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Severity of Scleritis

and Episcleritis

Maite Sainz de la Maza, MD, PhD, Nada S. Jabbur, MD,


e. Stephen Foster, MD, FACS

Purpose: Inflammation of the wall of the eyeball may extend to adjacent ocular
tissues with blinding consequences and may be associated with potentially lethal sys-
temic disorders. This study was undertaken to evaluate the ocular complications and
systemic disease associations of the different types of scleritis and episcleritis.
Methods: Ocular complications and specific disease association were evaluated in
266 patients (358 eyes) with different types of scleritis (diffuse, nodular, necrotizing,
scleromalacia perforans, and posterior) and episcleritis (simple and nodular).
Results: In patients with scleritis, decrease in vision occurred in 37%, anterior uveitis
was present in 42%, peripheral ulcerative keratitis developed in 14%, glaucoma occurred
in 13%, cataract formed in 17%, fundus abnormalities appeared in 6%, and specific
disease association was uncovered in 57%. These findings were most commonly as-
sociated with necrotizing scleritis. In patients with episcleritis, decreased vision occurred
in 2%, anterior uveitis was present in 11 %, glaucoma developed in 4%, cataract formed
in 2%, and specific disease association was uncovered in 32%. These findings were
similar in simple and nodular episcleritis.
Conclusions: In a patient with scleritis, examination of visual acuity, anterior uvea,
cornea, lens, intraocular pressure, and fundus must be performed in every follow-up
visit, and a meticulous approach for detection of a specific associated disease must be
undertaken since the first visit. Scleritis is more severe than episcleritis, and necrotizing
scleritis is the most severe type of scleritis. Classification of scleritis and episcleritis
provides valuable prognostic information. Ophthalmology 1994;101:389-396

Inflammation of the wall of the eyeball includes a spec- Some patients experience a single, relatively brief, be-
trum that ranges from harmless simple episcleritis to nign episode of scleritis or episcleritis. Others have recur-
painful, sight-threatening, destructive necrotizing scleritis. rent and/or prolonged attacks. As with other recurrent
The inflammatory process may extend to the adjacent conditions, both physicians and their patients are eager
tissues causing ocular complications such as uveitis, ker- to know the probabilities for ocular and systemic com-
atitis, glaucoma, cataract, optic neuritis, macular edema, plications of scleritis and episcleritis. Because of the com-
subretinal mass, annular ciliochoroidal detachment, se- parative rarity of these diseases, statistics on severity and
rous retinal detachment, or perforation of the globe. In prognosis are not so easy to find.
addition, scleritis may be associated with potentially lethal The excellent episcleritis and scleritis survey and trea-
systemic disorders. 1- 8 tise performed by Watson and Hayreh 7 at Moorfields Eye
Hospital in London in 1976 provided us with an excep-
tional perspective of the spectrum of these diseases. They
Originally received: March 31,1993. proposed a classification based on the anatomic site and
Revision accepted: August 10, 1993.
clinical appearance of the inflammation at presentation.
From the Ocular Immunology Service and the Hilles Immunology Lab-
oratory, Massachusetts Eye and Ear Infirmary, Harvard Medical School,
This classification was useful because the majority of the
Boston. patients remained in the same clinical type throughout
Supported in part by MEC/Fulbright grant FU 90/37276861 and the the course of their disease. A subsequent study performed
Susan Morse Hilles Fund, Boston, Massachusetts. by Tuft and Watson 8 at the same hospital in 1991 on
Reprint requests to C. Stephen Foster, MD, Immunology Service, Mas- progression of scleritis confirmed these findings; only 8%
sachusetts Eye and Ear Infirmary, 243 Charles St, Boston, MA 02114. of their patients progressed from one type of scleritis to

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Ophthalmology Volume 101, Number 2, February 1994

