| 胡晶晶,管彩虹.IgG4相关性肺疾病1例及文献复习[J].浙江中西医结合杂志,2019,29(9): |
| IgG4相关性肺疾病1例及文献复习 |
| A review of 1 case of IgG4-related lung disease |
| 投稿时间：2018-12-17 修订日期：2019-07-15 |
| DOI： |
| 中文关键词: 肺疾病 免疫球蛋白G4 |
| 英文关键词:IgG4 related lung disease |
| 基金项目: |
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| 中文摘要: |
| 【】目的 通过分析1例IgG4相关性肺疾病的临床资料并复习文献,增加对IgG4相关性肺疾病知识的了解,提高临床诊断准确率。方法 分析本院收治的1例IgG4相关性肺疾病患者的临床表现、实验室检查、影像学及病理资料以及治疗过程等,并复习文献。结果 患者为51岁男性,因“活动后气急4月”入院,胸部(肺部)增强CT示：左肺上叶病变,伴纵隔内多发肿大淋巴结,建议穿刺活检明确。两肺散在多发慢性炎症性病变；两侧胸腔积液；血清免疫球蛋白G4 3200.0mg/dl；左上肺穿刺病理示：为呈慢性炎伴增生、部分玻变的纤维化组织,纤维组织中有散在较多CD38阳性浆细胞,并有聚集成巢趋势,免疫组化： IgG4较多+(>100个/高倍视野),诊断为IGG4相关性肺疾病,应用糖皮质激素治疗1月后症状明显缓解,复查CT提示病灶逐渐变小,肺容积增大,胸水吸收,疗效显著。结论 IgG4相关性肺疾病属于少见病,且由于临床表现复杂,影像表现多样,易与常见的肺疾病相混淆,出现误诊和漏诊,应提高对IgG4相关性肺疾病知识的了解。 |
| 英文摘要: |
| 【Abstract】Objective To describe the clinical characteristics of IgG4-related lung disease. Methods The elinical symptoms,laboratory examination,radiographic patterns,histopathological features and treatment of a patient with IgG4-related lung disease were summarized through literature reviewing. Results A 51-yea-old male was admitted to our department because he has have been short of breath for 4 months, Chest CT revealed lesions in the left upper lobe, with mediastinal multiple enlarged lymph nodes , Both lungs are scattered in multiple chronic inflammatory lesions; Pleural effusion on both sides, The serum IgG4 concentration was 3200.0mg/dl,and pathological examination of the lung tissues found chronic inflammation, fibrous tissue with scattered more CD38 positive plasma cells, and gathered into a nest,IgG4 immunostaining revealed predominant lgG4+ plasnm cells(>100 / HP), The patient was diagnosed as IgG4-related lung disease and received oral methylprednisolone at the dose of 4mg per day for one month,his symptoms improved,Two months later a CT scan revealed revealed improvement．Conclusion IgG4-related lung disease is a rare disorder and easily overlooked in clinical practice．This study could help clinicians improve recognition and knowledge of this rare disease. |
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