Surgical outcomes and unique histological features of tonsils after tonsillectomy in adults with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome
Surgical outcomes and unique histological features of tonsils after tonsillectomy in adults with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome
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DOI：
10.1016/j.anl.2019.08.009
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发表时间：
2020-04-01
影响因子：
1.7
通讯作者：
Ikegami, Satoshi
中科院分区：
文献类型：
--
作者：
Yamahara, Kohei;Lee, Kana;Ikegami, Satoshi
目的：关于成人发作的周期性发热、口疮性口炎、咽炎和宫颈腺炎（PFAPA）综合征的资料很少。欧洲研究报道，与儿科发病的PFAPA不同，扁桃体切除术对成人发病的PFAPA无效。本研究的目的是(1)评估日本成年发病PFAPA患者对扁桃体切除术的反应，(2)评估成年发病PFAPA患者扁桃体的组织学外观，并将其与年龄和性别匹配的慢性扁桃体炎对照组的扁桃体进行比较。方法：在这项回顾性队列研究中，招募了5名成年PFAPA患者和15名接受扁桃体切除术的对照组。以CD3、CD4、CD8为T细胞标记物，CD20为B细胞标记物，采用免疫组化方法测定扁桃体生发中心的大小，测定生发中心B淋巴细胞和T淋巴细胞的数量和密度。结果：所有患者术后症状均完全缓解。PFAPA患者生发中心面积明显小于对照组。与对照组相比，PFAPA组扁桃体生发中心CD8+细胞的数量和密度显著降低。两组间CD3+、CD4+和CD20+细胞均无差异。这些结果与儿科发病PFAPA的扁桃体特征相一致。结论：我们的报告表明扁桃体切除术可能对成人发病的PFAPA有效，并且成人和儿科发病的PFAPA扁桃体具有相同的组织学特征。这些结果表明，成人和儿童发病的PFAPA的致病机制是相同的。(C) 2019 Elsevier B.V.版权所有
Objectives: Data on the adult-onset periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome are scarce. European studies reported that unlike pediatric-onset PFAPA, tonsillectomy is ineffective for adult-onset PFAPA. The aims of this study were (1) to assess the response to tonsillectomy in a cohort of Japanese adult-onset PFAPA patients and (2) to evaluate the histologic appearance of tonsils in adult-onset PFAPA patients and to compare them with those of tonsils from age- and sex-matched controls with chronic tonsillitis.Methods: In this retrospective cohort study, 5 adults with PFAPA and 15 controls who had undergone tonsillectomy were recruited. The size of the tonsil germinal centers was measured by hematoxylin and eosin staining, and the number and density of B and T lymphocytes in germinal centers were measured by immunohistochemistry, using CD3, CD4 and CD8 as T cell markers and CD20 as B cell marker.Results: All patients had complete remission of the symptoms after surgery. PFAPA patients had significantly smaller germinal center areas than controls. The number and density of CD8+ cells in germinal centers were significantly lower in tonsils from PFAPA compared with controls. No differences were found between the two groups in CD3+, CD4+, and CD20+ cells. These results are compatible with the tonsillar features of pediatric-onset PFAPA.Conclusion: Our report demonstrates that tonsillectomy might be effective for adult-onset PFAPA and that tonsils of adult- and pediatric-onset PFAPA share the same histological features. These results suggest that the pathogenic mechanisms of adult- and pediatric-onset PFAPA are identical. (C) 2019 Elsevier B.V. All rights reserved.