Epidemiology of uveitis in the mid-Atlantic United States

Figures & data

Figure 1 Map showing centers of the US-based epidemiological studies (created in Microsoft MapPoint^® 2013; Microsoft Corp, Redmond, WA, USA).

Note: The red dots indicate center locations.

Figure 2 Mean age at diagnosis and presentation.

Note: Intervals are non-overlapping. Each interval is open on the left and closed on the right (eg, [0, 10 years]).

Table 1 Age groups

Age group (years)*	Patients, n	Percentage
<18	39	7.9
18–35	129	26.3
18–65	355	72.3
>65	97	19.8

Notes:

* The groups were defined as: <18 years = pediatric group; 18–35 years = young adults; 18–65 years = working-age group; >65 years = older-age group.

Table 2 Distribution of diagnoses by sex

Diagnosis	Female n (%)	Male n (%)	P-value*
Anterior uveitis, undifferentiated	73 (26.3)	53 (24.9)	0.755
Trauma	40 (14.3)	20 (9.4)	0.098
Viral
Herpetic anterior uveitis	30 (10.8)	9 (4.2)	0.011
Acute retinal necrosis	9 (3.2)	4 (1.9)	0.408
CMV uveitis	2 (0.7)	2 (0.9)	1.000
Herpetic panuveitis	1 (0.4)	0 (0.0)	1.000
Post-procedural uveitis	21 (7.6)	28 (13.1)	0.048
Anterior uveitis HLA-B27	18 (6.5)	15 (7.0)	0.857
Sarcoidosis	17 (6.1)	16 (7.5)	0.588
Intermediate uveitis	11 (4.0)	8 (3.8)	1.000
Panuveitis, undifferentiated	11 (4.0)	12 (5.6)	0.397
Others	8 (2.9)	11 (5.2)	0.240
Infectious, nonviral
Toxoplasma	8 (2.9)	6 (2.8)	1.000
Fungal	3 (1.1)	1 (0.5)	0.637
Syphilis	1 (0.4)	0 (0.0)	1.000
Tuberculosis	1 (0.4)	0 (0.0)	1.000
Posterior uveitis, undifferentiated	6 (2.2)	3 (1.4)	0.738
White dot syndrome
Birdshot	5 (1.8)	2 (0.9)	0.704
MEWDS	2 (0.7)	1 (0.5)	1.000
Multifocal choroiditis	1 (0.4)	8 (3.8)	0.012
APMPPE	1 (0.4)	3 (1.4)	0.321
Serpiginous	1 (0.4)	2 (0.9)	0.582
Seronegative SpA
Ankylosing spondylitis	2 (0.7)	3 (1.4)	0.657
Crohn’s disease	2 (0.7)	2 (0.9)	1.000
Ulcerative colitis	1 (0.4)	1 (0.5)	1.000
Reactive arthritis	1 (0.4)	0 (0.0)	1.000
Psoriatic arthritis	0 (0.0)	1 (0.5)	0.434
Eales disease	1 (0.4)	0 (0.0)	1.000
Collagen vascular disease
Polyarteritis nodosa	1 (0.4)	0 (0.0)	1.000
Granulomatosis with polyangiitis	0 (0.0)	1 (0.5)	0.434
Total	279 (100.0)	214 (100.0)	0.066#

Notes:

* Fisher’s exact test on 2×2 table, eg, female vs male compared on sarcoidosis vs all other diagnoses.

# Fisher’s exact test applied to entire table, using Monte Carlo sampling (P=0.085).

Abbreviations: APMPPE, acute posterior multifocal placoid pigment epitheliopathy; CMV, cytomegalovirus; HLA, human leukocyte antigen; MEWDS, multiple evanescent white dot syndrome; seronegative SpA, seronegative spondyloarthropathy.