another. This classification also was found to be useful mass, optic nerve swelling, macular edema, annular cili-
because it relates clinically to the severity of the disease. ochoroidal detachment, serous retinal detachment, intra-
Different types of scleritis differ in degree of ocular and retinal deposits, and retinal striae; and (7) specific disease
systemic complications. association, ascribed to patients as a result of compatible
Fifteen years after Watson and Hayreh 7 did their study historical, review of systems, laboratory, and biopsy data.
on episcleral and scleral inflammation, we performed a Data were analyzed using a customized database soft-
scleritis and episcleritis survey at the Massachusetts Eye ware and compared between patients with scleritis and
and Ear Infirmary in Boston, and compared patient char- patients with episcleritis, between patients with different
acteristics, ocular complications, and systemic disease as- types of scleritis (diffuse anterior, nodular anterior, nec-
sociations between patients with scleritis and patients with rotizing anterior, scleromalacia perforans anterior, and
episcleritis, between patients with different types of scleri- posterior scleritis), between patients with different types
tis, and between patients with different types of episcleritis. of episcleritis (simple and nodular), and between patients
Our study on scleritis and episcleritis patients is the second with necrotizing scleritis and patients with other types of
largest to date, after the one performed in England. We scleritis. We chose to report the results in terms of patients
found, as Watson and co-workers did, that classification (age, sex, bilaterality, decrease in vision, anterior uveitis,
of scleral and episcleral inflammation is useful as a ba- peripheral ulcerative keratitis, glaucoma, cataract, fundus
rometer of severity and as an indicator for therapeutic findings, and specific disease association) because results
decisions. of both eyes of a patient are dependent. To determine the
extent that an observed series of proportions differed from
a theoretic or expected distribution of proportions, sta-
Patients and Methods tistical analysis was performed using the chi-square test
(statistically significant: P < 0.05) for all conditions except
We reviewed the records of all patients with scleritis or age. Patients with scleromalacia perforans were not in-
episcleritis seen on the Immunology Service of the Mas- cluded in the chi-square statistical studies due to low
sachusetts Eye and Ear Infirmary during the II-year pe- number (the expected values were sometimes less than
riod from May 1980 to May 1991 on whom follow-up 5). The condition "age" was statistically analyzed between
information was adequate. For the purpose of this study, three or more groups with the Kruskal-Wallis nonpara-
scleritis was defined by the following characteristics: (1) metric test and between two groups with the Mann-Whit-
edema of the sclera and the episclera which displaces both ney U nonparametric test.
the superficial and deep edges of the thin slit-lamp beam
forward as the beam makes an excursion across the surface
of the sclera; (2) associated congestion of the superficial
Results
and the deep episcleral vessels; congestion of the deep
episcleral vessels remains after the application of 10% Patient Characteristics
phenylephrine drops. Episcleritis was defined by the fol- A total of 266 patients (358 eyes) constituted the study
lowing characteristics: (1) edema of the episclera which population. One hundred seventy-two of the patients had
displaces the superficial edge of the thin slit-lamp beam scleritis and 94 had episcleritis (Table 1). Most of the pa-
forward as the beam makes an excursion across the surface tients with scleritis had anterior scleritis (94%), including
of the sclera; (2) associated congestion of the superficial 77 (45%) with diffuse scleritis, 39 (23%) with nodular
episcleral vessels, which disappears after the use of 10% scleritis, 39 (23%) with necrotizing scleritis, and 6 (3%)
phenylephrine drops. Scleritis and episcleritis were clas- with scleromalacia perforans. Seventy-eight ofthe patients
sified after the anatomic types proposed by Watson and with episcleritis (83%) had simple episcleritis and 16 (17%)
Hayreh.7 Scleritis was divided in anterior and posterior. had nodular episcleritis. Episcleritis did not progress to
Anterior scleritis included diffuse, nodular, necrotizing scleritis in these patients.
with inflammation (necrotizing), and necrotizing without The mean age of patients with scleritis was 51.59 years
inflammation (scleromalacia perforans) scleritis. Epis- (range, 11-87 years) (Table 2). Patients with necrotizing
cleritis was divided in simple and nodular. scleritis (mean age, 61.92 years) were older than those
Patient data, including age, sex, scleral disease laterality, with other types of scleritis (P = 0.0001) (Tables 2 and
and follow-up period, were recorded. The following con- 3), including patients with nodular scleritis (mean age,
ditions were considered ocular or systemic complications: 49.80 years), posterior scleritis (mean age, 49.10 years),
( 1) decrease in vision, defined as decrease in visual acuity and diffuse scleritis (mean age, 46.82 years). Patients with
greater than or equal to two Snellen lines at the end of scleritis were older than patients with episcleritis (mean
the follow-up period, or visual acuity of 20/80 or worse age, 43.39 years; range, 14-79 years) (P = 0.0012) (Tables
at presentation (worse of the 2 eyes); (2) anterior uveitis 2 and 4).
detected at some point during the course of the disease; Sixty-seven of the patients with scleritis (39%) were
(3) peripheral ulcerative keratitis, defined as corneal men and 105 (61%) were women (Table 5). Twenty-four
stromal infiltration with epithelial defect adjacent to the of the patients with episcleritis (26%) were men and 70
region of the scleral inflammation which may progress (74%) were women. Female predominance did not differ
circumferentially and centrally; (4) glaucoma; (5) cataract; significantly among patients with different types of scleri-
(6) fundus findings, including choroidal folds, subretinal tis, or among those with different types of episcleritis.

