Juvenile Systemic Sclerosis 4.5/5 (2)

Last updated: 5 / 12 / 20
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Swollen and stiff fingers in systemic sclerosis. Russo RA, Pediatrics Rheumatol Online J (2007). CC BY 2.0

Definition

Systemic sclerosis / systemic scleroderma is rare in both children and adults. Among children with systemic sclerosis, internal organs are less frequently attacked than by Systemic sclerosis in adults. However, the course of disease is chronic and the disease is regared as serious. Internal organs such as esophagus, stomach, bowel, heart, lungs or kidneys can be attacked.

Occurrence

In total, fewer than 10% are under 20 years of age at the onset of illness.

  • On average, 0 - 3 children are diagnosed with Juvenile Systemic Sclerosis annually in Norway

Difference from debut in adulthood:

Disease attacks of the lungs, esophagus, gastrointestinal tract, kidney and Pulmonary arterial hypertension is less common in childhood than among adults

  • Raynaud's is common, but somewhat less common than among adults
  • Children get more often Arthritis (inflammed joints)
  • Children get more muscle inflammation (Myositis) with elevated CK in blood samples
  • The diffuse form (please see below) is more common among children
  • Overlap with myocyte and dermato myocyte is more common

As among in adults systemic sclerosis is divided into different groups:

  • Diffuse shape: thickened skin on the body (truncus), face and arms / legs. Often available Scl-70 Antibodies in blood test
  • Limited form: The skin changes are limited to arms and legs distal (below) elbows and knees, as well as around the mouth. Anti-centromer / CENP antibody is common
  • Overlapping cases
  • Pre-scleroderma (early form)
  • “Scleroderma sine scleroderma” (scleroderma without skin attack) is most rare and most often develops with skin symptoms over time. The diagnosis is made on the basis of a combination of characteristic changes in internal organs, typically capillaroscopy and antibodies in blood samples

The diffuse form

Most common form in children (about 70%). Typical symptoms are

  • Raynaud's phenomenon (70%)
  • Increasing tightness of the skin on the hands, face and (less often) of the body
  • Swelling in the fingers and hands ("puffy hands") often occurs before the skin becomes tight
  • New wounds on fingertips (digital ulcer) in combination with Raynaud's phenomenon are particularly suspicious of the diagnosis

Blood tests: About 25% has anemia (low blood percentage), most often due to chronic disease, also bleeding from the stomach (“GIFT") may be a cause. Uncommonly, macrocytic Anemia of vitamin B12- and / or folic acid deficiency with reduced intestinal uptake (malabsorption). Some have moderately elevated CK (creatine kinase) when overlapping Myositis. Eosinophilia is present in the blood of 15%.

Limited form

Compose 20-30% of systemic sclerosis in children.

  • Fingers fingers and hands, sometimes also forearms, feet and legs as well as the area around the mouth, but not larger parts of the face or body (truncus)
  • Raynaud's phenomenon
  • Sore throat / necrosis on fingertips (digital ulcer)
  • Calcinosis (Calcium accumulations beneath the skin)
  • Telangiectasia (Bloodvessels under the skin)
  • Reduced esophageal function (esophagus dysfunction: swallowing problems, stomach acid regurgitation)
  • Pulmonary hypertension
  • Neurological manifestation with headache, cramps, encephalitis (brain inflammation)
  • Uveitis
  • Arthritis
  • Vasculitis diseases
  • Kidney/renal crisis and “GIFT" are less common than in adults

Diagnosis, investigation and treatment overlap partly with the equivalent of adults: See also general information here

Similar conditions / Differential diagnoses

Literature

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