Murata A, Takeda Y, Usuki J, Niitani H, Kamoi S, Araki T, Koizumi K, Syouji T, Kawanami O
Nihon Kyobu Shikkan Gakkai Zasshi. 1989 Sep;27(9):1106-11.
A 35-year-old primigravida was admitted to the Department of obstetrics complaining of dyspnea and left back pain at 21 weeks' gestation. Chest roentgenogram revealed diffuse reticulonodular shadows predominantly in both lower lung fields and arterial hypoxemia was present. Pulmonary function tests showed restrictive impairment and decreased carbon monoxide diffuse capacity. From these results, interstitial pneumonia was suspected and she was first treated with prednisolone. However during her pregnancy, spontaneous pneumothorax occurred. Following spontaneous delivery of healthy infant at 37 weeks, left chylothorax occurred, and pleurodesis was performed with OK432. Thereafter the histological diagnosis of pulmonary lymphangiomyomatosis was made by transbronchial lung biopsy and treatment of prednisolone was stopped. She was treated with tamoxifen. In addition, progesterone-receptor was detected in the pulmonary tissue obtained at open lung biopsy. She was treated with cyclophosphamide in addition to tamoxifen. At present, shortness of breath has decreased slightly in comparison with one year previously, but no improvement has been seen in lung function tests or chest roentgenogram.
一名35岁初产妇在妊娠21周时因呼吸困难和左背痛入住产科。胸部X线片显示双下肺野为主的弥漫性网状结节影，存在动脉低氧血症。肺功能测试显示限制性损害和一氧化碳弥散能力下降。根据这些结果，怀疑为间质性肺炎，她首先接受泼尼松龙治疗。然而，在她怀孕期间，发生了自发性气胸。在37周时自然分娩出健康婴儿后，出现了左乳糜胸，并使用OK432进行了胸膜固定术。此后，通过经支气管肺活检做出了肺淋巴管平滑肌瘤病的组织学诊断，泼尼松龙治疗停止。她接受了他莫昔芬治疗。此外，在开胸肺活检获取的肺组织中检测到孕激素受体。除他莫昔芬外，她还接受了环磷酰胺治疗。目前，与一年前相比，呼吸急促略有减轻，但肺功能测试或胸部X线片未见改善。