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Sainz de la Maza et al . Severity of Scleritis and Episcleritis

Table 1. Scleritis and Episcleritis Classification


No. of No. of No. of No. of
Diagnosis Patients (%) Eyes Type Patients (%) Eyes
Scleritis 172 (64.7) 231 Diffuse 77 (44.77) 107
Nodular 39 (22.67) 50
Necrotizing 39 (22.67) 48
Scleromalacia 6 (3.49) 11
Posterior 11 (6.40) 15
Episcleritis 94 (35.3) 127 Simple 78 (82.98) 108
Nodular 16 (17.02) 19
Total 266 358

Bilateral inflammation was present in 34% of patients tistically significant (P = 0.0001): necrotizing scleritis was
with scleritis (Table 6); it was more frequent in scleroma- the most common type of scleritis associated with a de-
lacia perforans (83%) as opposed to other types of scleritis, crease in vision (82%) (P = 0.0001) (Tables 3 and 7),
including diffuse scleritis (39%), posterior scleritis (36%), followed by posterior scleritis (45%). The most common
nodular scleritis (28%), and necrotizing scleritis (23%). reasons for decrease in vision were anterior uveitis and/
Bilateral inflammation was present in 35% of the patients or peripheral ulcerative keratitis in necrotizing scleritis,
with episcleritis; it was more frequent in simple episcleritis and macular edema in posterior scleritis.
(40%) than in nodular episcleritis (12%) (P = 0.0375). A decrease in visual acuity greater than or equal to two
The mean follow-up period was 15 months (range, I- Snellen lines at the end of the follow-up period, or a visual
II years) for patients with scleritis and 11 months (range, acuity of 20/80 or worse at presentation (worse of the 2
1 month-II years) for patients with episcleritis. eyes) was detected in 2% of the patients (3 eyes) with epi-
scleritis (Table 7); this decrease in vision was attributed
to the natural development of cataract in all three eyes.
Ocular Complications Decrease in vision was more commonly associated with
scleritis (37%) than with episcleritis (2%) (P = 0.0001)
Decrease in Vision. A decrease in visual acuity greater (Tables 4 and 7).
than or equal to two Snellen lines at the end of the follow- Anterior Uveitis. Anterior uveitis was present in 42%
up period, or a visual acuity of 20/80 or worse at presen- of the patients (95 eyes) with scleritis (Table 8). The an-
tation (worse of the 2 eyes) was recorded in 37% of the terior uveitis associated with scleritis ranged from mild
patients affected by scleritis (Table 7). The association to moderate intensity and most commonly appeared dur-
between decrease in vision and type of scleritis was sta-

Table 3. Statistical Associations between


Table 2. Age Distribution in Scleritis Necrotizing Scleritis and Other Conditions
and Episcleritis
Condition p-
Age (yrs) Age 0.0001
Diagnosis/Type Mean Range P Sex NS
Scleritis 51.59 11-87 Bilaterality NS
Diffuse 46.82 11- 78 Disease association 0.0001
Nodular 49.80 18-82 Decrease in visiont 0.0001
Necrotizing 61.92 34-87 0.0001- Anterior uveitis 0.0001
Scleromalacia 61.67 38-75 Peripheral ulcerative keratitis 0.0001
Posterior 49.10 26-67
Episcleritis 43.39 14-79 Glaucoma 0.0287
Simple 42.09 14-79 NSt Cataract 0.0001
Nodular 49.75 20-78
NS = not significant.
Total 48.83 11-87 • Chi-square between necrotizing scleritis and nonnecrotizing scleritis
(diffuse, nodular, scleromalacia, and posterior) for all conditions except
NS = not significant. age (Mann-Whitney U).
- Kruskal-Wallis nonparametric test between the different types of scleritis. t Decrease in visual acuity greater than or equal to two Snellen lines at
t Mann-Whitney U non parametric test between the different types of the end of the follow-up period, or visual acuity of 20/ SO or worse at
episcleritis. presentation; mean follow-up, 15 months (range, 1-132 months).

