Clinical and imaging features of lacrimal gland involvement in granulomatosis with polyangiitis

Lee Teak Tan, Indran Davagnanam, Hazlita Mohd Isa, Geoffrey E. Rose, David H. Verity, Charles D. Pusey, Sue Lightman

Research output: Contribution to journalArticle

7 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)2125-2129
Number of pages5
JournalOphthalmology
Volume122
Issue number10
DOIs
Publication statusPublished - 1 Oct 2015

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Lacrimal Apparatus
Granulomatosis with Polyangiitis
Orbital Diseases
Biopsy
Antibodies
Ophthalmology
Tears
Early Diagnosis
Age Groups

ASJC Scopus subject areas

  • Ophthalmology

Cite this

Tan, L. T., Davagnanam, I., Mohd Isa, H., Rose, G. E., Verity, D. H., Pusey, C. D., & Lightman, S. (2015). Clinical and imaging features of lacrimal gland involvement in granulomatosis with polyangiitis. Ophthalmology, 122(10), 2125-2129. https://doi.org/10.1016/j.ophtha.2015.06.026

Clinical and imaging features of lacrimal gland involvement in granulomatosis with polyangiitis. / Tan, Lee Teak; Davagnanam, Indran; Mohd Isa, Hazlita; Rose, Geoffrey E.; Verity, David H.; Pusey, Charles D.; Lightman, Sue.

In: Ophthalmology, Vol. 122, No. 10, 01.10.2015, p. 2125-2129.

Research output: Contribution to journalArticle

Tan, LT, Davagnanam, I, Mohd Isa, H, Rose, GE, Verity, DH, Pusey, CD & Lightman, S 2015, 'Clinical and imaging features of lacrimal gland involvement in granulomatosis with polyangiitis', Ophthalmology, vol. 122, no. 10, pp. 2125-2129. https://doi.org/10.1016/j.ophtha.2015.06.026
Tan, Lee Teak ; Davagnanam, Indran ; Mohd Isa, Hazlita ; Rose, Geoffrey E. ; Verity, David H. ; Pusey, Charles D. ; Lightman, Sue. / Clinical and imaging features of lacrimal gland involvement in granulomatosis with polyangiitis. In: Ophthalmology. 2015 ; Vol. 122, No. 10. pp. 2125-2129.
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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.",
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N2 - 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.

AB - 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.

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