Table 3 Distribution of diagnoses by race

Diagnosis	African American n (%)	Caucasian n (%)	P-value*
Viral
Herpetic anterior uveitis	2 (1.5)	32 (10.8)	<0.001
Acute retinal necrosis	5 (3.7)	8 (2.7)	0.553
CMV uveitis	1 (0.7)	3 (1.0)	1.000
Herpetic panuveitis	0 (0.0)	1 (0.3)	1.000
Sarcoidosis	23 (17.2)	9 (3.0)	<0.001
Anterior uveitis, undifferentiated	40 (29.9)	63 (21.2)	0.067
Trauma	22 (16.4)	35 (11.8)	0.219
Post-procedural	14 (10.4)	27 (9.1)	0.723
Anterior uveitis HLA-B27	8 (6.0)	22 (7.4)	0.686
Others	2 (1.5)	15 (5.1)	0.108
Intermediate uveitis	3 (2.2)	14 (4.7)	0.291
Panuveitis, undifferentiated	7 (5.2)	13 (4.4)	0.805
Infectious (bacterial, fungal, parasitic)
Toxoplasma	1 (0.7)	10 (3.4)	0.185
Fungal	0 (0.0)	4 (1.3)	0.315
Syphilis	0 (0.0)	1 (0.3)	1.000
Tuberculosis	0 (0.0)	0 (0.0)	1.000
Posterior uveitis, undifferentiated	2 (1.5)	6 (2.0)	1.000
White dot syndrome
Multifocal choroiditis	3 (2.2)	5 (1.7)	0.708
Birdshot	0 (0.0)	5 (1.7)	0.330
APMPPE	0 (0.0)	4 (1.3)	0.315
Serpiginous	0 (0.0)	3 (1.0)	0.556
MEWDS	0 (0.0)	2 (0.7)	1.000
Seronegative SpA
Ankylosing spondylitis	0 (0.0)	5 (1.7)	0.330
Crohn’s disease	0 (0.0)	4 (1.3)	0.315
Reactive arthritis	0 (0.0)	1 (0.3)	1.000
Ulcerative colitis	0 (0.0)	1 (0.3)	1.000
Psoriatic arthritis	0 (0.0)	1 (0.3)	1.000
Collagen vascular disease
Granulomatosis with polyangiitis	0 (0.0)	1 (0.3)	1.000
Polyarteritis nodosa	0 (0.0)	1 (0.3)	1.000
Fuch’s iridocyclitis	0 (0.0)	1 (0.3)	1.000
Eales disease	1 (0.8)	0 (0.0)	0.311
Total	134 (100.0)	297 (100.0)	<0.001†

Notes:

* Fisher’s exact test on 2×2 table, eg, African American vs Caucasian compared on sarcoidosis vs all other diagnoses.

† Fisher’s exact test applied to entire table, using Monte Carlo sampling.

Abbreviations: APMPPE, acute posterior multifocal placoid pigment epitheliopathy; CMV, cytomegaovirus; HLA, human leukocyte antigen; MEWDS, multiple evanescent white dot syndrome; seronegative SpA, seronegative spondyloarthropathy.

Table 4 Multivariate analysis for race and sex

Diagnosis	Odds ratio (95% confidence interval)*
	Race	Sex
Sarcoidosis	6.54 [2.98, 15.29]	0.89 [0.42, 1.91]
Anterior uveitis, undifferentiated	1.58 [0.99, 2.51]	1.02 [0.65, 1.60]
Herpetic anterior uveitis	0.13 [0.02, 0.47]	3.03 [1.32, 7.71]
Multifocal choroiditis	1.24 [0.24, 5.52]	0.11 [0.01, 0.68]
Trauma	1.51 [0.83, 2.69]	1.76 [0.97, 3.25]
Post-procedural	1.14 [0.56, 2.24]	0.55 [0.29, 1.06]

Notes:

* Odds ratios were computed via exact logistic regression. The odds ratios are >1 when African Americans or females are more likely to have the disease. The odds ratios are statistically significant if the 95% confidence interval does not include 1.

Figure 3 Map of Virginia with the residential postal codes of the patients.

