Table 1.

Demographic features, ocular and extra-ocular manifestations at disease onset or developed during follow-up, ANCA pattern and disease activity in 22 patients with granulomatosis with polyangiitis

Pt. No.	Age, Sex	BCVA OD OS	IOP OD OS	Ophthalmologic Diagnosis	Extraocular Organ or System Involvement	ANCA Pattern of Positivity	BVASv3
1	48, M	20/60 20/40	13 14	Peripheral ulcerative keratitis OD	Arthralgia/arthritis, paranasal sinusitis, bilateral lung infiltrates, proteinuria > 1.5 g/day	c-ANCA	NA/NP
2	59, F	20/60 20/40	15 14	Diffuse episcleritis OU	Fever, weight loss, fatigue, increased serum creatinine levels	c-ANCA	9
3	54, F	20/60 20/40	13 14	Diffuse episcleritis OD and conjunctivitis OS	Constitutional, sinusitis, proteinuria, hematuria, hypertension, congestive heart failure	c-ANCA	23
4	68, F	20/40 20/50	13 14	Nodular episcleritis and conjunctivitis OS	Paranasal sinusitis, proteinuria, hypertension	c-ANCA	13
5	46, F	20/40 20/60	14 18	Unspecified scleritis OS	General and mucocutaneous symptoms, paranasal sinusitis, urinary abnormalities, hypertension	c-ANCA	23
6	60, M	20/40 20/40	13 14	Unspecified scleritis OU	General, nasal granulomata, torpid genital ulcers, mild proteinuria	c-ANCA	14
7	35, M	20/40 20/40	18 14	Diffuse anterior scleritis OU	Mild constitutional symptoms, sinusitis, urinary protein output > 1 g/24 hr	c-ANCA	9
8	55, F	20/40 20/40	13 14	Diffuse anterior scleritis OS with peripheral ulcerative keratitis OD	Serotine fever, myalgias, conductive hearing loss, right lung with initial cavitating infiltration	NA/NP	13
9	51, M	20/60 20/40	16 14	Necrotizing scleritis OD	Constitutional symptoms, mouth ulcers, bloody nasal discharge, bilateral pleural effusion	p-ANCA	18
10	41, F	20/160 20/160	13 20	Necrotizing scleritis OU	Pulmonary nodules, serum creatinine > 30% of ULN, nasal crusts, mucocutaneous and general symptoms	NA/NP	29
11	65, F	20/40 20/80	15 19	Necrotizing scleritis OS	General, bilateral cavitating pulmonary lesions, saddle nose deformity	NA/NP	18
12	64, F	20/200 20/40	23 16	Necrotizing scleritis, scleral melt and uveal prolapse OD	Patchy ground-glass opacities in both lungs, nasal ulcers and granulomata, constitutional symptoms	NA/NP	24
13	71, F	20/40 20/80	14 18	Circumscribed scleromalacia OS	Necrotizing glomerulonephritis, mononeuritis multiplex	c-ANCA	27
14	46, M	20/200 20/40	13 14	Orbital mass OD	Irregular fever, arthromyalgias	c-ANCA	9
15	66, M	20/140 20/40	13 14	Retro-orbital pseudotumor with proptosis OD	Bloody-purulent nasal discharge, purpura of the legs, pleural effusion	NA/NP	16
16	58, M	20/40 20/120	15 24	Left orbital inflammatory pseudotumor	Fever, weight loss and arthralgia, recurrent bloody nasal discharge with nasal septum perforation	c-ANCA	15
17	59, F	20/40 20/40	13 14	Right orbital mass with dacriocystitis	Nasal granulomata, persistent proteinuria in the nephrotic range and hematuria	c-ANCA	20
18	73, F	20/40 HM	18 26	Left retro-orbital Inflammatory pseudotumor with diplopia	Constitutional, purpuric eruptions, paranasal sinusitis, bilateral pulmonary nodules, focal glomerulonephritis	c-ANCA	NA/NP
19	60, F	20/160 20/120	13 14	Orbital inflammatory disease OU	Rhinosinusitis, rapidly progressive glomerulonephritis	ANCA-neg → pos	18
20	43, M	CF 20/40	13 14	Compressive neuropathy OD	Consolidated nodules in both lungs, biopsy-shown crescentic glomerulonephritis	c-ANCA	NA/NP
21	50, F	20/40 CF	13 14	Optic perineuritis with compressive neuropathy OS	Ground-glass opacities in the left lung, radiological signs of pulmonary hemorrhages in the right lung	c-ANCA	12
22	47, M	20/40 20/160	13 14	Retinal vasculitis with blurring vision OS	Patchy ground-glass opacities in the right lung, sensory peripheral neuropathy	c-ANCA	16

BCVA best-corrected visual acuity, BVASv3 Birmingham Vasculitis Activity Score version 3 (Mukhtyar C et al.²¹), CF counting fingers, HM hand motion, IOP intraocular pressure (normal values 10–21 mmHg), NA/NP not available or not performed, OD right eye, OS left eye, OU both eyes, ULN upper limit of normal