Session Information
Session Type: ACR Poster Session C
Session Time: 9:00AM-11:00AM
Background/Purpose: Ophthalmologic involvement in Granulomatosis with Polyangiitis (GPA) is present in 50-60% of the patients and can affect any part of the ocular glove and the orbit. We aimed to describe clinical and subclinical ophthalmologic manifestations, association with systemic symptoms, disease activity and damage in patients with GPA.
Methods: Cross-sectional study including patients with GPA diagnosis (ACR 1990 classification criteria and/or 2012 Chapel Hill Consensus definitions). All patients underwent rheumatologic and ophthalmologic evaluation. Information regarding demographics, comorbidities, clinical variables, ophthalmologic symptoms, serologic markers, radiographic studies, disease activity and damage was retrieved. Descriptive statistics, correlation and univariable logistic regression analyses, Student t, Mann-Whitney U, chi square and Fisher’s exact tests were performed.
Results: Fifty patients were included, 60% female, age 56 years (24-82), disease duration 72.5 months (0-469). Nineteen patients (38%) had ophthalmic manifestations at GPA diagnosis; being sleritis/episcleritis the most frequent (18%), while 27 (54%) presented them during follow-up, with repeated scleritis/episcleritis and lacrimal gland involvement being the most frequent. Concomitant ophthalmic and sinusal involvement was present in 12 (24%). Radiologic abnormalities were observed in 35 (76%), being sinusitis the most frequent (Table 1). BVAS/GPA was 1 (0-17) and VDI 5.5 (0-11), with ocular and ENT damage being present in 58% and 70%, respectively. Forty three patients (86%) presented at least one ophthalmologic symptom, being epiphora and blurred vision the most frequent (40%). At least one clinical abnormality was found in 31 (62%). Scleromalacia (27%) and conjunctival hyperemia (26%) were the most frequent findings (Table 2). Association between radiographic granulomas and clinical proptosis (r=0.69, p=0.001), and between extraconal fat herniation and episcleritis (r=0.69, p=0.0001) was found. Ophthalmic involvement at diagnosis was associated with concomitant ophthalmic and sinusal involvement at follow-up (OR 4.72, 95% CI 1.17-19.01, p=0.01). Ophthalmic involvement at follow-up was associated with age at GPA diagnosis (OR 0.94, 95% CI 0.90-0.99, p=0.03), VDI (OR 1.29, 95% CI 1.03-1.61, p=0.02), and ENT damage (OR 5.27, 95% CI 1.37-20.13, p=0.01).
Conclusion: In GPA, clinical and subclinical ophthalmic involvement is frequent at diagnosis and follow-up, it is associated with concomitant sinusal involvement and has impact on ocular and ENT damage.
Table 1.
Variable |
n (%) |
Ophthalmic manifestations at diagnosis Scleritis/Episcleritis Lacrimal gland involvement Optic nerve involvement Retinal artery occlusion Ulcerative keratitis Uveitis Pseudotumor |
19 (38) 9 (18) 2 (4) 3 (6) 2 (4) 3 (6) 2 (4) 1 (2) |
Ophthalmic manifestations at follow-up Scleritis/Episcleritis Repeated scleritis/episcleritis Lacrimal gland involvement Chronic dacryocystitis Retinal involvement Uveitis Optic nerve involvement Other |
27 (54) 6 (12) 8 (16) 8 (16) 4 (8) 2 (4) 3 (6) 4 (8) 7 (14) |
Concomitant ophthalmic and sinusal involvement |
12 (24) |
Radiologic findings (n=46) Radiologic abnormalities Sinusitis Granulomas Pseudotumor Bone erosions Pachymeningitis Mastoiditis Otomastoiditis Lateral cartilage collapse Septal perforation Exophthalmos Lacrimal gland enlargement Extraconal fat herniation |
n (%) 35 (76) 25 (54) 4 (9) 2 (4) 10 (22) 2 (4) 5 (11) 4 (9) 17 (37) 19 (41) 7 (15) 6 (13) 2 (4) |
Table 2.
Sign |
Either eye n (%) |
Blindness |
12 (24) |
Extraocular muscular involvement |
2 (4) |
Exophthalmos |
10 (20) |
Orbital granuloma |
2 (4) |
Palpebral edema/inflammation |
3 (6) |
Proptosis |
2 (4) |
Ptosis |
3 (6) |
Dacryocystitis |
9 (18) |
Nasal conjunctive fistulas |
2 (4) |
Optic neuropathy |
3 (6) |
Diplopia |
1 (2) |
Epiphora |
10 (20) |
Conjunctival inflammation/hyperemia |
13 (26) |
Episcleritis |
1 (2) |
Necrotizing scleritis |
1 (2) |
Ulcerative peripheral keratitis |
2 (4) |
Keratoconjunctivitis sicca |
4 (8) |
Scleromalacia |
13 (27) |
Retinal vasculitis |
1 (2) |
Retinal exudates |
1 (2) |
To cite this abstract in AMA style:
Hinojosa-Azaola A, García-Castro A, Juárez-Flores A. Prevalence of Clinical and Subclinical Ophthalmologic Manifestations in Association with Systemic Symptoms, Disease Activity and Damage in Patients with Granulomatosis with Polyangiitis [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/prevalence-of-clinical-and-subclinical-ophthalmologic-manifestations-in-association-with-systemic-symptoms-disease-activity-and-damage-in-patients-with-granulomatosis-with-polyangiitis/. Accessed .« Back to 2018 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/prevalence-of-clinical-and-subclinical-ophthalmologic-manifestations-in-association-with-systemic-symptoms-disease-activity-and-damage-in-patients-with-granulomatosis-with-polyangiitis/