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Ophthalmology Volume 101, Number 2, February 1994

Table 4. Statistical Associations between Scleritis Table 6. Bilaterality in Scleritis and Episcleritis
or Episcleritis and Other Conditions
Diagnosis/Type Bilaterality (Ofo) Total Chi-square
Condition P* Scleritis 59 (34.30) 172
Age 0.0012 Diffuse 30 (38.96) 77
Sex 0.0274 Nodular 11 (28.21) 39
Necrotizing 9 (23.08) 39 NS*
BUaterality NS Scleromalaciat 5 (83.33) 6
Disease association 0.0001 Posterior 4 (36.36) 11
Decrease in visiont 0.0001 Episcleritis 33 (35.11) 94
Anterior uveitis 0.0001 Simple 31 (39.74) 78 0.0375
Peripheral ulcerative keratitis 0.0001 Nodular 2 (12.50) 16
Glaucoma O.oz5
NS = not significant.
Cataract 0.0003
• p > 0.05.
NS = not significant. t Not included in the chi-square study due to low number of patients.
• Chi-square between scleritis and episcleritis for all conditions except
age (Mann-Whitney U).
t Decrease in visual acuity greater than or equal to two Snellen lines at
the end of the follow-up period, or visual acuity of 20/80 or worse at scleritis (Table 9). The presence of peripheral ulcerative
presentation; mean follow-up period for scleritis, 15 months (range, 1 keratitis was statistically associated with the type of scleritis
month-II years); mean follow-up period for episcleritis, 11 months (range, (P = 0.0001): peripheral ulcerative keratitis was present
1 month-lO years).
in 41 % of the patients with necrotizing scleritis (P =
0.000 I) (Tables 3 and 9), 6% of the patients with diffuse
scleritis, and 8% of the patients with nodular scleritis.
ing the late course of the scleral inflammation. The pres- None of the patients with episcleritis had peripheral ul-
ence of anterior uveitis was statistically associated with cerative keratitis. There was obviously a statistical asso-
the type of scleritis (P = 0.0012): it was present in 69% ciation between the presence of peripheral ulcerative ker-
of the patients with necrotizing scleritis (P = 0.0001) (Ta- atitis and scleritis (P = 0.000 I) (Tables 4 and 9).
bles 3 and 8) and in 45% of the patients with posterior Glaucoma. Glaucoma was detected in 13% ofthe pa-
scleritis. Six of the seven eyes with posterior scleritis and tients (30 eyes) with scleritis (Table 10); of those, 13 eyes
anterior uveitis also had some degree of anterior scleritis. had keratitis and uveitis, 3 had keratitis without uveitis,
Anterior uveitis was present in II % of the patients (12 and 6 had uveitis without keratitis. The patient with pos-
eyes) with episcleritis (Table 8). The uveitis associated terior scleritis had a secondary angle closure glaucoma
with episcleritis was mild. Anterior uveitis was more due to annular ciliochoroidal detachment. Although the
commonly associated with scleritis (42%) than with epi- presence of glaucoma was not associated with the type of
scleritis (II %) (P = 0.000 I) (Tables 4 and 8).
Peripheral Ulcerative Keratitis. Peripheral ulcerative
keratitis was present in 14% of the patients (34 eyes) with Table 7. Decrease in Vision in Scleritis
and Episcleritis*
Table 5. Sex Distribution in Scleritis Diagnosis/Type Loss of Vision (Ofo) Total Chi-square
and Episcleritis
Scleritis 64 (37.21) 172
No. of No. of Diffuse 20 (25.97) 77
Males Females Chi- Nodular 5 (12.82) 39
Diagnosis/Type (Ofo) (Ofo)* Total square Necrotizing 32 (82.05) 39 0.0001
Scleromalaciat 2 (33.33) 6
Scleritis 67 (38.95) 105 (61.05) 172 Posterior 5 (45.45) 11
Diffuse 33 (42.86) 44 (57.14) 77 Episcleritis 2 (2.13) 94
Nodular 13 (33.33) 26 (66.67) 39 Simple 2 (2.56) 78 NSf
Necrotizing 17 (43.59) 22 (56.41) 39 NS* Nodular 0 16
Scleromalaciat 1 (16.67) 5 (83.33) 6
Posterior 3 (27.27) 8 (72.73) 11 NS = not significant.
Episcleritis 24 (25.53) 70 (74.47) 94 • Decrease in visual acuity greater than or equal to two Snellen lines
Simple 21 (26.92) 57 (73.08) 78 NS* (worse of the 2 eyes) at the end of the follow-up period, or visual acuity
Nodular 3 (18.75) 13 (81.25) 16 of 20/80 or worse at presentation; mean follow-up period for scleritis,
15 months (range, 1 month-ll years); mean follow-up period for epis-
NS = not significant. cleritis, 11 months (range, 1 month-l0 years) .
• p > 0.05. t Not included in the chi-square study due to low number of patients.
t Not included in the chi-square study due to low number of patients. t p > 0.05.

