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. 2023 Jul 5;62(7):2517-2524.
doi: 10.1093/rheumatology/keac663.

Ocular manifestations of ANCA-associated vasculitis

Mats L Junek  1 , Lily Zhao  2 , Stephanie Garner  1 , David Cuthbertson  3 , Christian Pagnoux  4 , Curry L Koening  5 , Carol A Langford  6 , Carol A McAlear  7 , Paul A Monach  8 , Larry W Moreland  9 , Rennie L Rhee  7 , Philip Seo  10 , Ulrich Specks  11 , Antoine G Sreih  7 , Kenneth Warrington  11 , Michael E Wechsler  12 , Peter A Merkel  7 , Nader A Khalidi  1
Affiliations

Ocular manifestations of ANCA-associated vasculitis

Mats L Junek et al. Rheumatology (Oxford). .

Abstract

Objectives: ANCA-associated vasculitis (AAV) is a group of multisystem diseases that can have several ocular manifestations. There are published data on ocular manifestations of granulomatosis with polyangiitis (GPA), but few for eosinophilic granulomatosis with polyangiitis (EGPA) or microscopic polyangiitis (MPA). There is little information concerning chronicity, complications, and association with other cranial manifestations of AAV.

Methods: This study retrospectively analysed longitudinal multicentre cohorts of individuals with AAV followed between 2006 and 2022. Data included diagnosis, demographics, cranial manifestations of disease, presence of manifestations at onset of disease and/or follow-up, and ocular complications of disease. Univariate and multivariable logistic regression analysis assessed associations across disease manifestations.

Results: Data from 1441 patients were analysed, including 395 with EGPA, 876 with GPA, and 170 with MPA. Ocular manifestations were seen within 23.1% of patients: 39 (9.9%) with EGPA, 287 (32.7%) with GPA, and 12 (7.1%) with MPA at any time in the disease course. There were more ocular manifestations at onset (n = 224) than during follow-up (n = 120). The most common disease-related manifestations were conjunctivitis/episcleritis and scleritis. In multivariable analysis, dacryocystitis, lacrimal duct obstruction, and retro-orbital disease were associated with sinonasal manifestations of GPA; ocular manifestations were associated with hearing loss in MPA. The most common ocular complications and/or damage seen were cataracts (n = 168) and visual impairment (n = 195).

Conclusion: Ocular manifestations occur in all forms of AAV, especially in GPA. Clinicians should be mindful of the wide spectrum of ocular disease in AAV, caused by active vasculitis, disease-associated damage, and toxicities of therapy.

Keywords: ANCA; epidemiology; ocular disease; vasculitis.

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Figures

Figure 1.
Figure 1.
Ocular, sinonasal, and CNS manifestations of granulomatosis with polyangiitis
Figure 2.
Figure 2.
Ocular complications of ANCA-associated vasculitis (AAV) stratified by diagnosis. Bar labels are frequency of findings. EGPA: eosinophilic granulomatosis with polyangiitis; GPA: granulomatosis with polyangiitis; MPA: microscopic polyangiitis
See this image and copyright information in PMC

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