Juvenile Systemic Sclerosis, scleroderma in children 4.5/5 (2)

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Swollen and stiff fingers in systemic sclerosis. Russo RA, Pediatrics Rheumatol Online J (2007). CC BY 2.0

Definition

Scleroderma in children (juvenile scleroderma) is divided into two main groups: systemic sclerosis which is discussed here and localize scleroderma / morphea which has its own page. The diseases have different degrees of severity and course, but early diagnosis and treatment are considered important for a good prognosis (Zulian F, 2018). Systemic sclerosis / systemic scleroderma is rare in both children and adults. Among children with systemic sclerosis, internal organs are less frequently attacked than by wood 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 systemic sclerosis, less than 10% are under the age of 20 at the onset of the disease. On average, 0 - 3 children are diagnosed with Juvenile systemic sclerosis annually in Norway. Girls and boys are attacked just as often before the age of 8, after which the disease debuts 3 times more often among girls. Family occurrence is very rare (Mayes MD, 2003).

Symptoms

Forskjell from debut in adulthood. Disease attacks on the lungs, esophagus, gastrointestinal tract, kidney and Pulmonary arterial hypertension is less common in childhood than among adults. The diffuse form (please see below) is more common in children. Overlap against myocyte and dermato myocyte is more common

Raynaud's phenomenon is common, but somewhat less common than in adults.

Joint. Children get more often Arthritis (inflammed joints)

Muscles. Children more often get muscle inflammation (Myositis) with elevated CK in blood samples

Diffuse form is most common in children. Audio in adults systemic sclerosis is divided into different groups: Diffuse form: thickened skin on the body (truncus), face and arms / legs. Increasing tightness in the skin of the hands, face and (less frequently) the body. Swelling in the fingers and hands ("puffy hands") often occurs before the skin becomes tight. Raynaud's phenomenon (70%). New wounds on fingertips (digital ulcers) in combination with Raynaud's phenomenon is particularly suspicious of the diagnosis. Often also available Scl-70 antibody in blood test.

Blimited form accounts for 20-30% of systemic sclerosis in children. The skin changes with hard skin are seen on the fingers and are maximally limited to the arms and legs below the elbows and knees, as well as around the mouth. Larger parts of the face or body (truncus) are not attacked. Raynaud's The phenomenon of "dead fingers" is seen in almost everyone. Wounds / necrosis on fingertips (digital ulcers), m calcinosis (calcium accumulations under the skin) and telangiectasias (blood vessel drawings under the skin) are not always present. Some have reduced esophageal function (esophageal dysfunction: swallowing problems, stomach acid regurgitation). Anti-centromer / CENP antibody in blood tests 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 antibody in blood samples.

Medical examinations

Medical history with Raynaud's phenomenon, swollen fingers and eventually tight skin are important (see symptoms above).

Clinical examines skin on arms, legs, face and body, joints, lungs and other internal organs. Blood pressure and heart rate.

Blood tests include inflammatory tests (CRP and SR), but they are often normal. Kidney function tests. ANA with any subgroups, including scleroderma-specific antibody (centromer / CENP, topoisomerase-1 (Scl 70), fibrillarin, PM-Scl 70/100, RNA polymerase I or III) can be evaluated. In arthritis, supplement with anti-CCP.

Urine sticks is current.

Capillaroscopy of nail folds often show changes with thick blood vessels and small hemorrhages.

Imaging. CT of lungs on suspicion of lung manifestations. Older children can do X-rays of the esophagus with contrast agent for swallowing difficulties.

Other. Older children can do lung function tests. ECG and ultrasound of the heart.

Similar conditions / Differential diagnoses

Treatment

Raynaud's phenomenon can be symptomatically treated with calcium channel blockers, most often nifedipine. Other treatment is individually adapted and should take place in collaboration with a department with experience.

Prognosis

Internal organs are attacked less frequently than in adults with systemic sclerosis. The forecast is thus generally better. A very slow progression of cutaneous manifestations can be expected. 

Literature



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