Pediatric emergency medicine trisk 2955 2955
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Current Evidence
Although the pathologic process in vasculitis is limited to the blood vessels, the
presence of vasculature in every organ of the body means that virtually any symptom
could be a presentation of vasculitis. Vascular inflammation and damage can lead to
anything from numbness to pain, thrombosis to bleeding, and aneurysm formation to
vascular obstruction. Pediatric vasculitides are very rare, however. This section will be
limited to a general overview of situations in which the diagnosis should be considered,
followed by more detailed discussions of anti-neutrophil cytoplasmic antibody
(ANCA)-associated vasculitis, polyarteritis nodosa (PAN), juvenile dermatomyositis
(JDM), and Behỗet disease (BD). Along with PAN, Kawasaki disease is one of the
most prevalent vasculitides of childhood and will be discussed separately.
Goals of Treatment
The long-term goals of treatment for the vasculitides are to minimize vascular
inflammation and attendant end-organ damage while monitoring for progression of
disease and adverse effects of therapies. For the emergency physician, management of
life-threatening emergencies will occur in the usual manner. Stress doses of
corticosteroids and increases in immunosuppressant dosing may be required.
Clinical Considerations
Clinical Recognition
Early in the course of a vasculitis, findings are generally nonspecific, primarily
reflecting systemic inflammation (fever, malaise, fatigue, failure to thrive, elevated
acute-phase reactants). As vascular damage progresses, evidence of vascular
compromise characteristic of the particular vessels involved becomes evident on
physical examination. For example, hypertension may evolve as renal vascular
involvement progresses.
Despite the extreme variability of the manifestations of vasculitis, certain symptoms
are particularly suggestive of vascular inflammation. Involvement of large- or mediumsized muscular arteries, as may be seen in Takayasu arteritis (TA) or PAN, initially
causes symptoms related to the severity of the inflammatory response. As vascular
compromise progresses, symptoms of arterial insufficiency begin to dominate.
Involvement of large vessels to the extremities, such as the subclavian or femoral
arteries, typically leads to claudication, whereas involvement of visceral vessels causes
hypertension (renal arteries), abdominal pain (mesenteric and celiac axes), chest pain
(aortic or coronary artery involvement), or neurologic symptoms (focal neurologic
deficits or neuropathic pain).
Inflammation of smaller arteries and arterioles leads to symptoms in richly
vascularized organs. Skin involvement—livedo reticularis, purpuric (generally
palpable) or nonblanching lesions, and palmar or plantar rashes—is most suggestive of
vascular inflammation. Pulmonary, renal, and GI arterial beds are often involved as
