Juvenile systemic sclerosis: experience from a tertiary care center from India

Area of Science:

• Pediatric Rheumatology
• Autoimmune Diseases
• Systemic Sclerosis

Background:

• Juvenile systemic sclerosis (JSSc) is a rare autoimmune condition with limited data on treatment and long-term outcomes.
• Understanding JSSc is crucial for managing its impact on children's health and development.

Purpose of the Study:

• To analyze the demographic, clinical, laboratory, treatment, and outcome data of children diagnosed with systemic sclerosis.
• To evaluate the effectiveness of current treatment protocols in managing JSSc and improving patient quality of life.

Main Methods:

• Retrospective analysis of case records of 32 children diagnosed with systemic sclerosis between January 1998 and June 2016.
• Disease outcome was classified based on daily activities and the presence of specific clinical features.
• Treatment regimens included naproxen, methotrexate, calcium channel blockers, and steroids, with HCQ added in select cases.

Main Results:

• The study included 32 children (21 girls) with a mean age of 112.79 months at presentation and a median diagnostic delay of 28.5 months.
• Common clinical features included Raynaud's phenomenon (68.7%), skin rash (62%), and arthritis/arthralgia (50%).
• Treatment resulted in disease control in 59.3% of children, partial control in 26.6%, and no response/progression in 16.6%.

Conclusions:

• Juvenile systemic sclerosis, though rare, is associated with significant morbidities.
• While a cure is lacking, methotrexate and steroid-based immunosuppressive therapy can achieve disease control and a reasonable quality of life for affected children.
• Further research is needed to optimize treatment strategies and improve long-term outcomes for JSSc patients.