J Korean Ophthalmol Soc.  2017 Oct;58(10):1115-1121. 10.3341/jkos.2017.58.10.1115.

A Retrospective Analysis of Granulomatosis with Polyangiitis with Ocular Manifestations

• Kim BR
• Seo Y
• Yoon JS

• KMID: 2393162
• DOI: http://doi.org/10.3341/jkos.2017.58.10.1115

Abstract

PURPOSE
To analyze the treatment and prognosis of patients from our tertiary medical center with ocular and orbital involvement of granulomatosis with polyangiitis.
METHODS
A retrospective analysis of the medical records of patients diagnosed with granulomatosis with polyangiitis that visited our single tertiary referral center from July 2008 to September 2014 was performed.
RESULTS
A total of 51 patients diagnosed with granulomatosis with polyangiitis visited our center, and 21 of those patients had received an ophthalmologic examination. Of these, 9 patients (4 males, 5 females) had symptoms of the eye and orbit, and the clinical presentations were as follows: episcleritis, scleritis, marginal keratitis, orbital inflammation, orbital abscess, retinal vasculitis, and nasolacrimal duct obstruction. The patients each received treatments according to clinical presentation with topical, oral, or intravenous steroids or immunomodulatory agents such as cyclophosphamide. Nasolacrimal duct obstruction was treated with surgery in some cases. After an average follow-up period of 58 ± 30 months, all patients showed clinical improvement of their ocular and orbital involvement of granulomatosis with polyangiitis.
CONCLUSIONS
Granulomatosis with polyangiitis is a relatively rare disease that sometimes has ocular or orbital involvement and can lead to blindness. Therefore, when ocular symptoms and signs present without a definitive cause, granulomatosis with polyangiitis must be ruled out, and appropriate treatment is needed. However, there are few published reports on the clinical presentation and prognosis of ocular and orbital involvement of granulomatosis with polyangiitis in Asians. This study showed that the incidence of ocular and orbital involvement in granulomatosis with polyangiitis was lower than previous reports.

Keyword

Granulomatosis with polyangiitis; Ocular involvement; Wegener's granulomatosis

MeSH Terms

Abscess
Asian Continental Ancestry Group
Blindness
Cyclophosphamide
Follow-Up Studies
Granulomatosis with Polyangiitis*
Humans
Incidence
Inflammation
Keratitis
Male
Medical Records
Nasolacrimal Duct
Orbit
Prognosis
Rare Diseases
Retinal Vasculitis
Retrospective Studies*
Scleritis
Steroids
Tertiary Care Centers
Cyclophosphamide
Steroids

Figure

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  Figure 1. Case 7. This 68-year-old woman presented with epiphora. She had a history of sinusitis and periorbital inflammation. (A) A photograph showing the collapse of the nasal bridge, also known as the ‘saddle nose’. (B) An endoscopic view of the right nasal cavity shows friable mucosa and easy bleeding. (C, D) The coronal and axial views of contrast computed tomography show chronic inflammatory changes in form of the destruction of the nasal septum and ethmoid sinus (yellow arrow).

Reference

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