According to a story from Scleroderma News, a recent study found that while treatment approaches for juvenile localized scleroderma were quite consistent in the UK, there were significant disparities in how patients were screened for the disease. The Paediatric Rheumatology European Society (PRES) has developed a comprehensive set of guidelines for assessing, managing, and diagnosing juvenile localized scleroderma.
Scleroderma, which is also referred to as systemic sclerosis, describes a group of autoimmune diseases that can cause system-wide effects in the most severe cases. The mechanism of this disease is believed to be an autoimmune response in which the immune system mistakenly attacks body tissue. Juvenile localized scleroderma is a less severe form that primarily affects the skin and soft tissues. Some factors that may contribute to triggering the autoimmune response include mutations of the HLA genes and exposure to certain materials, such as certain solvents, white spirits, ketones, and silica. Symptoms are broad ranging and systemic, including kidney failure, erectile dysfunction, fatigue, stroke, headaches, facial pain, congestive heart failure, skin abnormalities, high blood pressure, chest pain, indigestion, and many more. Treatments are varied and depend on the symptoms, but most patients take medications in an attempt to suppress the autoimmune response. In severe cases, life expectancy is around 11 years from onset; however, the juvenile localized form does not affect lifespan. To learn more about scleroderma, click here.
About The Study
The researchers set out the with goal of conducting an audit of treatment and diagnostic practices for juvenile localized scleroderma in the UK. The study used data from 149 patients with the disease. The PRES has selected two diagnostic tools that it recommends for caregivers. These are the localized scleroderma cutaneous assessment tool (LoSCAT) and the physician global assessment of activity (PGA-A).
However, the study found that these tools were not universally used by doctors in the UK, far from it in fact. Only 37.6 percent of patients received the LoSCAT and just 54.4 percent had the PGA-A. The PRES also recommends that patients with any head involvement be screening for uveitis, or eye layer inflammation. A MRI brain scan is also recommended. Only 38.5 percent were screened for uveitis and 71.2 percent had an MRI.
Treatment for juvenile localized scleroderma was more consistent. 95.5 percent were treated with methotrexate and 89.6 percent received mycophenolate mofetil. These treatments closely followed PRES guidelines.
The study revealed the need for more consistent diagnostic screening practices in the UK. Check out the original study at in the journal Pediatric Rheumatology here.