Current Eye Research

Volume 9 supplement 1990

Jorge Palmares, Maria Fatima Coutinho and Jose Castro-Correia

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Department of Ophthalmology, Faculty of Medicine, Hospital S. Joiio, 4200 Porto, Portugal

ABSTRACT During the p a s t f o u r years 450 p a t i e n t s (247 female mean age 36 years and 203 male mean age 34 years) with i n t r a o c u l a r i n f l a m m a t i o n were evaluated u s i n g standard d i a g n o s t i c c r i t e r i a , t o e s t a b l i s h a u v e i t i s survey p r o f i l e . P a t i e n t s were d i v i d e d i n t o f o u r anatomic groups: a n t e r i o r u v e i t i s (270 cases = 60%), p o s t e r i o r u v e i t i s (108 cases = 2 4 % ) , p a n u v e i t i s (54 cases = 12%) and i n t e r m e d i a t e u v e i t i s (18 cases = 4 % ) . A probable a e t i o l o g i c a l d i a g n o s i s was made i n 232 cases (51.5%). Rheumatic diseases were diagnosed most commonly (55 cases = 12.2%), mainly a n k y l o s i n g s p o n d y l i t i s , as i s t r u e f o r o t h e r European c o u n t r i e s . Ocular toxoplasmosis was a f r e q u e n t cause o f u v e i t i s , as i n B r a z i l and West A f r i c a , thus r e q u i r i n g a prompt d i a g n o s i s and treatment. BehCet’s syndrome was associated w i t h HLA B 5 (65%) as i n Japanese and Mediterranean populations. I n our area AIDS r e t i n i t i s i s becoming an i m p o r t a n t d i f f e r e n t i a l diagnosis.

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be i n v a l u a b l e t o understand t h e a e t i o l o g y o f t h e disease and t h e proper c l a s s i f i c a t i o n o f i t s subtypes.

I n order t o obtain a c o r r e c t incidence

o f u v e i t i s , a common s e t o f c r i t e r i a f o r d i f f e r e n t d i a g n o s i s must be defined. T h i s survey corresponds t o t h e update o f t h e o n l y d a t a e x i s t a n t f o r t h e Portuguese p o p u l a t i o n , which we i n i t i a t e d i n 1985 and was p u b l i s h e d as a p r e l i m i n a r y r e p o r t (3).

Our h o s p i t a l i s a major r e f e r r a l c e n t r e (for p r i m a r y care and a l s o t e r t i a r y care) c o v e r i n g t h e Northern P o r t u g a l , down t o t h e Oouro r i v e r ; t h i s area i s i n h a b i t e d by 3 m i l l i o n people (1981 census).

The aim o f t h e p r e s e n t study was t o

determine t h e i n c i d e n c e o f u v e i t i s among our

INTRODUCTION

h o s p i t a l p a t i e n t s and t o i d e n t i f y t h e d i f f e r e n t

Epidemiologic s t u d i e s o f u v e i t i s , although

u n d e r l y i n g causes or syndromes.

i m p o r t a n t as i n o t h e r n o s o l o g i c a l e n t i t i e s , a r e

c o u l d then b e compared w i t h s i m i l a r d a t a bases

d i f f i c u l t t o organise because, as P e r k i n s (1)

from o t h e r areas and used t o e s t a b l i s h e f f e c t i v e

noted, i t i s n o t easy t o make v a l i d comparisons

measures f o r t h e d i a g n o s i s and t r e a t m e n t o f

between u v e i t i s s t a t i s t i c s from d i f f e r e n t coun-

uveitis.

tries,

T h i s d a t a base

due t o d i f f e r e n t d i a g n o s t i c c r i t e r i a and

a e t i o l o g i c a l theories. Moreover, t h e eye u s u a l l y responds i n a very s i m i l a r way t o a l a r g e range o f systemic diseases and l o c a l i n f e c t i o n s , f o r a g i v e n human popul a t i o n l i v i n g i n a g i v e n environmental s e t t i n g .