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Table 8. Anterior Uveitis in Scleritis Table 10. Glaucoma in Scleritis and Episcleritis
and Episcleritis
Diagnosis /Type Glaucoma (%) Total Chi-square
Anterior Scleritis 22 (12.79) 172
Diagnosis/Type Uveitis (%) Total Chi-square Diffuse 7 (9.09) 77
Scleritis 73 (42.44) 172 Nodular 4 (10.26) 39
Diffuse 28 (36.36) 77 Necrotizing 9 (23.08) 39
Nodular 11 (28.21) 39 Scleromalaciat 1 (16.67) 6
Necrotizing 27 (69.23) 39 0.0012 Posterior 1 (9.09) 11
Scleromalacia" 2 (33.33) 6 Episcleritis 4 (4.25) 94
Posterior 5 (45.45) 11 Simple 3 (3.85) 78
Episcleritis 10 (10.64) 94 Nodular 1 (6.25) 16
Simple 9 (11.54) 78 NSt
Nodular 1 (6.25) 16 NS = not significant.
• P > 0.05.
NS = not significant. t Not included in the chi-square study due to low number of patients.
• Not included in the chi-square study due to low number of patients.
t P > 0.05.

with posterior scleritis. In our series of 11 patients with


posterior scleritis (15 eyes), nine eyes had choroidal folds,
scleritis, it was present in 23% of the patients (13 eyes) six had subretinal mass, seven had disc edema, and six
with necrotizing scleritis (P = 0.0287) (Tables 3 and to). had macular edema. Annular ciliochoroidal detachments
Glaucoma was present in 4% of the patients (5 eyes) were present in three eyes, two of which had bullous serous
with episcleritis (Table 10); of those, four eyes had chronic retinal detachments. Intraretinal deposits were found in
open-angle glaucoma before the onset of episcleritis. three eyes. Retinal striae were detected in two eyes. There
Glaucoma was more commonly associated with scleritis were no episcleritis patients with fundus abnormalities.
(13%) than with episcleritis (4%) (P = 0.025) (Tables 4
and to).
Cataract. Cataract was present in 17% of the patients Disease Associations
(38 eyes) with scleritis (Table 11). Sixteen of these eyes An associated disease was found in 98 patients (57%) with
had concomitant anterior uveitis, and most had posterior scleritis (Table 12), including 82 patients (48%) with con-
subcapsular cataract. The presence of cataract was statis- nective tissue or vasculitic diseases, 12 (7%) with infectious
tically associated with the type of scleritis (P = 0.0002): diseases, and 4 (2%) with miscellaneous diseases (rosacea,
cataract was present in 41 % of the patients with necrotizing atopy, gout, and a foreign body, 1 patient each). The de-
scleritis (P = 0.0001) (Tables 3 and 11),9% of the patients tection of an associated disease was strongly associated
with diffuse scleritis, and 10% of the patients with nodular with the type of scleritis (P = 0.0001): the most common
scleritis. type of scleritis associated with a specific disease was nec-
Age-related nuclear sclerotic cataract was present in rotizing scleritis (95%) (P = 0.0001) (Tables 3 and 12)
2% of the patients (3 eyes) with episcleritis. Cataract was followed by scleromalacia perforans (67%), posterior
more commonly associated with scleritis (17%) than with scleritis (45%), diffuse scleritis (45%), and nodular scleritis
episcleritis (2%) (P = 0.0003) (Tables 4 and 11).
Fundus Findings. Few patients with diffuse, nodular,
or necrotizing scleritis had macular edema or optic nerve
swelling. Most fundus findings were ascribed to patients Table 11. Cataract in Scleritis and Episcleritis

Diagnosis/Type Cataract (%) Total Chi-square


Scleritis 29 (16.86) 172
Table 9. Peripheral Ulcerative Keratitis in Scleritis Diffuse 7 (9.09) 77
Nodular 4 (10.26) 39
Diagnosis/Type PUK(%) Total Chi-square Necrotizing 16 (41.02) 39 0.0002
Scleritis 24 (13.95) 172 Scleromalacia· 1 (16.67) 6
Diffuse 5 (6.49) 77 Posterior 1 (9.09) 11
Nodular 3 (7.69) 39 Episcleritis 2 (2.13) 94
Necrotizing 16 (41.03) 39 0.0001 Simple 2 (2.56) 78 NSt
Scleromalacia" 0 6 Nodular 0 16
Posterior 0 11
NS = not significant.
PUK = peripheral ulcerative keratitis. • Not included in the chi-square study due to low number of patients.
• Not included in the chi-square study due to low number of patients. t P > 0.05 .

