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Jae Young You, Esen Akpek; Characterization of progressive cicatricial conjunctival disease with negative immunofluorescence. Invest. Ophthalmol. Vis. Sci. 2017;58(8):564.
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© ARVO (1962-2015); The Authors (2016-present)
Progressive cicatricial conjunctivitis with no detectable immunoreactant deposition in the basement membrane zone (BMZ) by direct immunofluorescence microscopy (DIF) is a subset of mucosal autoimmune disease that is poorly understood. This study aims to characterize the clinical features and outcomes of those patients who have undergone light microscopy (LM) and DIF examination of biopsied conjunctivas for suspected mucus membrane pemphigoid (MMP).
We created a list of all patients who have undergone diagnostic conjunctiva biopsy between January 2007 and October 2015 using the Current Procedural Terminology code 68100. 118 patients were identified. Of them, 30 patients who had a conjunctiva biopsy for cicatrizing conjunctivitis and had both LM and DIF examinations were included for a retrospective chart review. Information regarding demographics, clinical presentation, medical history and clinical course was collected. The primary outcome was the rate of the clinical diagnosis of MMP which was defined as progression of the conjunctival scarring in cases which initial conjunctival biopsy was negative for immunofluorescence.
Conjunctiva biopsy was consistent with MMP in three patients (3/30, 10.0%) with 3 months of mean presentation-to-treatment time (PTT) (range, 1 to 4 months). 26 patients (26/30, 86.7%) had negative DIF with mean follow-up of 40 months (range, 1-138 months). Eight of the patients (8/26, 30.8%) showed progression of conjunctival scarring. Two of the eight patients had positive repeat biopsy, 3 and 21 months after the initial biopsy; six patients required immunosuppression with mean PTT of 4 months (range, 1 to 7 months). Three other patients (3/26, 11.5%) were diagnosed with psoriasis (n=1), Sjogren’s syndrome (n=1) and sarcoidosis (n=1) based on microscopic examination of biopsied conjunctiva and required immunosuppression for progressive scarring in six months of PTT (range, 4 to 8 months). Remaining 15 patients with negative biopsy showed no signs of worsening disease over the mean follow-up of 25 months (range, 1-103 months). Six of the 15 patients had a follow-up that was less than one year.
Progressive conjunctival cicatrization is not uncommon in patients with negative DIF for auto-antibodies to BMZ. This study emphasizes the importance of vigilant clinical follow-up in all patients with cicatricial conjunctivitis to prevent vision loss.
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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