Scleritis is a severe and painful ophthalmic disorder, in which a pathogenic role for collagen-directed autoimmunity was repeatedly suggested. We evaluated the presence of sclera-specific antibodies in a large cohort of patients with non-infectious scleritis. Therefore, we prospectively collected serum samples from 121 patients with non-infectious scleritis in a multicenter cohort study in the Netherlands. In addition, healthy (n = 39) and uveitis controls (n = 48) were included. Serum samples were tested for anti-native human type II collagen antibodies using a validated enzyme-linked immunosorbent assay (ELISA). Further, sclera-specific antibodies were determined using indirect immunofluorescence (IIF) on primate retinal/scleral cryosections. Lastly, human leukocyte antigen (HLA) typing was performed in 111 patients with scleritis. Anti-type II collagen antibodies were found in 13% of scleritis patients, in 10% of healthy controls and in 11% of uveitis controls (p = 0.91). A specific reaction to scleral nerve tissue on IIF was observed in 33% of patients with scleritis, which was higher than in healthy controls (11%; p = 0.01), but similar to uveitis controls (25%; p = 0.36). Reactivity to the scleral nerve tissue was significantly associated with earlier onset of scleritis (48 versus 56 years; p < 0.001), bilateral involvement (65% versus 42%; p = 0.01), and less frequent development of scleral necrosis (5% versus 22%; p = 0.02). HLA-B27 was found to be twice as prevalent in patients with scleritis (15.3%) compared to a healthy population (7.2%). In conclusion, scleral nerve autoantibody reactivity was more common in scleritis and uveitis patients in contrast to healthy controls. Further research is needed to characterize these scleral-nerve directed antibodies and assess their clinical value.

中文翻译：

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巩膜炎中巩膜特异性自身免疫之谜

巩膜炎是一种严重且痛苦的眼科疾病，其中胶原蛋白导向的自身免疫的致病作用被反复提出。我们评估了一大群非感染性巩膜炎患者中巩膜特异性抗体的存在情况。因此，我们在荷兰进行的一项多中心队列研究中前瞻性地收集了 121 名非感染性巩膜炎患者的血清样本。此外，还包括健康对照（n = 39）和葡萄膜炎对照（n = 48）。使用经过验证的酶联免疫吸附测定 (ELISA) 检测血清样本中的抗天然人类 II 型胶原蛋白抗体。此外，使用间接免疫荧光（IIF）对灵长类动物视网膜/巩膜冷冻切片测定巩膜特异性抗体。最后，对 111 名巩膜炎患者进行了人类白细胞抗原 (HLA) 分型。 13% 的巩膜炎患者、10% 的健康对照者和 11% 的葡萄膜炎对照者中发现了抗 II 型胶原抗体 (p = 0.91)。在 33% 的巩膜炎患者中观察到 IIF 对巩膜神经组织的特异性反应，该反应高于健康对照者（11%；p = 0.01），但与葡萄膜炎对照者相似（25%；p = 0.36）。对巩膜神经组织的反应性与巩膜炎较早发作（48 岁与 56 岁；p < 0.001）、双侧受累（65% 与 42%；p = 0.01）以及巩膜坏死发生频率较低（5% 与 5%）显着相关。 22%；p = 0.02)。研究发现，巩膜炎患者 (15.3%) 的 HLA-B27 患病率是健康人群 (7.2%) 的两倍。总之，与健康对照相比，巩膜神经自身抗体反应在巩膜炎和葡萄膜炎患者中更常见。需要进一步的研究来表征这些巩膜神经定向抗体并评估其临床价值。