Table 5 States of residence for patients seeking care at the University of Virginia

State	Number of patients
AL	1
CA	1
FL	2
GA	2
MD	3
MI	2
NC	1
NM	1
NY	1
OR	1
SC	2
TN	1
VA	462
WA	1
WV	7
WY	1

Abbreviations: AL, Alabama; CA, California; FL, Florida; GA, Georgia; MD, Maryland; MI, Michigan; NC, North Carolina; NM, New Mexico; NY, New York; OR, Oregon; SC, South Carolina; TN, Tennessee; VA, Virginia; WA, Washington; WV, West Virginia; WY, Wyoming.

Table 6 Change in visual acuity

Overall
Variable	N	Mean	SD	SE	Median
Initial logMAR	458	0.54	0.74	0.03	0.3
Final logMAR P=0.002*	458	0.52	0.82	0.04	0.18
Anterior uveitis
Initial logMAR	308	0.44	0.63	0.04	0.18
Final logMAR P=0.058*	308	0.45	0.78	0.04	0.16
Intermediate uveitis
Initial logMAR	25	0.61	0.83	0.17	0.3
Final logMAR P=0.038*	25	0.27	0.35	0.07	0.14
Posterior uveitis
Initial logMAR	64	1.07	1.05	0.13	0.63
Final logMAR P=0.197*	64	0.99	1.05	0.13	0.53
Panuveitis
Initial logMAR	57	0.43	0.4	0.05	0.39
Final logMAR P=0.216*	57	0.45	0.63	0.08	0.25

Notes:

* Wilcoxon rank sum test, analysis done at patient level.

Abbreviations: SD, standard deviation; SE, standard error of the mean.

Table 7 Ophthalmic management and interventions

Treatment/intervention	Number (%)
Local steroids	365 (74.6%)
Systemic steroids	133 (27.3%)
Anti-TNF agents	17 (3.5%)
Antimetabolites	52 (10.6%)
Intravitreal injection	54 (11.0%)
Sub-Tenon injection	23 (4.7%)
Glaucoma topical treatment	116 (23.6%)
Glaucoma surgery	43 (8.8%)
Cataract surgery	129 (26.4%)
Pars plana vitrectomy	46 (9.4%)

Abbreviation: TNF, tumor necrosis factor.

Table 8 Anatomical classification

Definition	Number	Percentage	Number bilateral	Number unilateral
Anterior uveitis	332	67.6	75	257
Intermediate uveitis	26	5.3	19	7
Posterior uveitis	62	12.6	32	30
Panuveitis	71	14.5	44	27

Figure 4 Differentiated (diagnosed) vs undifferentiated (undiagnosed) cases.

Table 9 Uveitis diagnoses at University of Virginia

Diagnosis	Patients, n	Percentage
Anterior uveitis, undifferentiated	126	25.7
Trauma	60	12.2
Post-procedural	49	10.0
Viral
Acute retinal necrosis	13	2.7
Herpetic anterior uveitis	39	7.9
CMV uveitis	4	0.8
Herpetic panuveitis	1	0.2
Sarcoidosis	33	6.7
HLA-B27 anterior uveitis	33	6.7
Fuch’s iridocyclitis	1	0.2
Intermediate uveitis	26	5.3
Panuveitis, undifferentiated	23	4.7
Infectious
Toxoplasma	14	2.9
Syphilis	1	0.2
Tuberculosis	1	0.2
Fungal	4	0.8
Posterior uveitis, undifferentiated	9	1.8
White dot syndrome
Multifocal choroiditis	9	1.8
Birdshot	7	1.4
APMPPE	4	0.8
Serpiginous	3	0.6
MEWDS	3	0.6
Eales disease	1	0.2
Collagen vascular disease
Granulomatosis with polyangiitis	1	0.2
Polyarteritis nodosa	1	0.2
Seronegative SpA
Reactive arthritis	1	0.2
Ankylosing spondylitis	5	1.0
Crohn’s disease	4	0.8
Ulcerative colitis	2	0.4
Psoriatic arthritis	1	0.2
Others	19	3.9
Total	491	100.0

Abbreviations: APMPPE, acute posterior multifocal placoid pigment epitheliopathy; CMV, cytomegalovirus; HLA, human leukocyte antigen; MEWDS, multiple evanescent white dot syndrome; seronegative SpA, seronegative spondyloarthropathy.

