Scleroderma

The pattern and course of disease in patients who develop systemic sclerosis in childhood or adolescence is similar to that in patients who develop the disease as adults, data from Europe has shown.

There were 60 patients among 5,000 included in the EUSTAR database (1.2%) who developed systemic sclerosis before the age of 16. Their mean age of onset was 12.4 years. They were compared with another 910 patients who first developed symptoms between the ages of 20 and 40, at a mean of 32 years.

Both groups had a mean disease duration of 17 years, and the illness was active at the last follow-up in about one-quarter of cases. The distribution of disease subsets was similar in juvenile-onset and adult-onset cases: roughly 40% had diffuse disease, 50% had limited disease and 10% had overlap syndrome.

There were no significant differences between the two groups in the extent of organ involvement. Raynaud’s phenomenon was most common, affecting 95%, followed by oesophageal involvement (60-65%), digital ulcers (35-41%), joint contractures (30-37%) and pulmonary fibrosis (23-36%). Antibody profiles were also similar, except that the frequency of anti-centromere antibodies was higher in the juvenile-onset cohort.

In 15 of the 60 juvenile-onset patients, the first non-Raynaud’s symptoms emerged before the age of 10 years. Compared to post-pubertal onset, pre-pubertal onset was associated with a higher proportion of male cases and a higher modified Rodnan skin score. “Juvenile systemic sclerosis is a rare disease in childhood, with an estimated incidence of 0.5 per 100,000 and a prevalence of about 1 in 1,000,000,” the researchers said.

“It has been hypothesised that about 10% of all patients develop the disease before the age of 16 years but nothing is known about whether puberty has any influence on the incidence.”

Source: James, T. (2012), "Juvenile-onset systemic sclerosis resembles adult-onset disease"; Rheumatology Update; Original article can be viewed here.