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Jing Hua, Aseef Ahmed, Karen Small, Tongzhen Zhao, Stephen D Anesi, C. Stephen Foster; Effective Immunopathology Protocol for Diagnostic Challenges of Patients Suspected of Having Ocular Citatricial Pemphigoid (OCP), the MERSI Experience. Invest. Ophthalmol. Vis. Sci. 2017;58(8):3359.
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© ARVO (1962-2015); The Authors (2016-present)
Immunopathology diagnosis is crucial for therapeutic decisions of patients with OCP. Timely immune modulatory therapy (IMT) can prevent sight-robbing progression of OCP, however, a false-negative biopsy can wrongfully deprive the patient of possible IMT with disastrous consequences. To address this diagnostic challenge, we developed an immunopathology protocol for assessment of conjunctiva from patients with citatricial conjunctivatis.
We reviewed 136 consecutive OCP suspect patients, who received conjunctival biopsy between 2008 and 2014 at MERSI. Frozen sections of specimens were stained with H&E, PAS and Giemsa; as well as direct immunofluorescent (DIF) for IgA, IgG, IgD, IgM, IgE, C3 and C4; albumin and fibrinogen served as negative and positive controls. In high suspect DIF negative or inconclusive cases, supplement avidin-biotin complex (ABC) immunoperoxidase staining for the same antigens was performed. We also reviewed clinical characteristics, history of previous biopsy, biopsy outcomes, treatment, and clinical response to therapy of these patients.
Among 136 patients, 108 (79.4%) were diagnosed with OCP, and 42 of these 108 (38.9%) required use of ABC to confirm the diagnosis. Sensitivity increased from 68.8% with DIF to 96.4% with DIF and ABC. There are 66 OCP positive patients with available follow up who required IMT; 62 (94%) achieved durable remission. Among 42 who were diagnosed using ABC, 17 with available follow up required IMT, and all 17 (100%) achieved remission. Of all 136 patients, 38 had previous negative biopsy elsewhere (27.9%) before referral to MERSI, 32 were diagnosed with OCP at MERSI (84.2%), and 10 of these 32 required ABC (31.3%). Seven of these 10 with available follow up required IMT, and all 7 (100%) achieved remission.
The MERSI protocol profoundly reduced false-negative diagnosis for OCP patients, and is therefore, crucial for the clinical outcome. Our protocol employs a panel of multiple antibodies with positive and negative controls and a two-stage assessment, which may be adopted for other referral centers.
This is an abstract that was submitted for the 2017 ARVO Annual Meeting, held in Baltimore, MD, May 7-11, 2017.
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