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 :

» Scientists and Clinicians Address Diagnosis and Treatment for Autoimmune and Autoinflammatory Diseases

Autoimmune and autoinflammatory diseases are a major health issue. The National Institutes of Health reports that up to 23.5 million Americans are afflicted. The American Autoimmune Related Diseases Association reports the number is closer to 50...

» The SCOT Study

SCOT is a clinical research study designed for people with severe forms of . SCOT stands for Scleroderma: Cyclophosphamide Or Transplantation. The SCOT study will compare the potential benefits of stem cell transplant and high-dose monthly...

» Millions Around World To Observe Rare Disease Day

Millions Around World To Observe Rare Disease DayThe Relief Foundation will be joining the National Organization for Rare Disorders (NORD) and others around the world in observing World Rare Disease Day on February 28, 2011.  On this day,...

» Musculoskeletal Rehabilitation in the Person with Scleroderma

Janet L. PoolePosted: 03/12/2010; Curr Opin Rheumatol. 2010;22(2):205 © 2010 Lippincott Williams & WilkinsAbstractPurpose of Review: The purpose of this review is to examine current evidence for the efficacy of rehabilitation techniques for...

» Unite Against Scleroderma 2012

{gallery}unite3{/gallery}

» Patients With Scleroderma May Not Exhibit Increased Corneal Thickness

in patients with systemic sclerosis may not differ significantly from those of healthy patients, contrary to prior hypothesis, a study found."Because [] is characterized by increased deposition of and other connective tissue components in the skin...