Navigating the labyrinth: Phenotypes of IgG4-related disease.

Journal of postgraduate medicine Pub Date : 2025-01-01 Epub Date: 2025-03-14 DOI:10.4103/jpgm.jpgm_757_24
M Shah, K Bajan, R Samant
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Abstract

Abstract: We report a case of an elderly female with subacute submental neck swelling, dry cough, and a history of significant weight loss. Unexpectedly, a left submandibular chronic sialadenitis was noted on ultrasound of the neck, along with a thyroid nodule that, on biopsy, showed lymphocytic infiltration. High-resolution computed tomography of the lungs revealed multifocal irregular consolidations with lymphadenopathy, and possible infective causes, including tuberculosis, were ruled out after a thorough microbiological evaluation. It was only after a positron emission tomography-guided lung biopsy with specialized immunohistochemical staining was performed that the diagnosis of IgG4-related disease (IgG4-RD) was confirmed. Our patient exhibited overlapping features of head and neck-limited and Mikulicz/systemic phenotype of IgG4. In this case report, we highlight the clinical phenotypes of IgG4-RD, their respective differential diagnoses, and discuss our approach to this challenging case.

导航迷宫:igg4相关疾病的表型
摘要:我们报告一例老年女性亚急性颏下颈部肿胀,干咳,并有明显的体重减轻史。出乎意料的是,颈部超声检查发现左侧下颌下慢性涎腺炎,同时活检显示甲状腺结节淋巴细胞浸润。肺部高分辨率计算机断层扫描显示多灶性不规则实变伴淋巴结病,在彻底的微生物学评估后排除了可能的感染原因,包括结核病。只有在进行正电子发射断层扫描引导下的肺活检并进行专门的免疫组织化学染色后,才能确诊为igg4相关疾病(IgG4-RD)。我们的患者表现出头颈受限和Mikulicz/全身IgG4表型的重叠特征。在这个病例报告中,我们强调了IgG4-RD的临床表型,它们各自的鉴别诊断,并讨论了我们对这个具有挑战性的病例的方法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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