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Management of Complications and Emergencies
The most serious emergencies in childhood polyarteritis are (i) renal insufficiency; (ii)
severe hypertension; (iii) cardiac complications such as CHF, myocardial infarction,
and dysrhythmias; (iv) GI vasculitis resulting in bowel infarction, intestinal perforation,
or cholecystitis; and (v) CNS manifestations, such as seizures and cranial nerve palsies
( Table 101.7 ).
Renal Emergencies. Although medical management of PAN has resulted in a
significantly improved prognosis, azotemia and hypertension at the time of diagnosis
continue to identify children with extremely aggressive disease. Arteritis of mediumsized vessels of the kidney may lead to renal infarction and ischemia or to
glomerulonephritis manifested by hematuria, hypertension, and uremia (see Chapter
100 Renal and Electrolyte Emergencies ). Management of renal failure includes high
doses of corticosteroids to control the underlying disease process (e.g., prednisone 2
mg/kg/day). Sudden flank pain associated with gross hematuria, falling blood pressure,
and an expanding abdominal mass suggest the possibility of aneurysmal dilatation and
rupture, with renal artery hemorrhage.
Hypertension. A mild to moderate elevation of blood pressure is noted in more than
90% of children with generalized PAN. Management follows the general rules for
treating renovascular hypertension and typically necessitates the input of a nephrologist
(see Chapter 37 Hypertension ).
Cardiac Emergencies. See Chapter 86 Cardiac Emergencies . Pericarditis may be
asymptomatic. Echocardiogram is the most sensitive means of confirming pericarditis.
Patients with pericarditis with no or small effusion may be treated with careful
monitoring and corticosteroids. Pericardiocentesis is indicated in the presence of
tamponade or if infection is suspected.
Chest pain with tachycardia, arrhythmia, and dyspnea may herald the occurrence of
myocardial infarction in a patient with PAN involving the coronary arteries. Pericardial
tamponade caused by a ruptured coronary aneurysm may present similarly.
Occasionally, a patient with coronary artery disease may present with CHF.
Characteristic EKG changes may be seen. Echocardiography is indicated to study the
myocardial function. Coronary arteriography is essential to establish the size, location,
and extent of aneurysms and occlusions.