PATIENTS AND METHOOS From J u l y 1985 t o J u l y 1989, 450 p a t i e n t s were observed ( n e a r l y 51 new c a s e d y e a r ) : 247 female

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mean age 36 years (range 9-79) mean age 34 years (range 3-80).

and 203 male

Our d a t a a r e

I t i s w e l l known t h a t some forms o f u v e i t i s have

b i a s e d by r e f e r r a l method.

a d e f i n i t e geographical d i s t r i b u t i o n such as

i n t r a o c u l a r i n f l a m m a t i o n was i n v e s t i g a t e d accord-

onchocerciasis, histoplasmosis, Behcpt’s syndrome

i n g t o s t a n d a r d d i a g n o s t i c c r i t e r i a as r e p o r t e d

( I ) , so t h a t s t u d i e s on t h e i n c i d e n c e o f u v e i t i s

by Smith and Nozik ( 4 ) , i n c l u d i n g : p a t i e n t s ’

w i t h i n a community a r e i m p o r t a n t t o enable

h i s t o r y , c l i n i c a l examination and l a b o r a t o r y

r a t i o n a l i n v e s t i g a t i o n and management (2).

s t u d i e s , and X-ray and f l u o r e s c e i n angiography as

Therefore, epidemiologic s t u d i e s i n u v e i t i s may

0 Oxford University Press

I n every p a t i e n t

i n d i c a t e d . The w i d e l y known c l a s s i f i c a t i o n o f t h e

31

Current Eye Research International Uveitis Study Group (5) was used.

in 171 cases (38% o f total), acute in 40% and

Cases of endophthalmitis and traumatic uveitis

chronic in 60%. In 99 cases (36.7% of anterior

(sympathetic ophthalmia excepted) were excluded

uveitis) diagnosis was obtained, including by

from this series, since the patients are seldom

order of‘ frequency mainly herpes simplex kerato-

referred to our uveitis clinic.

uveitis, ankylosing spondylitis, Fuchs’ hetero-

Some patients, presenting simultaneously with uveitis and focal infections (dental and tonsil

chromic iridocyclitis, syphilis, tuberculosis, In posterior uveitis cases, a diagnosis was

abscesses, upper respiratory tract viral

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herpes zoster keratouveitis and Reiter’s syndrome.

infections), were included in the idiopathic

made in 72.3% o f cases including: toxoplasmosis

group. The “mixed” group mentioned in the Table 2

retinocToroiditis, BehGet’s syndrome, lupus

includes rare cases, as listed at the end of the

retinitis, CMV retinitis in AIDS patients,

results section.

sarcoidosis, syphilis; retinal vasculitis was seen in 1 6 patients without an underlying cause. Panuveitis was observed in 54 patients. The

RESULTS Patients were divided into four anatomic groups

aetiology was determined in 34 cases including

(Table 1).

mainly Behqet’s syndrome (8 cases), toxoplasmosis

As reported in Table 2, a probable aetiologic

diagnosis was made in 232 cases (51.5%).

No sig-

(7 cases) and sympathetic ophthalmia (3 cases).

Patients with intermediate uveitis formed the

nificant difference was observed between males

smallest group, perhaps because pars planitis is

and females regarding the onset age of uveitis. Iridocyclitis (anterior uveitis) was idiopathic

often misdiagnosed at the first visit (primary care). Rheumatic diseases (55 cases) were the most common. Ocular toxoplasmosis, herpes simplex

Table 1: Uveitis by location Anterior uveitis 270 cases (60%) Posterior uveitis Panuveitis Int,ermediate uveitis

708 54 18

(24%) (12%) ( 4%)

keratouveitis, and BehFet’s syndrome (65% of them B5+ve) were the next most common group to be diagnosed. The rheumatic diseases included

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ankylosing spondylitis (27 cases 85% of them B27+ve), systemic lupus erythematosus (7 cases), Reiter’s syndrome (6 cases), juvenile chronic arthritis (4 cases), rheumatic fever (3 cases),

Table 2: AetioloQy and disease associations Idiopathic uveitis Rheumatisms

218 cases (48.5%) 55 (12.2%)

Toxoplasrnosis

41

( 9.1%)

Herpes simplex

28

Eehqet’s syndrome

20

Pars planitis

18

It

( 6.2%) ( 4.5%) ( 4.0%)

Syphilis Fuchs’ heterochromic

13 10

‘I If

( 2.9%) ( 2.2%)

Tuberculosis

10

If

( 2.2%)

Sarcoidosis CMV retinitis

7

( 1.6%)

6

Herpes zoster

6

( 1.3%) ( 1.3%) ( 4.0%)

Mixed _-

32

18

rheumatoid arthritis (3 cases), polymyalgia rheumatica (3 cases) and psoriatic arthritis

(2 cases). Mixed causes of ocular inflammation were toxocara (3 cases), sympathetic ophthalmia (3 cases), acute retinal necrosis (3 cases), multiple sclerosis (3 cases), brucellosis (2 cases), meningococcal meningitis (2 cases) and rickettsia1 infection ( 2 cases). DISCUSSION

There have been surprinsingly few epidemiologic studies concerning uveitis. The results of the present study, the first performed in the Portu-

Current Eye Research guese population, do, however, confirm the simi-

tions (13). A similar result (4.2%) in an Aus-

larity between the pattern of uveitis syndromes

tralian survey may be due to a large proportion

seen in this country and other previously pub-

of the population from Greek or Italian descent (2). The presence of 85 in the Northern Portugal

lished studies. The differential diagnosis of uveitis has been

population may include an intense interchange

revised in the last four decades, due mainly to

with the Middle East since the X W I century, and

either new diseases being recognized (such as

the settlement by Jews, besides the Silk Route theory postulated by Ohno (13).

