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解析肉芽肿性淋巴细胞间质性肺病:一例具有不寻常临床特征且对静脉注射免疫球蛋白有反应的常见可变免疫缺陷病例。

Unravelling Granulomatous-Lymphocytic Interstitial Lung Disease: A Case of Common Variable Immunodeficiency With Unusual Clinical Features and Response to Intravenous Immunoglobulin.

作者信息

Pragash Nirosha, Mann Jennifer, Antonov Anton

机构信息

Department of Thoracic Medicine Peninsula Health Melbourne Victoria Australia.

The Institute for Breathing and Sleep Melbourne Victoria Australia.

出版信息

Respirol Case Rep. 2025 Feb 1;13(2):e70102. doi: 10.1002/rcr2.70102. eCollection 2025 Feb.

Abstract

Granulomatous-lymphocytic interstitial lung disease (GL-ILD) is a rare pulmonary complication associated with common variable immunodeficiency (CVID), complicating diagnosis due to overlapping symptoms with other chronic respiratory conditions. This case involves a 33-year-old male with a history of sarcoidosis, presenting with recurrent sino-pulmonary infections, mediastinal and axillary lymphadenopathy, and significant splenomegaly. Despite initial treatment with prednisolone, his symptoms persisted, and FDG-PET imaging showed metabolic activity in the sinuses and lymph nodes. Immunological assessment revealed markedly reduced immunoglobulin levels, leading to intravenous immunoglobulin (IVIG) therapy, which resulted in substantial improvement. A critical learning point is recognising that splenomegaly is commonly associated with CVID, which can aid in distinguishing it from other conditions. This case underscores the importance of considering CVID, with or without GL-ILD, as a differential diagnosis in patients with persistent respiratory symptoms and granulomatous lung disease, including sarcoidosis. Further research is needed to optimise treatment strategies for this rare condition.

摘要

肉芽肿性淋巴细胞间质性肺病(GL-ILD)是一种与普通可变免疫缺陷(CVID)相关的罕见肺部并发症,因其症状与其他慢性呼吸道疾病重叠而使诊断复杂化。该病例涉及一名33岁有结节病病史的男性,表现为反复的鼻窦肺部感染、纵隔和腋窝淋巴结肿大以及明显的脾肿大。尽管最初使用泼尼松龙治疗,但其症状仍持续存在,FDG-PET成像显示鼻窦和淋巴结有代谢活性。免疫学评估显示免疫球蛋白水平显著降低,导致采用静脉注射免疫球蛋白(IVIG)治疗,症状得到大幅改善。一个关键的学习点是认识到脾肿大通常与CVID相关,这有助于将其与其他疾病区分开来。该病例强调了在有持续性呼吸道症状和肉芽肿性肺病(包括结节病)的患者中,将CVID(无论有无GL-ILD)作为鉴别诊断的重要性。需要进一步研究以优化针对这种罕见疾病的治疗策略。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/143e/11786966/55983abc3eef/RCR2-13-e70102-g001.jpg

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