Table 10 Multicenter comparison of anatomic location of uveitis

	UVA	Centers
		United states						International
		MEEI^Citation3	Duke^Citation5	IA*^Citation10	UCLA*^Citation9	USC^Citation11	KY^Citation1	REH^Citation8	SBU^Citation7	UM^Citation6
Time period	1984–2014	1982–1992	1989–1994	1956–1960	1991–1994	2006–2007	1990–1995	1991–2013	–	2006–2010
Patients (N)	491	1,237	385	172	213	217,061	853	3,301	2,693 (ME) 4,379 (EU)	43 (FN) 45 (control)
Anterior uveitis (%)	67.6	51.6	25	60.0 (I) 63.0 (L)	90.6 (CBP) 60.6 (URP)	72	22.3	46	49.6 (ME) 51.3 (EU)	26.0 (FN) 73.0 (control)
Posterior uveitis (%)	12.6	17.4	24	29 (I) 19 (L)	4.7 (CBP) 14.6 (URP)	29	48.4	21.8	15.5 (ME) 23.7 (EU)	7.0 (FN) 2.0 (control)
Intermediate uveitis (%)	5.3	13	12	6	1.4 (CBP) 12.2 (URP)	6	10.9	11.1	10.3 (ME) 9.1 (EU)	0.0 (FN) 9.0 (control)
Panuveitis (%)	14.5	16	38	10.0 (I) 18.0 (L)	1.4 (CBP) 9.4 (URP)	21	18.4	21.1	24.9 (ME) 15.9 (EU)	67.0 (FN) 16.0 (control)

Notes:

* IA and UCLA frequencies extrapolated from data, as different localization systems used.

Abbreviations: UVA, University of Virginia; MEEI, Massachusetts Eye and Ear Infirmary; Duke, Duke University; IA, University of Iowa; UCLA, University of California at Los Angeles; USC, University of Southern California; KY, University of Louisville, Kentucky; REH, The Royal Eye Hospital, Manchester, UK; SBU, Shahid Beheshti University of Medical Sciences, Tehran, Iran; UM, University of Manitoba, Winnipeg, Canada; ME, Middle East; EU, Europe; FN, First Nation; L, London; I, Iowa; CBP, Community Based Practice; URP, University Referral Practice.

Table 11 Relative frequencies of key uveitis diagnoses

	UVA	Centers
		United States						International
		MEEI^Citation3	Duke^Citation5	IA^Citation10	UCLA^Citation9	USC^Citation11	KY^Citation1	REH^Citation8
				(IA frequencies*)	(university referral frequencies*)
Time period	1984–2014	1982–1992	1989–1994	1956–1960	1991–1994	2006–2007	1990–1995	1991–2013
Patients, n	491	1,237	385	172	213	217,061	853	3,301
Sarcoidosis, %	6.7	9.6	11	5	0.8	2	2.2	9.7
Granulomatosis with polyangiitis, %	0.2
Polyarteritis nodosa, %	0.2	0.2
Anterior uveitis, undifferentiated, %	25.7	37.8	12	60	30.5		54.2	7
Intermediate, %	3.9		12	6	12.2		78.7
Panuveitis, undifferentiated, %	4.7	22.2	17		40		30.9	6.6
Posterior uveitis, undifferentiated, %	1.8	13.3	2	17			19.3
Toxoplasma, %	2.9	4.8	10	9		4	3.9	6.9
Syphilis, %	0.2	1.7	0.5	1
Tuberculosis, %	0.2	0.6	0.5			0.4
Fungal, %	0.8	0.3
Reactive arthritis, %	0.2					1
Ankylosing spondylitis, %	1	3.8	2			3
Crohn’s disease, %	0.8	0.7
Ulcerative colitis, %	0.4	0.5
Psoriatic arthritis, %	0.2
Acute retinal necrosis, %	2.7		2
Herpetic anterior uveitis, %	7.9	9.7	3
CMV uveitis, %	0.8	2.2					15.1
Herpetic panuveitis, %	0.2
Multifocal choroiditis, %	1.8		6		6.5		4.9
Birdshot, %	1.4		0.78		6.5	1	1.6
APMPPE, %	0.8		0.5
Serpiginous, %	0.6				3.2		1.9
MEWDS, %	0.6					0.4
Trauma, %	12.2
Post-procedural, %	10
Fuch’s iridocyclitis, %	0.2		2			1		11.5
Eales disease, %	0.2
Anterior uveitis HLA-B27, %	6.7	31.3	19		17.8		8.4	3.6
Others, %	3.9