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AIDS), or to a better understanding of common

Intermediate uveitis still remains frequently

disorders using better diagnostic techniques. In this series the most common type of uveitis

underdiagnosed, specially in young adults; ac-

was the anterior form, usually idiopathic (63.3%)

cording Henderly et a1 ( 1 4 ) , was the most fre-

or associated with a rheumatic disease (17.8%).

quently diagnosed form of uweitis (15.4%),

Patients presenting with acute iridocyclitis have,

pared to the low figures of Perkins and Folk

in half of the cases, a 827 HLA haplotype (6) and

(4.6%)(15),

association with various seronegative spondylo-

com-

Schlaegel (4.6%)(16) and ours ( 4 . 0 % ) .

In a study o f patients with retinal vasculitis,

arthropaties (ankylosing spondylitis, Reiter’s

Graham et a1 (17) concluded that ophthalmological

syndrome, psoriatic arthritis); in our study, 85%

features can discriminate between patients with

of ankylosing spondylitis are B27+ve. Therefore,

isolated retinal vasculitis and those with retinal

it is advisable to refer all 827+ve acute iri-

vasculitis associated with systemic inflammatory

docyclitis patients to a rheumatologist, and to

disease. In o u r study, we were unable to identify

inform the patients about possibility of a

the underlying cause in the 16 retinal vasculitis

recurrence.

cases. Although the importance of syphilis and tubercu-

Toxoplasmic retinochoroiditis, a leading cause of posterior uveitis (7), and generally accepted as congenital, may be difficult to diagnose. W e

losis has decreased dramatically over the past four decades ( 1 6 ) , we are facing a resurgence of

followed the currently held opinion that a positive test by any of the serologic methods is

these diseases, making it imperative to recognise

compatible with the diagnosis of ocular toxo-

For syphilis, the effect of therapy was evaluated

plasmosis, although Rothova et a1

(8) concluded

that a precise diagnosis is not yet feasible and

them as possible causes of ocular inflammation. by the W.D.R.L.

test, followed by FTA-A05 in

positive cases; for tuberculosis, tuberculin skin

negative tests in adults should be the only ones

test and chest X-rays were routinely used.

relied upon. Toxoplasmosis is the first ( 9 , l O ) or second

Sarcoidosis is quite rare (1.5%)

(II,I~) most importante cause of uveitis in some

in our area,

contrary to other studies (1,12,14,15). Although uncommon in this series, AIDS uveitis

surveys, emphasizing the need of early pro-

is rapidly becoming an important differential

phylaxis, diagnosis and specific therapy, namely

diagnosis, mainly due to a large immigration from Africa.

where it is prevalent and widespread: West Africa, West Indies ( 1 ) and Brazil (10). In o u r survey, toxoplasmosis was the second most common cause of uveitis - 9.1% - similarly to Western European series

(I,I~).

We found an association between Beh~et’ssyn-

The aetiology of many cases o f ocular inflammation remains unknown. In our series we are able to establish a presumptive diagnosis in only 232 cases (51.5%). Extensive monitoring of the local population is required in the near future, to

drome (complete type) (4.4% of total) and HLA 65,

detect any small change in epidemiologic patterns,

as is true for Japanese and Mediterranean popula-

so to implement effective treatment measures.

33

Current Eye Research ACKNOWLEDGEMENTS Jorge Palmares i s p a r t i a l l y s u p p o r t e d by a I N I C (Portuguese N a t i o n a l I n s t i t u t e f o r S c i e n t i f i c Research) g r a n t .

73,

CORRESPONDING AUTHOR

Jorge Palmares, Department o f Ophthalmology, F a c u l t y o f Medicine, H o s p i t a l S. So&,

Porto,

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Portugal.

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Uveitis in northern Portugal.

During the past four years 450 patients (247 female - mean age 36 years and 203 male - mean age 34 years) with intraocular inflammation were evaluated...
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