2016 - 1 Issue

Original article

Ocular Cicatricial Pemphigoid – a Retrospective Study

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Introduction: Ocular cicatricial pemphigoid (OCP) is rare, severe, sight threatening autoimmune disease of the conjunctiva, which affects elderly patients, more often women.

Aim: To evaluate the success rate of stabilisation of ocular findings in patients with OCP.

Methods: Retrospective study of patients from Centre of Conjunctival and Corneal Diseases at Department of Ophthalmology, General University Hospital and 1st Medical Faculty of Charles University in Prague in 1992-2013 was performed. Frequency of OCP clinical stages, visual acuity (VA), disease activity and ocular complications of referred patients were monitored. Moreover, type of immunosuppressive (IS) therapy, the number of relapses of the disease and progress of OCP clinical stages were evaluated. Especially, we evaluated effects and side effects of mycophenolate mofetil (MM) therapy. In addition to that, type and frequency of ocular surgery that was carried out to the patients before and after the referral were recorded. Furthermore, we evaluated percentage of patients with mucous membranous pemphigoid (MMP). Also, the positive yield of diagnostic methods was assessed.

Results: The OCP was diagnosed and monitored in 51 patients (21 men and 30 women) during 21 years, the average age on the day of diagnosis was 68,4 years, the average period of observation was 57 months. 55 % of eyes were referred to our department at clinical stage 3, 27 % at stage 4. VA was maintained in 76 % of eyes, improved in 5 % of eyes and in 19 % of eyes deteriorated. Activity of OCP was detected during the first examination in 96 % of patients, the most common complications at that time was corneal ulcer or perforation. Patients were treated by immunosuppressive therapy, most often in combination: corticosteroids (47 patients), azathioprine (28 patients), cyclophosphamide (25 patients), MM (16 patients), sulphasalazine (5 patients), dapsone (5 patients). We ascertained relapses in 40 % of patients. The progression to the next stage of OCP were found in 7 eyes (6,9 %) and 95 eyes (93,1 %) remained stable. Activity of disease was well controlled in 11 patients out of 16 (69 %) by MM, IS therapy of remain 5 patients (31 %) had to be changed. Side effects of MM such as lymphopenia were present in 1 patient. Before OCP was diagnosed, patients underwent cataract surgery with the intraocular lens implantation, cryoepilation of eyelashes and eyelid plastic surgery, especially entropion. The most common indicated surgery in our clinic was amniotic membrane transplantation and retro position of muscular cutaneous leaf. 31 % of patients were diagnosed with MMP. Positive results of conjunctival biopsy were detected in
48 % from 42 examined samples and 22 % from 32 examined samples had positive results of indirect immunofluorescence (anti-desmosoms).

Conclusion: OCP diagnosis is established on the basis of patient´s ophthalmic history and clinical findings. Positive results of direct and indirect immunofluorescence support the diagnosis. Activity of the disease and progression of OCP is effectively suppressed by systemic immunosuppressive therapy (for example MM), mainly if started at early stage of the disease.