Juvenile Scleroderma Progression Similar To Adult Onset PDF Print E-mail
Friday, 27 July 2012 16:12
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.

 
More articles :

» Thank You

The Scleroderma Care Foundation launched its one month ago on September 1st, and we did so with several aims in mind. Firstly we sought to provide patients, their families, and friends with relevant and timely information on Scleroderma, tips for...

» Natural Alternatives for Dealing With Scleroderma

In a recent article, it was noted that conventional medicine attempts to treat the symptoms of with immunosuppressant drugs, NSAIDS and , but has no cure and little advice for prevention. It was noted however, that the world of natural healing has...

» New Research Findings May Bring Breakthrough For Scleroderma Patients

Research presented at the European Academy of Dermatology and Venereology in Prague, Czech Republic, suggests that inhibition of the may be a viable therapeutic target in patients.This finding builds on previous research by presenter and lead...

» Researchers Gain Deeper Insights Into Causes And New Treatment Strategies For Scleroderma

Using mice, lab-grown cells and clues from a related disorder, Johns Hopkins researchers have greatly increased understanding of the causes of , showing that a critical culprit is a defect in the way certain cells communicate with their structural...

» Godrick Weekes

I am thirty seven years old and I have for the past four years. For me, it has been a roller coaster ride with this condition and its symptoms. In the beginning, I first noticed that my fingers were always swollen on mornings, I could not bend them...

» A Brief History of Scleroderma

While scleroderma may not be very well known to the average person, investigation reveals it has been around for a long time.Classical ReferencesCases of skin disease similar to scleroderma may be found in the writings of Hippocrates as far back as...