A Case of Peripheral Ulcerative Keratitis Associated with Autoimmune Hepatitis.

Hamoon Eshraghi,Mohammad H. Dastjerdi,Aria Mahtabfar

doi:10.1155/2017/3939413

Abstract

Purpose To describe a case of peripheral ulcerative keratitis in the setting of autoimmune hepatitis and possible overlap syndrome with primary sclerosing cholangitis. Case Report A 48-year-old African American female with autoimmune hepatitis with possible overlap syndrome with primary sclerosing cholangitis presented with tearing, irritation, and injection of the left eye that was determined to be peripheral ulcerative keratitis. The patient was treated with topical and systemic steroids, immunosuppressant drugs (azathioprine and mycophenolate mofetil), a biologic (rituximab), and surgery (conjunctival resection), and the peripheral ulcerative keratitis epithelialized but ultimately led to corneal perforation. Conclusion In this unique case, a patient with peripheral ulcerative keratitis who underwent treatment ultimately had a corneal perforation. This case may suggest a possible relationship between autoimmune hepatitis and peripheral ulcerative keratitis.

Highlights

Peripheral ulcerative keratitis (PUK) is an autoimmune condition believed to involve complement system activation via immune complexes in the peripheral cornea. e end result is a localized in ammatory response, leading to the enzymatic destruction of local cellular architecture; in particular, neutrophils and macrophages release collagenase and other proteases that destroy the corneal stroma [1]. e characteristic lesion is a crescent-shaped stromal in ammation of the juxtalimbal cornea. e etiology of PUK has not been completely ascertained, but consensus has centered on an autoimmune response to a localized antigen
We report a case of PUK in the setting of autoimmune hepatitis with possible overlap syndrome with primary sclerosing cholangitis (PSC)
The reported upregulation of T-box expressed in T cells (T-bet) and self-antigen recognition in autoimmune hepatitis create a plausible etiology leading to PUK [5]

Summary

Introduction

Peripheral ulcerative keratitis (PUK) is an autoimmune condition believed to involve complement system activation via immune complexes in the peripheral cornea. e end result is a localized in ammatory response, leading to the enzymatic destruction of local cellular architecture; in particular, neutrophils and macrophages release collagenase and other proteases that destroy the corneal stroma [1]. e characteristic lesion is a crescent-shaped stromal in ammation of the juxtalimbal cornea. e etiology of PUK has not been completely ascertained, but consensus has centered on an autoimmune response to a localized antigen. Peripheral ulcerative keratitis (PUK) is an autoimmune condition believed to involve complement system activation via immune complexes in the peripheral cornea. Given the in ammatory nature of the disease, PUK is often associated with other autoimmune conditions including rheumatoid arthritis and lupus. Ere have been cases of association between peripheral corneal ulcers and Mooren’s ulcer and viral hepatitis [2, 3].

Results

Conclusion