嗜酸粒细胞性哮喘的罕见病因:高IgG4综合征(IgG4相关硬化性疾病)

2015/10/23

   摘要
   前言:
高IgG4综合征是一种新近认识的伴有多种胸部症状的疾病,包括嗜酸粒细胞性哮喘、腺体肿大或纵膈纤维化、哮喘、浸润型肺炎、支气管和胸膜的结节或假性肿瘤。
   病例报告:一例38岁女性患者,诊断为嗜酸粒细胞性哮喘急性发作期伴总IgE水平升高。有特发性急性胰腺炎病史,这与硬化性胆管炎和肾功能不全有关,结合该患者的迟发型严重和非特应性哮喘,提示诊断为高IgG4综合征。之前的肝组织学和肾脏CT检查可进一步确认该诊断。
   结论:IgG4综合征有不同的临床及影像学表现,对激素治疗敏感,但呈进行性发展,因此,应引起足够重视。

 

(苏欣 审校)
RevMalRespir.2015Jul30.pii:S0761-8425(15)00266-1.doi:10.1016/j.rmr.2015.06.011. [Epub ahead of print]



 

 

A rare cause of eosinophilic asthma: Hyper-IgG4 syndrome (IgG4-related sclerosing disease).
 

 Article in French
Mehdaoui A1, Manoila M2, Jaafar M2, Mahmoud H2, Devin E2.

 

Abstract
INTRODUCTION:
The hyper-IgG4 syndrome is an emerging disease with various thoracic manifestations: eosinophilic asthma, adenomegalies or mediastinal fibrosis, asthma, infiltrative pneumonia, nodules or pseudo-tumors with bronchial and pleural localizations.
CASE REPORT:We report the case of a 38-year-old woman who was admitted for acute exacerbation of eosinophilic asthma with high total IgE levels. A medical history of idiopathic acute pancreatitis, associated with sclerosing cholangitis and renal failure, suggested a diagnosis of hyper-IgG4 syndrome in this woman with late-onset severe and non-atopic asthma. A previous hepatic histology and a reinterpretation of renal tomodensitometry have confirmed this hypothesis.
CONCLUSION:The hyper-IgG4 syndrome is responsible of different clinico-radiological patterns that should be evoked because of the pejorative evolution of this steroid sensitive disease.

 

RevMalRespir.2015Jul30.pii:S0761-8425(15)00266-1.doi:10.1016/j.rmr.2015.06.011. [Epub ahead of print]

 


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