Periarteritis Nodosa in Infancy

POLYARTERITIS NODOSA IN AN INFANT A problem of diagnosis

“…The histological findings in our patient are apparently typical (Benyo and Perrin, 1968;Fager et al, 1951;Roberts and Fetterman, 1963) and the rapid deterioration in the last week is not uncommon in infancy, especially when the coronary arteries are involved. Until the case reported by Chamberlain and Perry (1971), the duration of illness in infants with coronary artery involvement had not exceeded 5 months.…”

“…The congestive failure was particularly perplexing and initially attributed to renal impairment with hypertension and fluid retention. The extensive arteritis of the myocardial and coronary vessels found at autopsy has been emphasized by several authors (Roberts and Fetterman, 1963; Benyo and Perrin, 1968) and particularly Munro-Faure ( 1959) who reported that in infancy the site of predilection for P.4N is the coronary arteries, and that they may be exclusively involved. Of 19 patients aged less than one year, 12 had predominant or exclusive involvement of the coronary arteries, and most of them had characteristic manifestations of the disease.…”

“…Apart from hyperventilation due to metabolic acidosis and terminal pulmonary oedema, our patient did not have any evidence of pulmonary disease and none was found at autopsy. The lungs are said to be rarely involved in childhood PAN (Benyo and Perrin, 1968), but commonly affected in adults (Rose, 1957).…”

Morphological observations on the vasculitis in the mucocutaneous lymph node syndrome

“…Subendothelial deposition of fibrinoid material is slight and not significant. These findings make it possible to differentiate MCLS from IPN, because the vasculitis in the latter disorder is most frequently observed in the medium-sized arteries and not the veins (Alarcdn-Segovia et al, 1964), it originates in the media leading to fibrinoid degeneration (Zeek, 1953), and lesions at five different stages (Arkin, 1930) are usually present simultaneously (Benyo et al, 1968).…”

Ruptured coronary aneurysm and valvulitis in an infant with polyarthritis nodosa

“…Polyarteritis nodosa of infancy differs from the adult counterpart both in the clinical presentation and in the distribution of the pathological lesions. In adults the disease is characterised by a protracted illness with multi-system involvement whilst in infancy the clinical course is rapid, the duration of the reported cases varying from 24 hr to 5 mth and the sudden, and often unexpected, fatal termination of the illness usually results from involvement of the coronary arteries (Benyo and Perrin, 1968).…”