Lee Teak Tan1, Indran Davagnanam2, Hazlita Isa3, Geoffrey E Rose4, David H Verity5, Charles D Pusey6, Sue Lightman7. 1. University College London Institute of Ophthalmology, London, United Kingdom; Moorfields Eye Hospital, London, United Kingdom; Multidisciplinary Vasculitis Clinic, Hammersmith Hospital, London, United Kingdom. 2. Moorfields Eye Hospital, London, United Kingdom; Brain Repair & Rehabilitation Unit, Institute of Neurology, London, United Kingdom. 3. University College London Institute of Ophthalmology, London, United Kingdom; Moorfields Eye Hospital, London, United Kingdom; Multidisciplinary Vasculitis Clinic, Hammersmith Hospital, London, United Kingdom; Ophthalmology Department, University Kebangsaan Malaysia, Bangi, Selangor, Malaysia. 4. University College London Institute of Ophthalmology, London, United Kingdom; Moorfields Eye Hospital, London, United Kingdom. 5. Moorfields Eye Hospital, London, United Kingdom. 6. Multidisciplinary Vasculitis Clinic, Hammersmith Hospital, London, United Kingdom; Department of Ophthalmology, Imperial College, London, United Kingdom. 7. University College London Institute of Ophthalmology, London, United Kingdom; Moorfields Eye Hospital, London, United Kingdom; Multidisciplinary Vasculitis Clinic, Hammersmith Hospital, London, United Kingdom. Electronic address: s.lightman@ucl.ac.uk.
Abstract
PURPOSE: Lacrimal gland involvement in granulomatosis with polyangiitis (GPA) commonly accompanies orbital disease, but occasionally may be the sole presentation preceding any other organ manifestation or systemic disease. Diagnosis of orbital GPA, especially in patients with lacrimal involvement as the initial presentation, can be difficult because of nonspecific clinical features and lack of diagnostic specificity on histologic and antineutrophilic cytoplasmic antibody (ANCA) testing. Orbital GPA can be associated with a high morbidity from potential visual loss or rapid progression of latent systemic disease, making early diagnosis important. The purpose of this study was to describe the clinical and imaging features of patients with lacrimal gland involvement secondary to GPA and to compare them with those of other orbital inflammatory conditions in the lacrimal gland fossa. DESIGN: Retrospective, noninterventional comparative case series. PARTICIPANTS: Two hundred forty-seven patients who had undergone orbital biopsy over a 21-year period were identified from the Institute of Ophthalmology Pathology database. Sixty-nine patients were found to have orbital inflammatory disease with lacrimal gland involvement, of whom 7 had a final diagnosis of GPA. METHODS: Clinical and imaging features of patients with GPA were analyzed and compared with those of the non-GPA group. MAIN OUTCOME MEASURES: Features associated with GPA. RESULTS: The median age at presentation for GPA patients was 30 years (mean ± standard deviation, 36.7±16.7 years; range, 14-57 years). The interval from presentation to definitive diagnosis of GPA ranged from 3 to 20 months (mean, 12.1 months; median, 12 months). Sinonasal involvement was demonstrated in 43% and bony changes were demonstrated in 29% of patients with GPA. A higher proportion of patients with GPA demonstrated sinonasal involvement (P = 0.011) and bony destruction (P = 0.048) compared with non-GPA patients. CONCLUSIONS: Associated sinonasal involvement and bony changes on imaging are highly suggestive of GPA and should prompt a full diagnostic workup. A high index of suspicion should be maintained, with repeated ANCA testing, biopsy, and imaging where indicated, especially in the younger age group.
PURPOSE: Lacrimal gland involvement in granulomatosis with polyangiitis (GPA) commonly accompanies orbital disease, but occasionally may be the sole presentation preceding any other organ manifestation or systemic disease. Diagnosis of orbital GPA, especially in patients with lacrimal involvement as the initial presentation, can be difficult because of nonspecific clinical features and lack of diagnostic specificity on histologic and antineutrophilic cytoplasmic antibody (ANCA) testing. Orbital GPA can be associated with a high morbidity from potential visual loss or rapid progression of latent systemic disease, making early diagnosis important. The purpose of this study was to describe the clinical and imaging features of patients with lacrimal gland involvement secondary to GPA and to compare them with those of other orbital inflammatory conditions in the lacrimal gland fossa. DESIGN: Retrospective, noninterventional comparative case series. PARTICIPANTS: Two hundred forty-seven patients who had undergone orbital biopsy over a 21-year period were identified from the Institute of Ophthalmology Pathology database. Sixty-nine patients were found to have orbital inflammatory disease with lacrimal gland involvement, of whom 7 had a final diagnosis of GPA. METHODS: Clinical and imaging features of patients with GPA were analyzed and compared with those of the non-GPA group. MAIN OUTCOME MEASURES: Features associated with GPA. RESULTS: The median age at presentation for GPA patients was 30 years (mean ± standard deviation, 36.7±16.7 years; range, 14-57 years). The interval from presentation to definitive diagnosis of GPA ranged from 3 to 20 months (mean, 12.1 months; median, 12 months). Sinonasal involvement was demonstrated in 43% and bony changes were demonstrated in 29% of patients with GPA. A higher proportion of patients with GPA demonstrated sinonasal involvement (P = 0.011) and bony destruction (P = 0.048) compared with non-GPA patients. CONCLUSIONS: Associated sinonasal involvement and bony changes on imaging are highly suggestive of GPA and should prompt a full diagnostic workup. A high index of suspicion should be maintained, with repeated ANCA testing, biopsy, and imaging where indicated, especially in the younger age group.