Moorfields Eye Hospital NHS Foundation Trust, London, UK.
Eye (Lond). 2012 Sep;26(9):1199-208. doi: 10.1038/eye.2012.119. Epub 2012 Jun 22.
Cicatrising conjunctival disorders are uncommon, and are difficult to diagnose and manage. This study was designed to assess the annual incidence and underlying diagnosis of patients with cicatrising conjunctivitis (CC) within the United Kingdom.
Clinical data of newly diagnosed cases of CC were reported via the British Ophthalmological Surveillance Unit at diagnosis and at 12 months follow-up.
A total of 50 (61%) ocular mucous membrane pemphigoid (OcMMP), 16 (20%) Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS-TEN) and 16 (20%) other causes of CC, equating to an incidence of 0.8, 0.2, and 0.2 per million, respectively, were reported. Although diagnosis of SJS-TEN was usually within a median of 7 days of symptom-onset, that for OcMMP and other CC was a median 225 days for both. At diagnosis, 64/163 (39%) eyes had moderate/severe conjunctival inflammation, and 102/164 (62%) had symblepharon formation. Although 43/82 (52%) patients were commenced on immunosuppression or had this therapy modified, at follow-up there was an increase in the number of symblepharon, despite control of inflammation (P<0.001). Mortality only occurred in the SJS-TEN group (4/16 (25%)).
CC has a substantial morbidity and for non-SJS-TEN causes, diagnosis is frequently delayed. The proportion of patients given immunosuppressive therapy to prevent disease progression may be less than optimal. These data highlight the need for developing patient access to specialist-designated centres with expertise in CC.
结膜瘢痕化疾病并不常见,且诊断和治疗较为困难。本研究旨在评估英国结膜瘢痕化(CC)患者的年发病率和潜在诊断。
通过英国眼科监测单位在诊断时和 12 个月随访时报告新诊断为 CC 的患者的临床数据。
共报告了 50 例(61%)眼黏膜类天疱疮(OcMMP)、16 例(20%)史蒂文斯-约翰逊综合征/中毒性表皮坏死松解症(SJS-TEN)和 16 例(20%)其他 CC 病因,发病率分别为 0.8、0.2 和 0.2 百万分之,中位数为 225 天。尽管 SJS-TEN 的诊断通常在症状出现后 7 天内,但 OcMMP 和其他 CC 的诊断均为 225 天。诊断时,64/163(39%)只眼有中度/重度结膜炎症,102/164(62%)有睑球粘连形成。尽管 43/82(52%)患者开始接受免疫抑制治疗或改变了治疗方案,但在随访时,尽管炎症得到控制,仍有更多的睑球粘连形成(P<0.001)。仅在 SJS-TEN 组发生了死亡(4/16(25%))。
CC 具有较高的发病率,而非 SJS-TEN 病因的患者诊断常常被延误。为预防疾病进展而给予免疫抑制治疗的患者比例可能不理想。这些数据强调了需要为 CC 患者提供专门指定的专家中心,以获得更好的治疗。