黄甲综合征呼吸系统受累二例并文献复习

Respiratory manifestations of yellow nail syndrome： report of two cases and literature review

目的探讨黄甲综合征（YNS）呼吸系统的临床表现，提高该病的诊治水平。方法回顾性分析北京协和医院呼吸内科治疗的2例不同临床亚型的YNS患者的临床及胸部高分辨率CT特点，并以＂黄甲综合征＂为检索词，在＂万方数据库、中国知网和中国期刊全文数据库（CNKI）＂中检索出中文文献6篇；以＂yellow nail syndrome, yellow nail and lung＂为关键词，在＂PubMed数据库＂检索出英文文献81篇，综合复习相关文献后进行分析。结果2例患者均为男性，年龄分别为60和76岁，均有双手＂黄甲＂的表现，1例还出现双足趾＂黄甲＂的表现。1例因反复肺部感染就诊，影像学表现为双下肺分布为主的弥漫性支气管扩张，间断给予针对性抗感染治疗、长期口服大环内酯类抗生素治疗后病情相对稳定；另1例则因咳嗽、活动后气短就诊，影像学表现为难治性双侧胸腔积液、左下肺分布为主的多发支气管扩张、双下肢淋巴水肿，给予间断抽胸腔积液，并尝试奥曲肽皮下注射治疗。从国内外文献来看，半数以上的YNS（共373例）患者均有不同形式的呼吸系统受累，其中以胸腔积液（152例）和弥漫性支气管扩张症（121例）多见。几乎所有胸腔积液的YNS患者都有淋巴水肿，多为双侧渗出液，胸腔积液中以淋巴细胞为主，对胸膜粘连、胸导管结扎等外科手术反应良好，但近年来也尝试用生长抑素或类似物治疗。结论YNS是一种以＂黄甲＂、呼吸系统受累及肢体淋巴水肿为表现的罕见病，除＂黄甲＂外，呼吸系统是最常见受累部位，影像学主要表现为弥漫性支气管扩张、难治性胸腔积液。

ObjectiveTo describe the clinical characteristics of respiratory manifestations of yellow nail syndrome.MethodsWe conducted a retrospective analysis of 2 patients with respiratory diseases associated with yellow nail syndrome. Their clinical and chest radiological data were collected. We searched PubMed, Wanfang and CNKI databases with the keywords ＂yellow nail syndrome, yellow nail and lung＂ in Chinese and English. And the relevant literatures, including 6 articles in Chinese and 81 articles in English, were reviewed.ResultsOur 2 patients were male, one 60 years old and the other 76. Typical yellow nails were present in their fingers, and one of them also showed toe yellow nails. One patient was admitted for refractory respiratory infection and he was diagnosed with diffuse bronchiectasis. The respiratory symptoms could be relieved with antibiotics according to the results of sputum microbiological analysis. The other patient was admitted for cough and exertional dyspnea, and refractory pleural effusions were revealed bilaterally. He received repeated effusion drainage by thoracentesis, and Octreotide was tried recently. A total of 373 cases were reviewed in Chinese and English literatures. Pleural effusions （152 cases） and diffuse bronchiectasis （121 cases） were the most common reported respiratory manifestations. Lymphoedema was present in almost all cases with pleural effusion associated with yellow nail syndrome, and the effusion was usually exudative and lymphocyte predominant. Pleurodesis and decortication were effective for them. But, somatostatin analogues had been tried effectively for these patients recently. On the other hand, literatures showed that diffuse bronchiectasis in yellow nail syndrome was less severe than idiopathic diffuse bronchiectasis, and might benefit from long-term macrolide antibiotics.ConclusionsYellow nail syndrome is a very rare disorder. Besides yellow nail, respiratory manifestations are the main clinical presentations. Diffuse bronchiectasis and recurrent pleural effusions are the common manifestations.