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Ophthalmology Volume 101, Number 2, February 1994

Table 12. Associated Disease in Scleritis tients (2% of patients with episcleritis); diagnoses included
and Episcleritis systemic lupus erythematosus in one patient and Cogan
syndrome in another patient.
Associated An associated disease was more commonly associated
Diagnosis/Type Disease (%) Total Chi-square with scleritis (57%) than with episcleritis (32%) (P
Scleritis 98 (56.98) 172 0.0001) (Tables 4 and 12).
Diffuse 35 (45.45) 77
Nodular 17 (43.59) 39
Necrotizing 37 (94.87) 39 0.0001 Discussion
Scleromalacia· 4 (66.67) 6
Posterior 5 (45.45) 11 The results of this analysis provide insights into the se-
Episcleritis 30 (31.91) 94 verity of scleritis and episcleritis and their respective types.
Simple 22 (28.21) 78 NSt Objective data on the incidence of episcleritis and scleritis
Nodular 8 5( 0) 16 are very difficult to obtain because these inflammatory
conditions are not extremely common and patient group
NS = not significant.
characteristics can vary greatly, depending on the insti-
• Not included in the chi-square study due to low number of patients. tution at which the analyses are performed. Data from
t p < 0.05. highly selected patient tertiary referral centers, such as the
Massachusetts Eye and Ear Infirmary, will inevitably be
biased to higher numbers of patients with scleritis, com-
pared with patients with episcleritis, and to higher num-
(44%). Within the connective tissue and vasculitic diseases, bers of patients with more destructive types of scleritis,
rheumatoid arthritis was the most common associated compared with patients with more benign types of scleritis.
disease (32 patients), followed by Wegener granulomatosis It is interesting to compare scleritis patient character-
(14 patients), relapsing polychondritis (11 patients), ar- istics between tertiary referral centers. The scleritis patient
thritis and inflammatory bowel disease (7 patients), and population from our Immunology Service showed a higher
systemic lupus erythematosus (7 patients); other diseases proportion of patients with necrotizing scleritis and a
included Reiter syndrome (3 patients), psoriatic arthritis lower proportion of patients with nodular scleritis than
(2 patients), polyarteritis nodosa (2 patients), ankylosing those reported in two large retrospective studies performed
spondylitis (I patient), Beh<;et disease (1 patient), giant by Watson and Hayreh 7 in 1976, and by Tuft and Watson8
cell arteritis (I patient), and Cogan syndrome (l patient). in 1991 , both at Moorfields Eye Hospital in London. Our
Bacteria (5 patients) were the most frequent microbes iso- series of patients with scleritis showed a mean age (51.6
lated in the cases of infectious scleritis, followed by viruses years) and a female-to-male ratio (1.57: 1) comparable with
(4 patients), parasites (2 patients), and fungi (I patient). the figures of51.5 years and 1.27: 1, respectively, reported
Scleritis was the first manifestation of a systemic con- by Tuft and Watson. 8 Scleritis was bilateral in 34% of our
nective tissue disease or vasculitic disease in 26 patients patients, which is between the figures of 45% reported by
(15% of patients with scleritis). Diagnoses were made 1 Watson and Hayreh 7 and 25.5% reported by Tuft and
month to 22 years after the onset of scleritis in these pa- Watson. 8
tients (mean duration, 37 months). Wegener granulo- Scleritis is a severe, destructive disease that may cause
matosis (14 patients) and relapsing polychondritis (6 pa- decrease in vision by leading to one or more ocular com-
tients) were the most frequent diseases discovered; other plications such as anterior uveitis, keratitis, glaucoma,
diagnoses included rheumatoid arthritis (2 patients), Rei- secondary cataract, or fundus changes. Visual acuity
ter syndrome (I patient), polyarteritis nodosa (1 patient), should pe evaluated in every follow-up visit of a patient
systemic lupus erythematosus (1 patient), and Beh<;et dis- with scleritis. In our series of patients with scleritis, 37%
ease (1 patient). Necrotizing scleritis was found to be the had a decrease in vision equal or greater to two Snellen
most frequent type in patients whose scleritis appeared as lines at the end of the follow-up period, or a visual acuity
a first manifestation of a systemic disease (15 patients). of 20/80 or worse at presentation; decrease in vision was
An associated disease was found in 32% of the patients least common in nodular scleritis, intermediate in diffuse
with episcleritis (Table 12), including 12 patients (13%) scleritis, and most frequent in necrotizing scleritis followed
with connective tissue or vasculitic diseases (3 patients by posterior scleritis. The most common reasons for de-
with rheumatoid arthritis, 3 with arthritis and inflam- crease in vision were anterior uveitis and/or peripheral
matory bowel disease, 2 with psoriatic arthritis, 1 with ulcerative keratitis. In Tuft and Watson's8 series of patients
systemic lupus erythematosus, 1with Reiter syndrome, with scleritis, decrease in vision (defined as a permanent
1 with Beh<;et disease, and 1 with Cogan syndrome), 2 drop in Snellen acuity of 2 or more lines) was least com-
(2%) with infectious diseases (1 patient with herpes zoster mon in diffuse scleritis, intermediate in nodular scleritis,
infection and 1 with herpes simplex infection), and 16 and most frequent in posterior scleritis followed by nec-
(17%) with miscellaneous diseases (7 patients with atopy, rotizing scleritis.
7 with rosacea, 1with chemical injury, and 1 with gout). Anterior uveitis and glaucoma are caused by extension
Episcleritis was the first manifestation of a systemic of the scleral inflammation. The presence of anterior uve-
connective tissue disease or vasculitic disease in two pa- itis in scleritis, particularly when associated with glau-

