272. Ocular Manifestations of ANCA-Associated Vasculitis

Background: ANCA-associated vasculitides (AAV) are multisystem diseases that can have multiple ophthalmic manifestations.  Although there are some data on ocular disease in granulomatosis with polyangiitis (GPA), even less are available for microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA).  Further, there also few reports differentiating symptoms seen at disease onset versus later in the disease or the ocular complications of AAV.

Methods: Patients with GPA, MPA, or EGPA enrolled in a longitudinal study between April 2006 and April 2021 were included in this study.  Data concerning diagnosis, demographics, cranial disease manifestations and their time of onset, treatment, and ocular complications were extracted.  Prevalence of ophthalmic manifestations at disease onset and incidence of manifestations over the course of follow up, median time to onset of new manifestations and complications of disease were calculated.

Results: Data from 1389 patients were included for analysis which included 6392.8 patient-years of follow up.  There were 852 cases of GPA, 165 cases of MPA, and 372 cases of EGPA; with 258 (30.3%), 7 (4.2%), and 13 (3.5%) ocular manifestations present at baseline, respectively (Table 1).  The most common manifestations seen were conjunctivitis/episcleritis and scleritis; multiple ophthalmic manifestations were seen in 79 (9.3%) of patients with GPA, 3 (1.8%) patients with MPA, and none with EGPA.  During follow up, 56 (6.6%) patients with GPA had incident ocular manifestations (of which 53.6% were new manifestations), while such events were rare in MPA (n=1) and EGPA (n=2). Frequent manifestations seen during follow up were conjunctivitis/episcleritis and dacrocystitis/lacrimal duct obstruction. The most common complication seen across all 3 diseases was cataracts, seen in 9.1-15.3% of patients.  Non-cataract complications followed a similar pattern to other manifestations: 67 (7.9%) patients with GPA experienced such complications (of whom 31 experienced vision threatening complications) followed by 10 (2.7%) of those with EGPA, and 7 (4.2%) of those with MPA.  Optic Neuritis (n=8) and orbital wall destruction (n=12) were only seen in those with GPA; 8 individuals with GPA experienced blindness as well as one with MPA.

Conclusion: Among patients with AAV, ophthalmic manifestations and complications are common in GPA, but rare in MPA and EGPA.  Inflammatory eye conditions are the most common ophthalmic manifestation seen, and cataracts are the most common complication.  New ophthalmic manifestations after disease onset are rare.  These data are informative for clinicians caring for patients with AAV and investigators studying this spectrum of vasculitis.

Disclosures:

Authors MJ, SG, LZ, DC, CK, CL, CM, LM – no disclosures. CP – Consultant, speaker and recipient of research support from GlaxoSmithKline, Roche, Otuska, Pfizer, Chemocentryx, and Astrazeneca. PM – Advisory board for Kiniksa and Chemocentryx, Consultant for Celgene

PS – Advisory/Review panel for Amgen and Janssen. US – Consultant and recipient of research support for ChemoCentryx; recipient of research support from Genentech.

AS – Employed by Bristol-Myers Squibb, Stock options in Alexion. KW – Recipient of research support from Eli Lilly and Kiniksa. PM – Consultant and recipient of research support for AbbVie, AstraZeneca, Boeringher-Ingelheim, Bristol-Myers Squibb, ChemoCentryx, Forbius, Genentech/Roche, Genzyme/Sanofi, GlaxoSmithKline, InflaRx, Consultant for CSL Behring, Dynacure, Eicos, EMDSerono, Forbius, Janssen, Kiniksa, Magneta, Neutrolis, Novartis, Pfizer, Star Therapeutics Takeda, Talaris, Recipient of research support from Sanofi, Receives royalties from UpToDate. NK – Trial support from Roche, BMS, Sanofi, Abbvie