ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 1947

Ocular Manifestations of ANCA-Associated Vasculitis

Sophie Cai1,2, George Papaliodis2,3, Leo Lu4, Hyon K. Choi5, Ulrich Specks6, Peter A. Merkel7, Philip Seo8, Robert F. Spiera9, Carol A. Langford10, Gary S. Hoffman11, Cees G.M. Kallenberg12, William St Clair13, Nadia Tchao14, Fernando Fervenza6, Paul A. Monach15, W Joseph McCune16, John H. Stone17, Eli Miloslavsky18 and RAVE-ITN and WGET Research Groups, 1Ophthalmology, Johns Hopkins Wilmer Eye Institute, Baltimore, MD, 2Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, MA, 3Uveitis and Immunology, Massachusetts Eye and Ear, Boston, MA, 4Allergy, Immunology, and Rheumatology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, 5Rheumatology, Allergy, and Immunology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, 6Mayo Clinic, Rochester, MN, 7Division of Rheumatology, University of Pennsylvania, Philadelphia, PA, 8Medicine, Johns Hopkins University, Baltimore, MD, 9Hospital for Special Surgery, Cornell, New York, NY, 10Rheumatic and Immunologic Diseases, Cleveland Clinic, Cleveland, OH, 11Rheumatology, Cleveland Clinic, Cleveland, OH, 12Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, Netherlands, 13Rheumatology, Duke University Medical Center, Durham, NC, 14Immune Tolerance Network, San Francisco, CA, 15Rheumatology, Boston University School of Medicine, Boston, MA, 16Int Med/ Rheum, University of Michigan, Ann Arbor, MI, 17Massachusetts General Hospital Rheumatology Unit, Harvard Medical School, Boston, MA, 18Division of Rheumatology, Massachusetts General Hospital, Harvard Medical School, Boston, MA

Meeting: 2016 ACR/ARHP Annual Meeting

Date of first publication: September 28, 2016

Keywords: , ,

Session Information

Date: Monday, November 14, 2016

Title: Vasculitis - Poster II: ANCA-Associated Vasculitis

Session Type: ACR Poster Session B

Session Time: 9:00AM-11:00AM

Background/Purpose: Ocular involvement in ANCA-associated vasculitis (AAV) can cause substantial morbidity. We aimed to characterize patterns of ocular involvement in AAV and their response to treatment.

Methods:  We analyzed data from two large randomized controlled trials of AAV – the Rituximab in ANCA-Associated Vasculitis (RAVE) trial and Wegener’s Granulomatosis Etanercept Trial (WGET). Ocular involvement was assessed at baseline and patients were followed until the common close-out dates. Pooled post-hoc analysis was performed of initial and overall prevalences of ocular involvement. Clinical features associated with ocular involvement, time to remission, and relapse were assessed by Cox proportional hazards and logistic regression models.

Results: 377 patients were included (Table 1). 64 patients (17.0%) had ocular involvement at study entry; conjunctivitis/episcleritis was the most common subtype, while uveitis and retinal involvement were the rarest (Table 2). Over a median follow-up time of 35.4 months, 24 patients (6.4%) developed new ocular involvement. Non-white race was associated with higher odds of having retroorbital mass/proptosis (OR 4.51; P = 0.014). Female sex (OR 2.70; P = 0.032) and PR3 ANCA positive status (OR 4.48; P = 0.048) were associated with higher odds of having scleritis. Among patients with ocular disease at study entry, median times to remission were 1.3 months for conjunctivitis/episcleritis, 1.4 months for retroorbital mass/proptosis, 0.2 months for scleritis, and 0.3 months for retinal exudates/hemorrhage. Treatment with rituximab trended toward association with decreased time to remission of retroorbital mass/proptosis (HR 10.0; P = 0.060). Relapses of ocular disease occurred in 5 patients (12.5%) with conjunctivitis/episcleritis, 3 patients (25.0%) with retroorbital mass/proptosis, and 6 patients (37.5%) with scleritis. No patient treated with rituximab developed a relapse of conjunctivitis/episcleritis or proptosis.

