Ann. rheum. Dis. (1971), 30, 343
Scleritis and rheumatoid arthritis
M. I. V. JAYSON*j AND D. E. P. JONES*tRoyal National Hospitalfor Rheumatic Diseases*, Bath, the Department of Medicine, University of Bristol+,and the Bath Eye Infirmaryt
Holthouse (1893) presented an unusual case ofulceration of the conjunctiva and sclera in a patientwho also suffered from rheumatoid arthritis anddescribed the typical histology of the condition.Nevertheless, van der Hoeve (1934), in the firstdescription of scleromalacia perforans, whichoccurred in three cases in association with a poly-arthritis, regarded the condition as a primarydegeneration of the sclera. Thereafter, however, theassociation of scleritis with rheumatoid arthritisand other connective tissue diseases has been wellestablished (Watson and Lobascher, 1965; Sevel,1967; Lyne and Pitkeathley, 1968) and is regarded asbeing due to the development of rheumatoidnodules within the sclera (Verhoeff and King, 1938;Edstr6m and Osterlind, 1948; Anderson andMargolis, 1952; Sevel, 1965; Smiley and Bywaters,1967; Watson, Hayreh, and Awdry, 1968). Theprevalence of scleritis in rheumatoid arthritis is notwell established and has been thought to be a rarecomplication (Smiley and Bywaters, 1967). Smith(1957) found one case of scleral involvementrecorded in a retrospective study of the records of465 rheumatoid patients, and by personal com-
munication, Sevel (1965) estimated the prevalenceto be one in three thousand cases.
This study has been directed at determining theprevalence and nature of scleritis in rheumatoidarthritis and the place of this complication in thenatural history of rheumatoid disease.
MethodA careful examination of the eyes, with particularattention to scleral inflammation and thinning, wasperformed in 142 consecutive in-patients and out-patientswith definite or classical rheumatoid arthritis (Ropes,Bennett, Cobb, Jacox, and Jessar, 1959) together withthe same number of controls (hospital in-patients withnon-inflammatory orthopaedic conditions matched forage and sex). A further five rheumatoid patients speciallyreferred because they were known to have scleritiswere included separately. Slit-lamp examinations of theeyes were performed in every positive case. Scleritisand episcleritis were differentiated according to thecriteria summarized in Table I, which are based on thedescriptions of Watson and others (1968). Inactivescleritis was diagnosed only when there was a past historyof scleral inflammation, a definite diagnosis of scleritishad been made at that time, and marked scleral thinninghad subsequently developed.
Table I Differentiation between scleritis and episcleritis
Inflammation of the episcleral (sub-conjunctival) tissues
Usually fairly acute in onset and resolveswithin 3 weeks
Discomfort only, vision not affected
Simple or nodular
Conjunctival and superficial episcleralvascular plexuses affected, nodules aresuperficial and can be movedCornea not affected
No residual physical signs
Inflammation of the scleral coat of the eye
Acute or slow in onset; may resolve in a fewweeks or last 1 to 2 years
May cause severe pain and general malaise,vision may be blurred
Simple, diffuse, nodular, or necrotizing
Deep episcleral vascular plexus affected,superficial vessels may also be affected,nodules are deep and fixed to the scleraCornea may be affected (sclerokeratitis)
Followed by scleral thinning; may haveserious complications, including perforation
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is: first published as 10.1136/ard.30.4.343 on 1 July 1971. Dow
344 Annals of the Rheumatic Diseases
A detailed study was made of both the joint andsystemic manifestations of the rheumatoid disease andcomparisons were drawn between those rheumatoidpatients who developed scleritis and those who did not.Whenever possible, follow-up assessments were
performed to determine the course of the eye disease andits relationship to activity of the arthritis.
