Paediatric Scleroderma: Kids Are Not Just Little Adults

Abstract

The sclerodermas are autoimmune rheumatic diseases associated with pathological fibrosis of tissues. The two forms, localised scleroderma (LS [also referred to as morphoea]) and systemic sclerosis (SSC), have different patterns of organ involvement depending upon age of onset. Juvenile LS (JLS) has a poorer prognosis than adult-onset LS (ALS), while juvenile systemic sclerosis (JSSC) has a better prognosis than adult-onset SSC (ASSC). Optimal care requires appreciating the major differences between paediatric- and adult-onset disease, as they affect treatment and management strategies. Because the majority of patients with JLS have deeper tissue involvement, systemic immunomodulator rather than topical treatment is needed to mitigate their risk for serious morbidity and functional impairment. JSSC initially has a lower frequency of vital organ involvement than ASSC, but organ involvement can progressively accrue over time, so prolonged, aggressive treatment regimens may be needed. The authors recommend the care team for patients with JLS and JSSC include a rheumatologist who will be experienced in assessing and monitoring the most common extracutaneous involvement (musculoskeletal), as well as other organ involvement. Long-term monitoring of these patients into adulthood is essential; JSSC is a lifetime disease, while JLS can relapse or smoulder, with the disease activity focused in the deeper tissues. The purpose of this review is to provide a clinically focused overview of JLS and JSSC disease patterns, highlighting differences between paediatric and adult-onset disease. The authors will review current care recommendations for JLS and JSSC, and discuss some of the challenges for their care, and areas for future research.