Conclusion: Conjunctivitis/episcleritis, scleritis, and retroorbital mass/proptosis were the most common ocular manifestations of AAV. Race and sex may be associated with certain subtypes of ocular involvement. The relative efficacies of cyclophosphamide and rituximab in the treatment of retroorbital mass/proptosis deserve further study.

Table 1. Study population baseline characteristics (N=377)
Age (years), mean (SD)  51.4 (15.5)
Sex, male, n (%) 208 (55.2)
Race, white, n (%) 350 (92.8)
New diagnosis, n (%) 176 (46.7)
Limited disease, n (%) 52 (13.8)
WG diagnosis, n (%) 327 (87.0)
PR3 ANCA positive, n (%) 277 (74.5)
MPO ANCA positive, n (%) 70 (18.8)
RAVE trial participant, n (%) 197 (52.3)
Treatment with rituximab, n (%) 99 (26.3)
WGET trial participant, n (%) 180 (47.7)
Treatment with etanercept, n (%) 89 (23.6)
Table 2. Prevalence of AAV ocular involvement
Baseline prevalence Overall prevalence
Conjunctivitis/episcleritis, n (%) 41 (10.9) 62 (16.4)
Retroorbital mass/proptosis, n (%) 12 (3.2) 17 (4.5)
Uveitis, n (%) 0 (0.0) 2 (0.5)
Scleritis, n (%) 16 (4.2) 22 (5.8)
Retinal exudates/hemorrhage, n (%) 1 (0.3) 1 (0.3)

References: 1. Etanercept plus standard therapy for Wegener’s granulomatosis. N Engl J Med. Jan 27 2005;352(4):351-361. 2. Specks U, Merkel PA, Seo P, et al. Efficacy of remission-induction regimens for ANCA-associated vasculitis. N Engl J Med. Aug 1 2013;369(5):417-427.

Disclosure: S. Cai, None; G. Papaliodis, None; L. Lu, None; H. K. Choi, None; U. Specks, Genentech, 5; P. A. Merkel, Bristol Myers Squibb, 2,CaridianBCT, 2,Celgene, 2,Chemocentryx, 2,Genentech/Roche, 2,GlaxoSmithKline, 2,Kypha, 2,Bristol-Myers Squibb, 5,Chemocentryx, 5,Genentech/Roche, 5,GlaxoSmithKline, 5,PrinicipioBio, 5,Auven, 5,Proteon Therapeutics, 5; P. Seo, None; R. F. Spiera, Roche/Genentech, 2,Roche/Genentech, 5; C. A. Langford, Genentech and Biogen IDEC Inc., 2,GlaxoSmithKline, 2,Bristol-Myers Squibb, 2; G. S. Hoffman, None; C. G. M. Kallenberg, Genentech/Roche, 2,Genentech/Roche, 5; W. St Clair, None; N. Tchao, None; F. Fervenza, None; P. A. Monach, Genentech and Biogen IDEC Inc., 2,Bristol-Myers Squibb, 2,MedScape, 5,GlaxoSmithKline, 2,Vasculitis Foundation Board of Directors, 6,Editorial Board of Arthritis and Rheumatology, 6; W. J. McCune, None; J. H. Stone, Roche Pharmaceuticals, 2,Roche Pharmaceuticals, 5; E. Miloslavsky, None.

To cite this abstract in AMA style:

Cai S, Papaliodis G, Lu L, Choi HK, Specks U, Merkel PA, Seo P, Spiera RF, Langford CA, Hoffman GS, Kallenberg CGM, St Clair W, Tchao N, Fervenza F, Monach PA, McCune WJ, Stone JH, Miloslavsky E. Ocular Manifestations of ANCA-Associated Vasculitis [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/ocular-manifestations-of-anca-associated-vasculitis/. Accessed .

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