Results (Tables II to IV)Evidence of scleritis was found in nine of the 142rheumatoid patients who were screened but in noneof the controls, a highly significant association(X2= 7 * 34; P < 0 *01). Ofthese nine, six showed activeand three inactive scleritis, and of the five added tothe series all showed active scleritis.Both eyes were affected in ten of the fourteen
patients with scleritis. The superior quadrant ofthe eye was involved most frequently and theinferior quadrant least often (Fig. 1), but thedifferences were not statistically significant. Allpatients with active scleritis showed diffuse in-flammation in the sclera, but four of these alsohad scleral nodules.
a (:1:11111::) 9 5 7
RIGHT (14) LEFT (10)
FIG. 1 Sites ofscleral inflammation infourteen rheumatoidpatients
The mean age and length of history of arthritiswere similar in the patients with and without
scleritis. The erythrocyte sedimentation rate washigher in the former group but the difference was ofborderline statistical significance. There was asignificant association between scleritis and rheu-matoid nodules and, in particular, the four patientswith scleral nodules all had rheumatoid nodules.There was an increased ftequency of pericarditis/pleurisy among the scleritics, the two patientsboth having suffered pericarditis and one alsopleurisy, eventually undergoing pericardectomy andpleurectomy. Evidence of arteritis was found in tenof the fourteen scleritic patients and in only six-teen of the remaining rheumatoid patients, ahighly significant association. Among these ten,four showed nail-fold lesions with microvasculitis,five arteritic skin ulceration, and two peripheralneuropathy. All systemic complications of rheu-matoid arthritis taken together showed a highlysignificant correlation with scleritis. Although allthe fourteen scleritics had positive Waaler-Rosetests and radiological erosions, these were notsignificantly more frequent than in the non-scleritics.Both groups of patients were receiving systemicsteroids in similar dosages.Four of the patients have observed that attacks of
inflammation in the eyes coincide with exacerbationsof arthritis but, in general, the eye symptoms weremild and one patient was unaware of the inflam-mation in the sclera. In one patient the scleritispreceded the onset of rheumatoid arthritis by4 years, and with the onset of joint symptoms theeye disease became worse.
Follow-up examinations were performed in tenpatients, at periods varying from 3 to 18 months.In two patients the eyes remained relatively un-changed. One of these had shown only thinning ofthe sclera with no active scleritis, but in the otherinflammatory changes and nodules persisted as
Table II Incidence of scleritis
Series Nos. studied Scleritis Sex
Male Female Active Inactive Male Female
Controls 27 115 0 0 0 0Rheumatoid arthritis 27 115 6 3 3 6Special referrals 3 2 5 0 3 2
Table III Age, duration, and sedimentation rate
Scleritis Present Absent t Significance
Meanage(yrs) 56-5 l10-7 580 11-2 0 48 P
Scleritis and rheumatoid arthritis 345
Table IV Scleritis related to other signs and symptoms
Rheumatoid nodules PresentAbsent
Systemic complications PresentAbsent
Waaler-Rose titre > 1 *32
346 Annals of the Rheumatic Diseases
latter, the eye inflammation was considerably reduced(Fig. 5). One year later only scleral thinning was observed(Fig. 6).
PATIENT 3, a 58-year-old woman with nodular sero-positive erosive rheumatoid arthritis, was receivingadrenocorticotrophic hormone and had normal eyes whenfirst seen, but 1 week later she simultaneously developedacute scleritis of both eyes (Fig. 7) and multiple micro-vasculitis of the fingers (Fig. 8, previous page).The corticotrophin therapy was increased and with
betamethasone eye drops the eye symptoms graduallyimproved and the vascular lesions healed. 6 monthslater there was still some active inflammation as well asscleral thinning (Fig. 9).
PATIENT 4, a 69-year-old man with nodular seropositiverheumatoid arthritis with evidence of microvasculitis ofthe fingers, was unaware of any eye symptoms. Heshowed both active inflammation and thinning in thesclera and also an area of scarring of the margin of thecornea typical of resolved scleral keratitis (Fig. 10).
Scleritis is a well-defined inflammatory change in thescleral coat of the eye. Nodular, diffuse, and necrot-izing types of scleritis have been described (Watsonand others, 1968), but in practice we have foundthese distinctions difficult as many cases show two oreven all three of these features. Episcleritis, on theother hand, is a more superficial inflammation deep tothe conjunctiva which may be difficult to distinguishfrom conjunctivitis. It may well not be true entityand we found no cases in the present series.
Scleritis often presents as a recurrent subacuteinflammation. Symptoms may be severe but areoccasionally minimal and the changes may then beeasily missed if the eyelids are not everted and thesclerae specifically examined. In the present studyscleritis was a not uncommon complication ofrheumatoid arthritis (over 6 per cent.) in contrastto its comparitive rarity in previous retrospectivesurveys.
Previous reports of scleritis