

Questions & Answers

2018 Patients photo Youth YogaHow scleroderma impacts children compared to the “average” patient, an adult.
Responses by Kathryn Torok, M.D., Children's Hospital of Pittsburgh

What is the incidence and prevalence of scleroderma in youth compared to adults?

Kids Room Sunday Conference 2018Pediatric onset scleroderma is DIFFERENT from adult scleroderma.  In adults, systemic scleroderma (SSc), affecting the skin and internal organs, like heart and lung, is the more common type of scleroderma.  In childhood onset scleroderma (<18 years old when symptoms start), the localized scleroderma, also known as ‘morphea’, type of scleroderma is more common.  In children, LS (morphea) is about 10 times more common than SSc (systemic sclerosis).  The prevalence of LS in children is similar to that of juvenile diabetes mellitus, affecting 50 per 100,000 children, whereas the prevalence of SSC in children is estimated to be 2 in 100,000. When scleroderma does occur in children, the average age of onset of juvenile localized scleroderma (jLS) is approximately 6 to 8 years old, and the average age of onset in juvenile systemic sclerosis (jSSc) is 8 to 11 years old.   These two entities, localized scleroderma and systemic sclerosis, are quite distinct entities, although in a very, very small number of patients, both can occur together, this is extremely rare (<0.1%).  When this does occur, they typically have a similar time of onset, and one does not evolve into the other.

What is the difference in disease progression between a youth and an adult?

Although every child’s experience with scleroderma is unique, there are some general patterns in regard to general outcomes and prognosis that have been reported, and these are different from adult-onset scleroderma. The main two forms of scleroderma in childhood are listed separately below:
 
Childhood-Onset Localized Scleroderma:
  • Kids Room Conference 2018Within the localized scleroderma (LS) type of scleroderma, there are subtypes; linear scleroderma (trunk/limb, head), circumscribed morphea (superficial or deep), generalized morphea, and mixed morphea. These are the same subtypes in children and adult onset LS. However, jLS has much more frequent linear scleroderma of the trunk/limb and head (scalp/face) and mixed morphea subtypes. Whereas adult LS has morphea circumscribed (superficial) and generalized morphea plaques.
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  • The more frequent deeper tissue involvement in jLS linear subtypes leads to certain ‘extracutaneous’ or non-skin involvement, which is more common in children, such as:
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    • Joint contracture 
    • Limb length discrepancy (shorter affected limb) 
    • Limb girth discrepancy (smaller girth of limb) 
    • Skull changes linear band on scalp or mandible/maxilla (jaw changes) if linear head involvement 
    • Brain lesions/changes and neurological symptoms (with linear head involvement)
     
  • The disease duration in jLS is longer than adult onset LS. Prior literature regarding morphea, or LS, was based on adult dermatology cohorts in which it was thought that LS would ‘burn out’ after 3-5 years. Now, more recent cohort studies have shown that CHILDHOOD ONSET LS is DIFFERENT, the disease duration in jLS has a wide range from 7 to 10+ years. A recent European cohort showed 12.5% of jLS patients still had active disease 10 years later. (Marini, Zulian Autoimmunity Reviews 17 (2018) 727-734) 
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  • In jLS, with a longer disease duration, there are more chances for damage or sequala of the underlying tissue if left untreated. Also, with childhood onset there is more of a chance to affect limb and face development and growth, which is not as much of an issue in adult-onset disease since they have already fully matured.  
Childhood-Onset Systemic Sclerosis:
  • Kids Conference 2018 PhiladelphiaRegarding systemic sclerosis (SSc), it is actually the opposite of what was mentioned regarding LS above. In jSSc, the general severity of the disease is LESS in childhood onset then adult onset. The general rate of internal organ involvement, like heart, kidney and lung, is LESS in childhood-onset compare to adult onset SSc; more favorable. 
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  • The subtypes of SSc; diffuse cutaneous, limited cutaneous and overlap occur in both jSSc and adult SSc, but the frequency of overlap jSSc is much more common in children. This overlap is typically manifested by the child presenting to clinic with Raynaud’s phenomenon (with or without digital tip ulcers) some muscle weakness or elevation of muscle enzymes and arthritis. These patients overall tend to get less pulmonary involvement, like interstitial lung disease.
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  • The mortality rates in jSSc is MUCH LOWER than that which is reported in the adult SSC literature. Several cohort studies (in Europe and North America) have shown a much higher overall survival rate in jSSc compared to adult onset disease.

How do symptoms among youth compare to symptoms of adults?

  • Juvenile Localized Scleroderma (jLS) 
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    • Since children are growing and developing the impact of linear scleroderma can manifest in the following ways, which is not typically seen in adult-onset Ls: 
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      • One might notice that the child’s leg, arm, or finger look smaller (underdeveloped from the deeper tissue involvement of linear scleroderma)
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      • It is hard to do certain extracurricular activities handstands, flexibly of legs, arms noted in those that have decrease range of motion of joints from deeper involvement
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      • Limp or putting weight on toes if joint contracture (decreased range of motion) lower extremity
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      • Smaller face, tongue from hemiatrophy associated linear scleroderma of the head.
     
  • Juvenile Systemic Sclerosis (jSSc)
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    • Mentioned above regarding frequency of overlap SSC subtype in children; usually present with combination Raynaud’s (+/- digital ulcers), myositis and arthritis 
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    • Failure to gain weight dropping percentiles on growth charts is unique to pediatric SSc since if they have gut involvement they are not getting the nutrition they need to grow, and weight may be stunted showing deceleration on growth chart
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      • Low BMI for children has seen in cohorts, published in recent CARRA Cohort
 

Videos

My Child Has Scleroderma…What Does That Mean?
Kathryn (Cassie) Torok, M.D.
YouTube My Child has Scleroderma Torok 2017

Your Child Has Been Diagnosed with Scleroderma…Now What?
Kate Silver, M.D., M.S.C.R.
YouTube Silver 2018

Medical Information Toolkit: How to Find Reliable Medical Information on the Internet
Jessica Farrell, Pharm.D.
YouTube 2018 Farrell

 

Articles

Juvenile Systemic Sclerosis
Ivan Foeldvari, M.D., Ph.D.
“On Call” excerpt from Scleroderma Voice, summer 2014

About Juvenile Localized Scleroderma
Suzanne C. Li, M.D., Ph.D., Joseph M. Sanzari Children’s Hospital, Hackensack University Medical Center, New Jersey
“On Call” excerpt from Scleroderma Voice, summer 2014

 

References

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