Overview of Pediatric Rheumatology: Part Two

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Section snippets

Key points

  • •

    When systemic lupus erythematosus presents in childhood, there is high risk for major organ involvement, particularly nephritis, and accrual of irreversible damage.

  • •

    Children with juvenile dermatomyositis have increased risk for calcinosis compared with those with adult-onset disease.

  • •

    Kawasaki disease is a self-limited vasculitis unique to childhood.

  • •

    A broad spectrum of autoinflammatory syndromes can present in childhood.

Brief introduction

Part two of this review on pediatric rheumatic diseases focuses on systemic lupus erythematosus, juvenile dermatomyositis and vasculitis, with particular emphasis on how the presentations, manifestations and prognoses of these diseases differ from the adult population. In addition, there is a brief review of some of the most common autoinflammatory syndromes in childhood.

Systemic lupus erythematosus

Approximately 20% of systemic lupus erythematosus (SLE) cases are diagnosed before age of 16. The estimated incidence is 0.3 to 0.9 per 100, 000 children-years.1 Several studies comparing the clinical features of adult and pediatric lupus highlight children commonly manifest major organ involvement and more frequently accrue damage than adults.2 Children have also been shown to have higher Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) scores at presentation in children when

Juvenile dermatomyositis

Juvenile dermatomyositis (JDM), although a rare condition, is the most common idiopathic inflammatory myopathy in childhood.22,23 Estimated annual incidence is 1.9 to 3.2 cases per million24,25 with typical median age of onset ranging from 5.7 to 7.7 years.26, 27, 28, 29 The primary manifestations are proximal muscle weakness and prototypic typical rashes, however, presentation can be variable.30 Although the exact etiology of JDM is still unclear, there are known genetic and environmental

Vasculitis

The classification criteria for vasculitis in childhood are adapted from classification criteria used in adults.54,55

Periodic fevers and autoinflammatory syndromes

Autoinflammatory syndromes are a group of disorders characterized by dysregulation of the innate immune system with recurrent fevers as the primary clinical hallmark. Most hereditary autoinflammatory syndromes are characterized by monogenic defects. As an in-depth description of autoinflammatory diseases is beyond the scope of this review, only the most common disorders are discussed. The reader is directed to several existing comprehensive reviews describing the full spectrum of the known

Summary

The care of children with rheumatic diseases is challenging given the impact of inflammatory conditions and their treatments on growth and development, functional abilities, self-image, self-efficacy, and family dynamics. Delay in recognition and appropriate referral results in a significant, negative impact on outcomes. Therefore, it is important for clinicians to recognize the presenting symptoms and physical manifestations of these diseases. This review highlighted the most common rheumatic

Disclosure

The authors have nothing to disclose.

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