Pediatric emergency medicine trisk 2978 2978
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series, large-vessel thrombosis was the leading cause of death, carrying a 30%
mortality rate.
Management
The general treatment of BD is similar to other forms of vasculitis discussed in this
chapter, consisting of anti-inflammatory/immunosuppressive agents. Life-threatening
cases of BD may require high-dose systemic corticosteroids and cyclophosphamide.
Thrombotic disease requires anticoagulation in addition to aggressive
immunosuppression.
ARTHRITIS
CLINICAL PEARLS AND PITFALLS
Childhood arthritis lasting 6 weeks or more without alternative etiology is
termed juvenile idiopathic arthritis (JIA).
JIA subtypes differ in the pattern of joints involved, extra-articular
manifestations, and treatment.
Macrophage activation syndrome (MAS) is a severe and potentially lifethreatening complication of systemic JIA (sJIA).
JIA is frequently treated with disease-modifying and biologic agents that may
suppress the immune system.
Arthritis is a clinical finding of joint inflammation characterized by warmth,
swelling, tenderness, and/or restriction of joint movement, often accompanied by
prolonged morning stiffness. Arthritis is a common childhood finding and has many
causes, including infection and autoimmunity. Infectious arthritis may be caused by a
multiplicity of pathogens, including bacteria, viruses, and fungi. Bacterial arthritis, also
known as septic arthritis, is addressed in Chapters 46 Limp and 94 Infectious Disease
Emergencies . In addition, an inappropriately self-directed immune response triggered
by infection may lead to a post-infectious reactive arthritis. While this class of arthritis
is typically self-limited, treatment, usually with NSAIDs, may be needed to ameliorate
symptoms. On occasion postinfectious inflammatory arthritis recurs or persists, leading
to (or unmasking) a chronic arthritis akin to more typical juvenile inflammatory
arthritis. When childhood arthritis persists and no alternative etiology is discovered, it
is termed JIA. In this chapter, we will focus our discussion on JIA. Lyme arthritis, a
late manifestation of systemic infection with Borrelia, is discussed in Chapter 94
Infectious Disease Emergencies .
JUVENILE IDIOPATHIC ARTHRITIS