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Sainz de la Maza et al . Severity of Scleritis and Episcleritis

coma, should be regarded as a grave sign. Fraunfelder and of complications which could cause permanent loss of
Watson,9 in a series of 30 enucleated eyes with a primary vision.
histologic diagnosis of scleritis, found that 68% had signs Scleritis may also be a manifestation ofa potentially
of having had uveitis and 46% had signs of having had life-threatening systemic autoimmune disease. I- 8 Delay
glaucoma; scleritis with uveitis and glaucoma was the most in diagnosis and initiation of appropriate therapy can re-
common combination of complications leading to enu- sult in vision loss, organ damage, and even death. In our
cleation. Anterior uveal inflammation and intraocular series of patients with scleritis, 57% had an associated dis-
pressure should be evaluated in every follow-up visit of a ease; diffuse scleritis was present in 45%, nodular scleritis
patient with scleritis. In our series of patients with scleritis, in 44%, necrotizing scleritis in 95%, scleromalacia per-
42% had anterior uveitis (41 % of the eyes), the majority forans in 67%, and posterior scleritis in 45%. Interestingly,
of those with necrotizing scleritis. This is a higher figure these proportions are much higher than those found by
than the one reported by Watson and Hayreh (30% of the Tuft and Watson. 8 In their series of patients with scleritis,
eyes), probably because of our higher proportion of pa- 25% had an associated disease; diffuse scleritis was present
tients with necrotizing scleritis. Our proportion of scleritis in 13%, nodular scleritis in 28%, necrotizing scleritis in
patients with glaucoma (13%) (13% of the eyes) is similar 45%, and posterior scleritis in 19%. 8 In both tertiary re-
to the proportions reported by Watson and Hayreh 7 (12% ferral center studies, rheumatoid arthritis was the most
of the eyes) and Tuft and Watson 8 (12% of the patients). common disease associated with scleritis, followed by
Anterior uveitis and glaucoma were present in 8% of our Wegener granulomatosis, relapsing poiychondritis, ar-
patients, the majority of those with necrotizing scleritis. thritis and inflammatory bowel disease, and systemic lu-
The detection of anterior uveitis and/or glaucoma ac- pus erythematosus. Medical and family history, meticu-
companying scleritis requires early and aggressive therapy lous review of systems, and a standard physical exami-
to control the scleral inflammation. nation including not only the eye but also the ears, nose,
Peripheral ulcerative keratitis also is caused by exten- mouth, skin, and joints is essential in patients with scleri-
sion of scleral inflammation. The presence of peripheral tis, even at their first episode. Specific systemic manifes-
ulcerative keratitis in scleritis also should be regarded as tations may lead the ophthalmologist to suspect certain
a grave sign because the layers of the peripheral cornea types of autoimmune diseases. Subsequent laboratory, x-
progressively are destroyed, leaving the cornea so thin it rays, or pathologic studies designed on the basis of infor-
can easily perforate. Corneal involvement should be eval- mation obtained from a careful review of systems ("tar-
uated in every follow-up visit of a patient with scleritis. geted workups") help to confirm the initial diagnostic
In our series, 14% of the patients with scleritis had pe- impressions. It must be emphasized that because systemic
ripheral ulcerative keratitis, and the majority ofthose had autoimmune diseases sometimes evolve slowly, one series
necrotizing scleritis. The detection ofperipheraI ulcerative of investigations may be insufficient, and sequential di-
keratitis accompanying scleritis requires early and ag- agnostic studies may be necessary to reach a diagnosis.
gressive therapy to control the scleral inflammation. Each of these steps in this approach to patients with scle-
Cataract formation in scleritis may be caused either ritis has been reviewed in detail elsewhere. 13
by longstanding anterior uveitis or by long-term systemic Episcleritis, unlike scleritis, is a benign disorder that
or local steroid use, and is most common in the necro- rarely causes decrease in vision, because the associated
tizing type of scleritis. Cataract formation should be complications such as anterior uveitis, keratitis, glaucoma,
evaluated in every follow-up visit of a patient with scleri- or secondary cataract are uncommon and are never se-
tis. A comparison between a group of patients without vere. 5,7 In our series of patients with episcleritis, only 2%
scleritis and a group of patients with scleritis, both re- had decrease in visual acuity equal to or greater than two
ceiving long-term systemic or local steroid treatment, Snellen lines at the end of the follow-up period, or a visual
showed an increased risk of development of posterior acuity of 20/80 or worse at presentation. This decrease
subcapsular cataract in the group of patients with scleritis in vision was attributed to the natural development of
(36%) as opposed to the group of patients without scleritis cataract in all of the patients. Mild peripheral corneal
(11.5%). 5 Our proportion of patients with scleritis who changes such as superficial and midstromal inflammatory
had cataract (17%) (16% of the eyes) is higher than the cell infiltration can be observed occasionally in patients
proportions reported by Watson and Hayreh 7 (7% of the with episcleritis in the area adjacent to the conjunctival
eyes) and Tuft and Watson 8 (6% of the patients), probably and episcleral edema but these infiltrates never progress
because of our higher percentage of patients with nec- to peripheral ulcerative keratitis. They rarely are perma-
rotizing scleritis. nent unless the attacks are recurrent in the same area.
Fundus findings in scleritis are most often ascribed to Intraocular structures are almost never involved. In a few
patients with posterior scleritis. Although posterior scleritis cases, cells in the anterior chamber and aqueous flare may
often is associated with anterior scleritis, it also may occur appear, but these are never severe. Glaucoma or cataract
alone, in which case the absence of anterior scleral in- are not directly attributed to the episcleral inflammation
volvement makes the diagnosis difficult. 10-12 In addition, unless they are induced by steroid treatment. 5 ,7 Some pa-
if the concomitant anterior scleritis is severe, posterior tients with episcleritis may have an associated disease but
scleritis may be overlooked. Fundus evaluation should be the majority defies all attempts to discover a cause. 6- 8 A
performed in every follow-up visit of a patient with scleri- "targeted workup" designed on the basis of information
tis. Early diagnosis leads to early therapy and prevention obtained from a careful review of systems and from a

