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Wegener肉芽腫症は1931年Klingerによつて始めて記載されたが,1936年Wegener1)の報告以来一つの独立疾患として扱われるようになつた。これは鼻・肺など呼吸器の壊死性肉芽腫,壊死性血管炎,壊死性糸球体腎炎を特徴とした致命的な疾患である。
従来多くの治療が試みられたが,その予後に効果を期待することはほとんどできなかつた。しかし最近免疫抑制剤の使用で一定の効果を得ることができるようになつてきた。
A woman, aged 33, complained of nasal impairement, epistaxis and a swelling of the cheek in her 9th month of pregnancy.
Examination showed the nasal cavity filled with necrotic tissues. Biopsy examination showed Wegener's granulomatosis.
Following the delivery the patient was treated by administration of betatron and bleomycin with no improvement of symptoms. The chest x-rayshowed an increased shadow in the lower portion of the left lung.
With the patient on imuran and predonine the nasal granuloma gradually subsided and the chest shadow, also, gradually disappeared. CRP showed improvement; RA became negative. However, the patient began to show signs of decreased adrenal function which precluded the further use of adrenal cortex hormone.
With this discontinuation of the predonine therapy the recurrence of the original symptoms were soon established. Predonine and other steroid therapy were reemployed, not only with no avail, but caused the establishment of symptoms of gastric ulcer and candida infection. The granuloma increased in growth. The patient died in the 7th month of treatment.
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