肺動脈病変を伴った大動脈炎症候群の病態と予後

Clinical findings and prognosis of aortitis syndrome associated with pulmonary vascular disease

　大動脈炎症候群は欧米に少なく，我が国を含むアジア地域に比較的多い非特異的な汎動脈炎であり，大動脈，主分枝動脈，肺動脈などに好発する。

　1910年太田¹が，大動脈炎症候群に肺動脈病変を伴った1剖検例を最初に報告している。その後いくつかの報告がみられ，その病態生理の究明とともに本症の病態が種々であり，症例により臨床経過および予後に著明な差異があることが判明してきた。近年血管造影や肺血流シンチグラムなどの進歩普及によって，肺動脈病変に大きな関心が寄せられている。

Although pulmonary vascular changes in aortitis syndrome were described by Ohta in 1940, little attentions have been given to these lesions or to the natural history of cases with them.

The clinical, hemodynamic and pulmonary angiographic features have been studied in patients with pulmonary vascular involvements.

Such involvements were found in 30 (38%) of 80 cases with aortitis syndrome. In most of the 30 cases, the aortic lesions were not always limited to the aortic arch, but were also found in the thoracic and abdominal portions of the descending aorta.

Moreover, other complications, such as aortic regurgitation (63%), coronary artery involvement (20%), hypertension (63%) and renal artery in-volvement (40%) were found more often in the patients with pulmonary involvement than in those without these changes. We found that changes in the main pulmonary artery and its branches are correlated with systolic pulmonary pressure. Pulmonary hypertension was found in 77% of the 30 cases. The patients with pulmonary hyperten-sion had pulmonary symptoms, such as hemo-ptysis, pulmonary infarction and right-sided heart failure, and their prognosis was worse than that of the other patients.

The present study shows that patients with pulmonary involvements should be followed up carefully.