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meticulous examination of the patient is appropriate in 5. McGavin DDM, Williamson J, Forrester JV, et al. Epi-
cases of persistent or recurrent episcleritis attacks. 13 Simple scleritis and scleritis: a study of their clinical manifestations
episcleritis does not differ significantly from nodular epi- and association with rheumatoid arthritis. Br J Ophthalmol
scleritis with respect to ocular complications and systemic 1976;60: 192-226.
6. Lyne AJ, Pitkeathley DA. Episcleritis and scleritis: associ-
disease associations.
ation with connective tissue disease. Arch Ophthalmol
Diagnosis and classification of inflammatory diseases 1968;80: 171-6.
of the wall of the eyeball provide valuable prognostic in- 7. Watson PG, Hayreh SS. Scleritis and episcleritis. Br J
formation that may be used as a guide for therapeutic Ophthalmol 1976;60:163-91.
decisions. 8. Tuft SJ, Watson PG. Progression of scleral disease. Oph-
thalmology 1991;98:467-71.
9. Fraunfelder Fr, Watson PG. Evaluation of eyes enucleated
References for scleritis. Br J Ophthalmol 1976;60:227-30.
10. Calthorpe CM, Watson PG, McCartney ACE. Posterior
1. Fong LP, Sainz de la Maza M, Rice BA, et al. Immuno- scleritis: a clinical and histological survey. Eye 1988;2:267-
pathology of scleritis. Ophthalmology 1991 ;98:472-9. 77.
2. Foster CS, Forstot SL, Wilson LA. Mortality rate in rheu- 11. Cleary PE, Watson PG, McGill n, Hamilton AM. Visual
matoid arthritis patients developing necrotizing scleritis or loss due to posterior segment disease in scleritis. Trans
peripheral ulcerative keratitis. Effects of systemic immu- Ophthalmol Soc U K 1975;95:297-300.
nosuppression. Ophthalmology 1984;91: 1253-63. 12. Benson WE, Shields JA, Tasman W, Crandall AS. Posterior
3. Foster CS. Immunosuppressive therapy for external ocular scleritis. A cause of diagnostic confusion. Arch Ophthalmol
inflammatory disease. Ophthalmology 1980;87: 140-50. 1979;97: 1482-6.
4. Hakin KN, Watson PG. Systemic associations of scleritis. 13. Foster CS, Sainz de la Maza M. The Sclera. New York:
Int Ophthalmol Clin 1991;31 (3): 111-29. Springer-Verlag, 1993; chap 3: 59-94.

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