摘要
目的 总结抗合成酶抗体综合征相关性间质性肺疾病的临床特征,提高临床医师对该病的认识。方法 收集2018年8月至2021年7月期间福建省立医院呼吸与危重症医学科诊断的15例抗合成酶抗体综合征相关性间质性肺疾病患者的病例资料,回顾性分析其临床特征并进行文献复习。结果 15例患者男性4例,女性11例,中位年龄64岁,主要症状为咳嗽、气促。胸部CT主要表现为双下肺胸膜下分布的磨玻璃影或实变影。8例完成肺通气功能检查,5例提示不同程度限制性通气功能障碍,6例完成肺弥散功能检查,均提示弥散功能下降。12例完成肺泡灌洗液细胞学分类检查,中性粒细胞、淋巴细胞、嗜酸性粒细胞升高者比例分别为83.3%(10/12)、8.3%(1/12)、83.3%(10/12)。10例经支气管肺活检中仅1例明确机化性肺炎。抗合成酶抗体方面,抗EJ抗体阳性6例,抗PL-7抗体阳性2例,抗PL-12抗体阳性5例,抗Jo-1抗体阳性4例,抗OJ抗体阳性1例,其中2例同时存在不同的抗合成酶抗体。从发病到诊断中位时间为60d。随访过程中4例死亡,11例好转。仍在随访的9例接受糖皮质激素联合免疫抑制剂治疗的患者中,2例复发。结论 对于不明原因的间质性肺疾病,及时完善抗合成酶抗体检测有助于早期诊断及治疗,经支气管肺活检在诊断方面作用有限,糖皮质激素联合免疫抑制剂是该病药物治疗基石。间质性肺疾病进行性加重提示预后不良。
Objective To summarize the clinical features of antisynthetase syndrome-related interstitial lung disease (ASS-ILD) and raise clinicians’ awareness of the disease.Method Case data of 15 patients with ASS-ILD diagnosed in the Department of Respiratory and Critical Care Medicine of Fujian Provincial Hospital from August 2018 to July 2021 were collected and analyzed retrospectively by literature review.Result Of the Fifteen patients,four were male and eleven were female.The median age at diagnosis was 64 years.The main symptoms were cough and dyspnea.The chest CT is mainly manifested as ground glass opacities or consolidation and predominantly in the subpleural with bilateral lower lungs predilection.Among eight patients completed ventilation function test,five cases indicated different degrees of restrictive ventilatory dysfunction.Among six patients completed diffusing capacity test,all indicated decreased diffusing capacity.The proportion of elevated neutrophil,lymphocyte,and eosinophile granulocyte were 83.3% (10/12),8.3% (1/12),and 83.3% (10/12),respectively in 12 patients who had undergone cytological classification of bronchoalveolar lavage fluid.Only one of the ten transbronchial lung biopsies indicated organic pneumonia.Of the antisynthetase antibodies,anti-EJ (n=6),anti-PL-7 (n=2),anti-PL-12 (n=5),anti-Jo-1 (n=4) and anti-OJ (n=1) were identified.Two patients simultaneously had different antisynthetase antibodies.The median total time to diagnosis was 60 days.Four deaths occurred,eleven patients had improved,and two relapse cases occurred in nine patients treated with glucocorticoids combined with immunosuppressive agents during the follow-up.Conclusion Timely antisynthetase antibodies test is helpful for the early diagnosis and treatment in unexplained interstitial lung diseases.However,transbronchial lung biopsy played a limited role in the diagnosis.Glucocorticoid combined immunosuppressant is the cornerstone of the medication.Progressive exacerbation of interstitial lung disease suggests a poor progn
作者
许航
林明
谢宝松
Xu Hang;Lin Ming;Xie Baosong(Shengli Clinical Medical College of Fujian Medical University,Fuzhou 350001,China;Department of Pulmonary Critical Care Medicine,Fujian Provincial Hospital,Fuzhou 350001,China)
出处
《创伤与急诊电子杂志》
2022年第1期22-28,31,共8页
Journal of Trauma and Emergency(Electronic Version)
