At both King Khalid University Hospital and King Faisal Specialist Hospital in Riyadh, Saudi Arabia, between 1990 and 1997, we identified 11 children with polyarteritis nodosa (PAN). Nine of them satisfied the American College of Rheumatology 1990 criteria for the classification of PAN. All patients were followed for at least 12 months. The male-to-female ratio was 1.8:1. Their mean age at disease onset was 5.8 years (range 2-11.5 years) and the mean duration of the disease was 2.5 years (range 1-7 years). The clinical presentations in the children were musculoskeletal symptoms in all patients, mucocutaneous findings in 10 (91%), fever in eight (73%), gastrointestinal manifestations in six (55%) and hypertension in five (45%). The most frequent laboratory findings were leucocytosis, anaemia, thrombocytosis and a high erythrocyte sedimentation rate. Both histopathological and angiographic findings were an effective means of diagnosis. Nine children responded to glucocorticoids (GC) with cytotoxic drugs while the rest improved on GC alone. None of the children died from the disease. A high index of suspicion with early combination therapy will help in decreasing the morality in these cases.
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