Riedel's Thyroiditis and Multifocal Fibrosclerosis Are Part of the IgG4-Related Systemic Disease Spectrum
Riedel's Thyroiditis and Multifocal Fibrosclerosis Are Part of the IgG4-Related Systemic Disease Spectrum
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DOI：
10.1002/acr.20215
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发表时间：
2010-09-01
影响因子：
4.7
通讯作者：
Stone, John H.
中科院分区：
文献类型：
--
作者：
Dahlgren, Mollie;Khosroshahi, Arezou;Stone, John H.
Objective.里德尔甲状腺炎是一种病因不明的慢性纤维化疾病，常伴有多灶性纤维硬化。“IgG4相关的全身性疾病的特征是IgG4+浆细胞浸润和许多器官的纤维化。我们假设里德尔甲状腺炎是IgG 4相关的全身性疾病谱的一部分。我们使用术语“里德尔氏病”、“甲状腺肿”、“甲状腺”和“纤维化”检索了我们机构的病理学数据库，并确定了3例里德尔氏甲状腺炎。如果有纤维炎症过程涉及全部或部分甲状腺，并有证据表明该过程扩展到周围组织，则诊断为里德尔甲状腺炎。免疫组织化学染色IgG 4和IgG。对每个受累器官进行组织病理学和免疫组化分析。报告1例多器官系统疾病患者的临床特点。所有3份甲状腺切除术样本均对携带IgG 4的浆细胞染色呈阳性。1例患者有广泛的甲状腺外受累，诊断为IgG 4相关的全身性疾病，包括胆管炎、肺和泪腺假瘤以及与甲状腺相邻的淋巴结，其IgG 4+浆细胞染色强烈。所有受累器官的组织学特征均与IgG 4相关的全身性疾病一致。患者3最初每个高倍视野有10个IgG 4+浆细胞，但2年后再次活检显示没有IgG 4+浆细胞。该患者的第二次活检以纤维化和轻微残留炎症为特征，进一步巩固了组织中携带IgG 4的浆细胞与Riedel甲状腺炎组织学演变之间的联系。里德尔甲状腺炎是IgG 4相关全身性疾病谱的一部分。在许多情况下，多灶性纤维硬化症和IgG 4相关的系统性疾病可能是同一个实体。
Objective. Riedel's thyroiditis is a chronic fibrosing disorder of unknown etiology often associated with "multifocal fibrosclerosis." IgG4-related systemic disease is characterized by IgG4+ plasma cell infiltration and fibrosis throughout many organs. We hypothesized that Riedel's thyroiditis is part of the IgG4-related systemic disease spectrum.Methods. We searched our institution's pathology database using the terms "Riedel's," "struma," "thyroid," and "fibrosis," and identified 3 cases of Riedel's thyroiditis. Riedel's thyroiditis was diagnosed if there was a fibroinflammatory process involving all or a portion of the thyroid gland, with evidence of extension of the process into surrounding tissues. Immunohistochemical stains for IgG4 and IgG were performed. The histopathologic and immunohistochemical features of each involved organ were evaluated. The clinical features of one patient with multiple organ system disease were described.Results. All 3 thyroidectomy samples stained positively for IgG4-bearing plasma cells. One patient had extensive extrathyroidal involvement diagnostic of IgG4-related systemic disease, including cholangitis, pseudotumors of both the lung and lacrimal gland, and a lymph node contiguous to the thyroid that stained intensely for IgG4+ plasma cells. The histologic features of all organs involved were consistent with IgG4-related systemic disease. Patient 3 had 10 IgG4+ plasma cells per high-power field initially, but rebiopsy 2 years later demonstrated no IgG4+ plasma cells. That patient's second biopsy, characterized by fibrosis and minimal residual inflammation, further solidifies the link between IgG4-bearing plasma cells in tissue and the histologic evolution to Riedel's thyroiditis.Conclusion. Riedel's thyroiditis is part of the IgG4-related systemic disease spectrum. In many cases, multifocal fibrosclerosis and IgG4-related systemic disease are probably the same entity.