IgG4 相关疾病可表现为复发性自发性血胸：病例报告。

IgG4-related disease can present as recurrent spontaneous hemothorax: a case report.

机构信息

Department of Respiratory Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.

Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.

出版信息

BMC Pulm Med. 2019 Feb 1;19(1):26. doi: 10.1186/s12890-019-0785-y.

DOI:10.1186/s12890-019-0785-y

PMID:30709387

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6359758/

Abstract

BACKGROUND

Immunoglobulin G4-related disease (IgG4-RD) encompasses a group of immune-mediated disorders that are gaining increasing recognition. Pulmonary presentations are common, with four types of patterns been described on radiography, including solid nodular, bronchovascular, ground glass opacities, and alveolar interstitial. Pleural thickening and pleural effusion have also been reported. However, there have been no reports of IgG4-RD that presents as spontaneous hemothorax.

CASE PRESENTATION

A 61-year-old Chinese woman experienced recurrent right-sided chest pain and transient syncope. A significant decrease in her hemoglobin level and thick bloody pleural fluid demonstrated spontaneous hemothorax. The elevated serum IgG4 and histopathological analysis of the right pleura and intercostal lymph node specimens all supported the diagnosis of IgG4-RD in this patient. Further diagnostic evaluation did not reveal other causes for spontaneous hemothorax. She received steroids and no recurrent bleeding event occurred during a follow-up period of more than 1 year.

CONCLUSION

Recurrent spontaneous hemothorax can be a rare manifestation of IgG4-RD, with pleural involvement as the most probable mechanism.

摘要

背景

免疫球蛋白 G4 相关疾病（IgG4-RD）包括一组免疫介导的疾病，其受到越来越多的关注。肺部表现较为常见，在影像学上有四种类型的表现模式，包括实性结节、支气管血管、磨玻璃影和肺泡间质。也有报道胸膜增厚和胸腔积液。然而，尚无 IgG4-RD 表现为自发性血胸的报道。

病例介绍

一名 61 岁的中国女性反复出现右侧胸痛和短暂晕厥。血红蛋白水平显著下降和浓稠血性胸腔积液提示自发性血胸。血清 IgG4 升高，右侧胸膜和肋间淋巴结标本的组织病理学分析均支持该患者的 IgG4-RD 诊断。进一步的诊断评估未发现自发性血胸的其他原因。她接受了类固醇治疗，在超过 1 年的随访期间没有再次发生出血事件。