Pediatric emergency medicine trisk 2918 2918
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CHAPTER 101 ■ RHEUMATOLOGIC EMERGENCIES
THERESA M. BECKER, MELISSA HAZEN
GOALS OF EMERGENCY CARE
Pediatric rheumatologic conditions are rare and are typically chronic conditions with an
indolent onset rather than acute conditions likely to bring a child to the emergency
department (ED). Nonetheless, there are several reasons why children with
rheumatologic conditions may present to the ED. First, the majority of rheumatologic
conditions involve a myriad of signs and symptoms affecting many organ systems,
which may bring an exasperated family to the ED searching for an elusive diagnosis.
Second, arthritis, lupus, and vasculitis (especially Kawasaki disease [KD]) may have
acute and life-threatening complications that require rapid initiation of appropriate
therapy. Finally, the treatment of rheumatologic disorders is becoming more
sophisticated and more specialized, involving combinations of anti-inflammatory,
immunosuppressive, and biologic agents, with a wide spectrum of undesired effects.
Often a key challenge is differentiating the effects of underlying disease from the
effects of therapy. Thus, the goals of emergency care are the prompt recognition of
these conditions, and the expeditious use of medical therapy to treat the complications
of the diseases and the side effects of drug therapy.
KEY POINTS
Kawasaki disease requires treatment in the first 10 days of the illness in
order to achieve an optimal clinical outcome.
Many rheumatologic conditions are treated with medications that suppress
the immune system.
Stress doses of corticosteroids may be required for fever and other acute
illnesses.
Childhood vasculitis may affect any organ system and may present
indolently or acutely with life-threatening end-organ involvement.
Juvenile idiopathic arthritis subtypes are varied in their presentation and
associated with different articular and extra-articular complications.
Hemophagocytic lymphohistiocytosis (HLH) and macrophage activation
syndrome (MAS) or reactive HLH should be considered in an ill child with
persistent fever, organomegaly, and neurologic symptoms with systemic
inflammation, cytopenias, and/or liver dysfunction.