Notes:

* IA and UCLA frequencies extrapolated from data, as different localization systems were used.

Abbreviations: APMPPE, acute posterior multifocal placoid pigment epitheliopathy; CMV, cytomegalovirus; Duke, Duke University; HLA, human leukocyte antigen; IA, University of Iowa; KY, University of Louisville, Kentucky; MEEI, Massachusetts Eye and Ear Infirmary; MEWDS, multiple evanescent white dot syndrome; REH, Royal Eye Hospital, Manchester, UK; UCLA, University of California at Los Angeles; USC, University of Southern California; UVA, University of Virginia.

Table 12 Anterior uveitis diagnoses

	UVA n=332 n, (%)	Duke^Citation5 n=97 %*	MEEI^Citation3 n=637 %*	USC^Citation26 n=167 %*	UCLA**^Citation9 n=129 %*	KY^Citation1 n=190 %*	IA**^Citation10 n=104 %*	REH**^Citation8 n=3,301 %*
Undifferentiated	126 (38)	49	37.8	12.1	50.5	54.2	60	12.3
Trauma	59 (17.8)			0.8		2.7
Herpetic uveitis	39 (11.7)	3.0	9.7	9.0		2.2		1.7
Post-procedural	36 (10.5)
HLA-B27	33 (9.9)	19.0	6.4	3.0	17.8	8.4		4.5
Sarcoidosis	19 (5.7)	3.0	5.8			1.2	11.0	2.39
Ankylosing spondylitis	4 (1.2)	9.0	7.4	1.5		4.7	13.0
IBD	4 (1.2)	1.0	2.4	0.7			7.0	0.96
Reactive	1 (0.3)	3.0	4.0	1.0			12.0	0.66
Psoriatic arthritis	1 (0.3)		0.8					0.57
Fuch’s heterochromic iridocyclitis	1 (0.3)	7.0	5.0	1.8		2.2	39.0	11.5
Granulomatosis with polyangiitis	1 (0.3)		0.5
Others	8 (2.7)

Notes: Others included: TINU (3), rheumatoid arthritis (1), juvenile rheumatoid arthritis (1), multiple sclerosis (1), and Posner–Schlossman syndrome (2).

* All values given as percentage only.

** UCLA, IA, and REH values extrapolated from given data.

Abbreviations: Duke, Duke University; HLA, human leukocyte antigen; IA, University of Iowa; IBD, Inflammatory bowel disease; KY, University of Louisville, Kentucky; MEEI, Massachusetts Eye and Ear Infirmary; REH, Royal Eye Hospital, Manchester, UK; TINU, tubulointerstitial nephritis and uveitis; UCLA, University of California at Los Angeles; USC, University of Southern California; UVA, University of Virginia.

Table 13 Intermediate uveitis diagnoses

	UVA n=26 (%)	Duke^Citation5 n=47*	MEEI^Citation3 n=162*	USC^Citation26 n=92*	UCLA**^Citation9 n=26*	KY^Citation1 n=94	IA**^Citation10 n=52*	REH**^Citation8 n=3,301*
Undifferentiated	20 (77)	98	69.1	100	30.8	94.7	40.0	7.8
Sarcoidosis	3 (11.5)	2.0	22.2		0			1.75
Multiple sclerosis	2 (3.8)		8.0		0	2.1		0.54
Ankylosing spondylitis	1 (3.8)

Notes:

* All values given as percentage only.

