Juvenile systemic sclerosis with rapid progression and multisystem involvement in an adolescent

Area of Science:

• Rheumatology
• Pediatrics
• Autoimmune Diseases

Background:

• Juvenile systemic sclerosis (JSSc) is a rare, progressive autoimmune disorder with diverse organ involvement.
• JSSc can impact the skin, musculoskeletal, gastrointestinal, pulmonary, cardiac, renal, and endocrine systems, leading to significant morbidity and mortality.
• Clinical manifestations are variable, including oesophageal dysmotility, interstitial lung disease, and pulmonary hypertension.

Purpose of the Study:

• To report a rare case of juvenile systemic sclerosis with extensive multisystem involvement in an adolescent male.
• To illustrate the clinical presentation, treatment challenges, and disease progression in a pediatric patient with JSSc.

Main Methods:

• Case report of an adolescent male diagnosed with juvenile systemic sclerosis.
• Description of clinical presentation, including polyarticular arthritis, skin thickening, and hypopigmented lesions.
• Documentation of treatment response to methotrexate and subsequent disease progression.

Main Results:

• The patient presented with a 5-month history of polyarticular arthritis and skin changes.
• Initial methotrexate treatment showed minimal improvement, with subsequent worsening after treatment cessation due to varicella zoster infection.
• Disease progression included worsening skin thickening, joint contractures, restricted mobility, interstitial lung disease, and dysphagia.

Conclusions:

• This case underscores the potential for severe multisystemic involvement in juvenile systemic sclerosis.
• The challenges in managing JSSc, including treatment adherence and disease flares, are highlighted.
• Early diagnosis and consistent management are crucial for improving outcomes in pediatric patients with JSSc.