Choi Rene Y, Shakoor Akbar, Bohnsack John, Vitale Albert T
Department of Ophthalmology and Visual Sciences, John A. Moran Eye Center, University of Utah, Salt Lake City, Utah.
Department of Pediatric Immunology and Rheumatology, University of Utah, Salt Lake City, Utah.
Retin Cases Brief Rep. 2019 Fall;13(4):367-370. doi: 10.1097/ICB.0000000000000600.
BACKGROUND/PURPOSE: To report two novel cases of intermediate uveitis associated with Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis syndrome.
Observational case reports and review of the literature.
Both patients in this report had an established diagnosis of Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis syndrome before the onset of ocular inflammation. Infectious and noninfectious systemic conditions known to be associated with intermediate uveitis were excluded. Intermediate uveitis was confirmed clinically in both patients by the presence of vitritis, snowballs, and peripheral snowbanks in the region of the pars plana. Both cases had a course characterized by recurrent inflammation; in which systemic steroid treatment, and in one case, immunomodulatory therapy was necessary.
Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis syndrome is an auto-inflammatory fever disorder in childhood. Although other auto-inflammatory disorders such as, Blau syndrome, Muckle-Wells syndrome, and Behcets disease have been associated with various forms of uveitis, Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis has never been reported to be associated with any type of ocular inflammation. We describe for the first time, two cases of Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis syndrome presenting with intermediate uveitis.
背景/目的：报告两例与周期性发热、阿弗他口炎、咽炎和颈淋巴结炎综合征相关的中间葡萄膜炎新病例。
观察性病例报告及文献复习。
本报告中的两名患者在眼部炎症发作前均已确诊周期性发热、阿弗他口炎、咽炎和颈淋巴结炎综合征。已知与中间葡萄膜炎相关的感染性和非感染性全身疾病均被排除。两名患者经临床检查均确诊为中间葡萄膜炎，表现为玻璃体炎、雪球样病变以及在睫状体平坦部区域出现周边雪堤样改变。两例病例病程均以炎症反复发作为特征；其中全身应用类固醇治疗，一例还需要免疫调节治疗。
周期性发热、阿弗他口炎、咽炎和颈淋巴结炎综合征是儿童期的一种自身炎症性发热性疾病。虽然其他自身炎症性疾病如Blau综合征、Muckle-Wells综合征和白塞病已与多种形式的葡萄膜炎相关，但周期性发热、阿弗他口炎、咽炎和颈淋巴结炎从未被报道与任何类型的眼部炎症相关。我们首次描述了两例表现为中间葡萄膜炎的周期性发热、阿弗他口炎、咽炎和颈淋巴结炎综合征病例。