** UCLA, IA, and REH values extrapolated from given data.

Abbreviations: Duke, Duke University; IA, University of Iowa; KY, University of Louisville, Kentucky; MEEI, Massachusetts Eye and Ear Infirmary; REH, Royal Eye Hospital, Manchester, UK; UCLA, University of California at Los Angeles; USC, University of Southern California; UVA, University of Virginia.

Table 14 Posterior uveitis diagnoses

	UVA n=62 (%)	Duke^Citation5 n=93*	MEEI^Citation3 n=240*	USC^Citation26 n=230*	UCLA**^Citation9 n=31*	KY^Citation1 n=414*	IA**^Citation10 n=52*	REH**^Citation8 n=3,301*
Toxoplasma	11 (17.7)	42.0	24.6	9.0	35.5	7.7	65.0	6.9
Multifocal choroiditis	9 (14.5)			18.0	9.7	10.1		0.8
Undifferentiated	9 (14.5)	9	13.3	15.0	16.2	19.3	48.0
Acute retinal necrosis	4 (6.5)	9	5.5			2.7		0.2
Birdshot	7 (11.3)	3	7.9	3.0	6.5	3.4		1.09
MEWDS	3 (4.8)		1.25			1.2		0.4
APMPPE	3 (4.8)	2	2.0
Serpiginous	3 (4.8)		1.6	5.0	3.2	3.9		0.3
Sarcoidosis	2 (3.2)		7.5	3.9		2.2	4.0	2.9
CMV	2 (3.2)		11.6	7.0		31.2		0.7
Syphilis	1 (1.6)		2.0				4.0
Polyarteritis nodosa	1 (1.6)		0.83
Eales disease	1 (1.6)
Others	2 (3.2)

Notes: Others included: IRVAN (2).

* All values given as percentage only.

** UCLA, IA, and REH values extrapolated from given data.

Abbreviations: APMPPE, acute posterior multifocal placoid pigment epitheliopathy; CMV, cytomegalovirus; Duke, Duke University; IA, University of Iowa; IRVAN, idiopathic retinitis, vasculitis, aneurysms, and neuroretinitis; KY, University of Louisville, Kentucky; MEEI, Massachusetts Eye and Ear Infirmary; MEWDS, multiple evanescent white dot syndrome; REH, Royal Eye Hospital, Manchester, UK; USC, University of Southern California; UCLA, University of California at Los Angeles; UVA, University of Virginia.

Table 15 Panuveitis diagnoses

	UVA n=71 (%)	Duke^Citation5 n=148*	MEEI^Citation3 n=198*	USC^Citation26 n=111*	UCLA**^Citation9 n=20*	KY^Citation1 n=155*	IA**^Citation10 n=18*	REH**^Citation8 n=3,301*
Undifferentiated	23 (32.4)	43.0	22.2	45.0	18.7	30.9	27.0
Post-procedural	13 (18.3)
Acute retinal necrosis	9 (12.7)			18.0		1.3
Sarcoidosis	9 (12.7)	27.0	14.1	21.0	2.3	4.5	28.0	2.99
Toxoplasma	3 (4.2)
Fungal	3 (4.2)		2.0
Herpetic uveitis	2 (2.8)							0.01
Trauma	1 (1.4)
Tuberculosis	1 (1.4)	1.0	2.0
Others	4 (5.6)

Notes: Others included: Behcet’s disease (1), Vogt–Koyanagi–Harada disease (1), juvenile rheumatoid arthritis (1).

* All values given as percentage only.

** UCLA, IA, and REH values extrapolated from given data.

Abbreviations: Duke, Duke University; IA, University of Iowa; KY, University of Louisville, Kentucky; MEEI, Massachusetts Eye and Ear Infirmary; REH, Royal Eye Hospital, Manchester, UK; UCLA, University of California at Los Angeles; USC, University of Southern California; UVA, University of Virginia.

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