Nakamura Masayuki, Okamoto Masaki, Momosaki Seiya, Nakayama Yoshihuku, Huruya Kiyomi, Ichiki Masao, Aizawa Hisamichi
Department of Pulmonary Medicine and Clinical Research Center, National Kyushu Medical Center.
Nihon Kokyuki Gakkai Zasshi. 2011 Feb;49(2):116-21.
A 34-year-old woman developed polydipsia, polyuria, amenorrhea and loss of pubic hair in 2001, but did not seek medical advice. On September 7th, 2009, she was admitted to our hospital complaining of acute exacerbation of dyspnea on exertion. Chest computed tomography (CT) showed multiple cystic lesions, predominantly in bilateral lower lung fields. Non-segmental, diffuse ground-glass attenuated areas and thickened bronchovascular bundles were also seen in bilateral lung fields. Pathological findings of lung specimens from a surgical lung biopsy (right S6 and S8) 14 years previously showed infiltration of S100 protein-positive histiocytoid cells in the bronchiolar wall. As a result, pulmonary Langerhans cell histiocytosis (PLCH) was diagnosed. Moreover, panhypopituitarism due to LCH was identified on endocrine testing. Dyspnea on exertion, reduction of carbon-monoxide diffusing capacity (D(LCO)) and ground-glass attenuation areas on CT were improved by smoking cessation alone, and she was discharged. However, similar acute deterioration of PLCH recurred 4 months after first admission. Her dyspnea on exertion, reduction of D(LCO) and ground-glass attenuation areas on CT were improved again by 500 mg/day methylprednisolone pulse therapy for 3 days. This case was a unique combination of panhypopituitarism and the appearance and disappearance of ground-glass attenuation areas on CT, paralleling PLCH disease activity.
一名34岁女性于2001年出现烦渴、多尿、闭经及阴毛脱落，但未就医。2009年9月7日，她因劳力性呼吸困难急性加重入院。胸部计算机断层扫描（CT）显示多个囊性病变，主要位于双侧下肺野。双侧肺野还可见非节段性、弥漫性磨玻璃样密度减低区及支气管血管束增粗。14年前手术肺活检（右肺S6和S8）的肺标本病理结果显示细支气管壁有S100蛋白阳性组织细胞样细胞浸润。因此，诊断为肺朗格汉斯细胞组织细胞增多症（PLCH）。此外，内分泌检查发现因LCH导致的全垂体功能减退。仅通过戒烟，劳力性呼吸困难、一氧化碳弥散量（D(LCO)）降低及CT上的磨玻璃样密度减低区均有所改善，她随后出院。然而，首次入院4个月后PLCH再次出现类似的急性恶化。通过给予500mg/天甲泼尼龙冲击治疗3天，她的劳力性呼吸困难、D(LCO)降低及CT上的磨玻璃样密度减低区再次得到改善。该病例是全垂体功能减退与CT上磨玻璃样密度减低区的出现和消失并存，且与PLCH疾病活动情况平行的独特组合。
