Friedman Joshua, Marcovich Arie L, Kleinmann Guy, Schattner Ami
*Departments of Medicine and †Ophthalmology, Kaplan Medical Center, Rehovot, Hebrew University Hadassah Medical School, Jerusalem, Israel.
Cornea. 2014 Oct;33(10):1066-70. doi: 10.1097/ICO.0000000000000168.
Ocular cicatricial pemphigoid (OCP) is a relatively rare autoimmune disease affecting elderly patients and causing severe symptoms that may culminate in blindness. Treatment is based on immunosuppression, but optimal regimens have not been established.
A prospective unmasked case series of all patients with severe OCP who gave consent and completed treatment and ≥6 months of follow-up in the cornea and immunomodulation outpatient clinics of an academic hospital. Monthly pulses of intravenous cyclophosphamide (IVC; 500 mg) were administered with ondansetron and adjusted according to response.
Over 14 years, 13 patients (median age 77 years) met inclusion criteria. All had bilateral OCP (2 eyes were previously blind), but extraocular involvement was rare (1/13). Three to 28 pulses were given, and the patients were followed up for a median of 32 (range, 6-167) months. Remission of inflammation in both eyes was achieved in 12 patients (92%). Vision improved in 5 of 13 patients, stabilized in another 5 (combined, 77%), and worsened in only 3 patients. One patient's condition flared up during treatment that responded to steroids and increasing IVC frequency. In 4 patients and 6 eyes (25%), cicatrization progressed (usually, Foster stages 1-3). Two late relapses occurred and responded to retreatment. IVC was generally well tolerated, although nausea led to modification in 2 patients. One patient developed candida keratitis.
Compared with other treatment modalities, low-dose monthly pulse IVC is found to be a relatively safe, simple, and usually effective alternative immunosuppressive treatment in severe OCP.
眼部瘢痕性类天疱疮（OCP）是一种相对罕见的自身免疫性疾病，影响老年患者并导致严重症状，最终可能导致失明。治疗基于免疫抑制，但尚未确立最佳方案。
对一所学术医院角膜与免疫调节门诊中所有同意并完成治疗且随访≥6个月的重度OCP患者进行前瞻性非盲病例系列研究。每月静脉注射环磷酰胺（IVC；500mg），同时使用昂丹司琼，并根据反应进行调整。
在14年期间，13例患者（中位年龄77岁）符合纳入标准。所有患者均为双侧OCP（2只眼先前失明），但眼外受累情况罕见（1/13）。给予3至28次脉冲治疗，患者中位随访32（范围6 - 167）个月。12例患者（92%）双眼炎症缓解。13例患者中有5例视力改善，另外5例稳定（合计77%），仅3例患者视力恶化。1例患者在治疗期间病情复发，对类固醇和增加IVC频率有反应。4例患者的6只眼（占25%）出现瘢痕形成进展（通常为福斯特1 - 3期）。发生2次晚期复发，再次治疗有效。IVC总体耐受性良好，但有2例患者因恶心而调整治疗。1例患者发生念珠菌性角膜炎。
与其他治疗方式相比，低剂量每月脉冲IVC被发现是重度OCP中一种相对安全、简单且通常有效的替代性免疫抑制治疗方法。
