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[惠普尔病双侧全葡萄膜炎:病例报告]

[Bilateral panuveitis in Whipple's disease: Case report].

作者信息

Loiseau V, Chopin M-C, Antoine P, Landrieux M, Moritz F

机构信息

Service d'ophtalmologie, hôpital Claude-Huriez, CHU de Lille, rue Michel-Polonowski, 59000 Lille, France.

Service maladies infectieuses et tropicales, centre hospitalier de Boulogne-Sur-Mer, rue Jacques-Monod, 62200 Boulogne-sur-Mer, France.

出版信息

J Fr Ophtalmol. 2024 Oct;47(8):104262. doi: 10.1016/j.jfo.2024.104262. Epub 2024 Aug 3.

Abstract

Whipple's disease is a rare disease linked to chronic infection with the intracellular gram-positive bacterium, Tropheryma whipplei. The clinical signs suggestive of this disease are the association of unexplained fever, lymphadenopathy, gastroenterological disorders (malabsorption) and inflammatory joint disorders (arthritis). However, isolated cardiological, neurological or ophthalmological forms have been described. We report the rare case of a 56-year-old patient complaining of floaters and recent visual loss, who presented with bilateral panuveitis in the absence of any systemic disorder. Clinical examination showed inflammation of the anterior segment, vitritis, inflammatory optic disc edema, focal retinitis, and venous vasculitis in both eyes. We describe the clinical characteristics and ancillary findings of the disease (fundus photos, visual field, auto-fluorescence, macular OCT, fluorescein and indocyanine green angiography). The diagnosis was made with the blood (T. whipplei) PCR test and with the help of accessory salivary gland biopsies. We describe the work-up leading to the diagnosis of Whipple's disease, the laboratory tests, and the recommended extended work-up. The patient's course was marked by complete resolution of the symptoms and clinical signs within a few months following corticosteroid therapy (1mg/kg/day) combined with hydroxychloroquine (600mg per day for 1 year) and life-long doxycycline therapy (200mg per day). In conclusion, this is a rare disease which should be discussed when dealing with steroid-resistant and/or steroid-dependent chronic uveitis with a negative work-up (especially in the presence of joint and/or digestive involvement).

摘要

惠普尔病是一种罕见疾病,与细胞内革兰氏阳性菌——惠普尔嗜组织细胞菌的慢性感染有关。提示该病的临床体征包括不明原因发热、淋巴结病、胃肠疾病(吸收不良)和炎症性关节疾病(关节炎)。然而,也有孤立的心脏、神经或眼科形式的病例报道。我们报告了一例罕见病例,一名56岁患者主诉有飞蚊症和近期视力丧失,在无任何全身疾病的情况下出现双侧全葡萄膜炎。临床检查显示双眼前段炎症、玻璃体炎、炎症性视盘水肿、局灶性视网膜病变和静脉血管炎。我们描述了该病的临床特征和辅助检查结果(眼底照片、视野、自发荧光、黄斑光学相干断层扫描、荧光素和吲哚菁绿血管造影)。通过血液(惠普尔嗜组织细胞菌)聚合酶链反应检测并借助辅助唾液腺活检做出诊断。我们描述了确诊惠普尔病的检查过程、实验室检查以及推荐的进一步检查。患者的病程特点是在接受皮质类固醇治疗(1毫克/千克/天)联合羟氯喹(每天600毫克,共1年)和终身强力霉素治疗(每天200毫克)后的几个月内,症状和临床体征完全缓解。总之,这是一种罕见疾病,在处理检查结果为阴性的类固醇抵抗和/或类固醇依赖的慢性葡萄膜炎时(特别是存在关节和/或消化系统受累时)应予以考虑。

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