AORTITIS SYNDROME IN CHILDREN - CLINICAL OBSERVATION OF 35 CASES IN JAPAN

The results of clinical observation of 35 patients with aortitis syndr ome (AS) in childhood, obtained by a nationwide survey in Japan, are r eported. The male to female ratio was 1 :2.5, the estimated age of ons et averaged 10.2 years, and the duration from the estimated age of ons et to thr diagnosis averaged 15 months. In HLA examination A24, Bw52, Cw7 and DR2 were relatively common, Arterial lesions tended to extensi vely involve the aortic arch and its branches, Fever was the most freq uently noted clinical symptom, followed by abdomen, joint and muscle p ain, The physical findings in order of frequency were impaired circula tion of the upper extremities, cardiac and vascular murmurs, hypertens ion, impaired cerebral circulation, visual disorder and impaired circu lation of the pulmonary artery. The murmurs were found not only over t he chest wall but also over the cervical area and abdomen, Pulselessne ss of the upper extremities occurred in 66% of patients, Percutaneous retrograde aortography and/or intravenous digital subtraction angiogra phy to make the final diagnosis was employed except for three cases. T here were not any specific abnormal signs in laboratory data, Steroid hormones were administered in 34 cases, and immunosuppressive agents i n 8 cases. Five cases had percutaneous transluminal angioplasty to the right renal artery as an interventional treatment. The high frequency of abdominal pain is considered to be one of the characteristics of A S in childhood, The high frequency of pulselessness of tile upper extr emities and cardiac and vascular murmurs in this report is considered significant for the diagnosis of AS in childhood.