Orbital manifestations of granulomatosis with polyangiitis: 12-year experience in Mexico City

ABSTRACT

Purpose

To present the clinical picture and radiological characteristics of orbital manifestations of granulomatosis with polyangiitis in a Mexican hospital and compare them with worldwide literature.

Methods

Retrospective, observational study from January 2007 to January 2019. An electronic file review was performed. All patients with the diagnosis of granulomatosis with polyangiitis (GPA) in the Oculoplastics department were included. Ophthalmological examination, biopsy, antibodies and tomographical results were included in the data collected. Descriptive statistics were obtained.

Results

One hundred and one patients in our institute had a diagnosis of GPA. Only 15 (14.8%) had orbital manifestations and were included in our study. 73.3% were female with a median age of 46.20 years (17–81). Diagnostic delay was on average 6 months. Only 6.7% had bilateral manifestations. No past medical history was found in 40%, 20% had a previous diagnosis of systemic GPA. Pain was reported in 73.3%. Increase of volume (proptosis or diffuse orbital mass) was present in 86.7%. C-ANCA antibodies were positive in seven patients (46.7%). In tomography, lacrimal gland involvement was present in 33.3% and diffuse orbital mass was present in 66.6%. Definite diagnosis was done with biopsy in 93.3%. One patient died from complications of GPA.

Conclusions

Ophthalmologists should consider this rare disease as a differential diagnosis of orbital tumors, as it may have different clinical manifestations, even in non-Caucasian population. When in doubt, biopsy is always valuable. The statistics at our reference center correspond with statistics reported worldwide.

KEYWORDS:

• Granulomatosis with polyangiitis
• Orbital granulomatosis
• orbital inflammation
• orbital tumors
• Wegener disease

Acknowledgemnts

To the past medical generations of Instituto Conde de Valenciana for their valuable examination of patients that allowed us to gather this information.

To Dr. Abelardo Rodriguez for the histopathological images.

To. Dr. Ana Gloria García Rodríguez, for her help with tables and images.

Disclosure statement

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.