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Kerato-Conjunctivitis Sicca and Rheumatoid Arthritis

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Kerato-Conjunctivitis Sicca and Rheumatoid Arthritis Ann Rheum Dis: first published as 10.1136/ard.15.1.21 on 1 March 1956. Downloaded from Ann. rheum. Dis. (1956), 15, 21. KERATO-CONJUNCTIVITIS SICCA AND RHEUMATOID ARTHRITIS BY MALCOLM THOMPSON* AND STELLA EADIE From the Rheumatic Unit, Northern General Hospital, Edinburgh, and the Edinburgh Royal Infirmary (RECEIVED FOR PUBLICATION OCTOBER 13, 1955) Although the ophthalmic lesions which constitute arthritis is an integral part of the symptom complex. kerato-conjunctivitis sicca have long been known, It is my feeling that the arthritis is an incidental the nature of the condition has remained obscure. finding." Ellman, Weber, and Goodier (1951) Duke-Elder (1930) reviewed those rare cases in remarked that: "rheumatoid arthritis is fairly which lesions of the lacrimal secretary nerves or of common among women, and that very few patients the gland parenchyma (including congenital aplasia, with rheumatoid arthritis ever manifest any symp- surgical extirpation, infiltrations by sarcoid, leu- toms resembling Sjogren's disease". They recorded kaemic or malignant tissue) resulted in deficient the case history and autopsy findings in a patient lacrimal secretion and the subsequent development who did suffer from rheumatoid arthritis, but in of corneal and conjunctival lesions of the sicca type. whom the symptoms of xerostoma, salivary gland These rare cases, however, accounted for only a enlargement, and ocular inflammation preceded the small proportion of all the patients suffering from development of the arthritis by several years. no However, Reader, Whyte, and Elmes (1951) con- kerato-conjunctivitis sicca, and in the majority copyright. local ophthalmic cause for deficient lacrimation and firmed the opinion of Holm (1949) that many sicca lesions could be found. patients who suffered from rheumatoid arthritis also Sjogren (1933) described in detail his observations had a deficiency of lacrimal secretion, as estimated on eighteen patients with idiopathic kerato-con- by Schirmer's test. Other workers have been more junctivitis sicca and drew attention to its frequent inclined to consider kerato-conjunctivitis sicca and association with such clinical features as poly- its allied pathology as part of the wider concept of arthritis, xerostoma, and salivary gland enlargement, rheumatoid disease; this view was expressed by occurring especially in middle-aged women. To Littler (1951), Gurling (1953), and Morgan (1954). http://ard.bmj.com/ this triad the name Sjogren's syndrome was given, During the past 3 years, we have treated eighteen but the variations and increasing complexity of the patients with severe or moderate kerato-conjunc- condition soon became apparent. Holm (1949) tivitis sicca, fourteen of whom (77 7 per cent.) had reviewing 440 cases of rheumatoid disease stated rheumatoid arthritis. An additional sixteen patients that "The conception of kerato-conjunctivitis sicca with milder symptoms and signs, consistent with a as a disease sui genesis (Sjogren) is fundamentally condition of conjunctivitis sicca, and also suffering incorrect": but he considered that rheumatoid from rheumatoid arthritis, have also been examined on September 30, 2021 by guest. Protected arthritis was of importance as an aetiological factor. and treated. The high percentage of cases asso- The term Sjbgren's syndrome continued to be ciated with polyarthritis is due to the fact that most applied to any condition in which the classical of the cases were identified at a rheumatic unit. triad of kerato-conjunctivitis sicca, polyarthritis, The ophthalmic features, associated lesions, and and salivary gland enlargement was present, as complications noted in these patients have some well as those in which arthritis was absent (approxi- points of interest, and are recorded together with mately 40 per cent. of all cases of kerato-conjunc- some observations on the results of treatment of the tivitis sicca). Writers, however, remained cautious ocular lesions. of associating the condition with rheumatoid arth- Incidence ritis. Coverdale (1948) considered that "Sj6gren's Kerato-conjunctivitis sicca is not the rarity it was syndrome" indicated a general constitutional or once considered to be. It is, indeed, the commonest systemic disturbance of unknown origin, while ocular complication of rheumatoid arthritis. In a Henderson (1950) stated: "I do not feel that poly- recent study of 210 former in-patients of the Rheu- * Present address: Royal Victoria Infirmary, Newcastle-on-Tyne. matic Diseases Unit, Northern General Hospital, 21 Ann Rheum Dis: first published as 10.1136/ard.15.1.21 on 1 March 1956. Downloaded from 22 ANNALS OF THE RHEUMATIC DISEASES Edinburgh, we found evidence of lesions of the sicca symptoms of kerato-conjunctivitis sicca, the more -type in thirty patients, an incidence of 14 3 per cent. serious risks to vision have not been fully appreci- In comparison with this, there were only seven ated. In the present series, corneal ulceration was patients (3 3 per cent.) who showed signs of active noted in three cases. In two instances the ulcers or old uveitis. No cases of episcleritis, sclero- were of the small marginal type which healed without malacia perforans, or superficial punctate keratitis incident. The third patient, however, suffered from were identified in this series. These findings accord bilateral corneal ulceration, and was initially referred with those of Holm (1949), who diagnosed kerato- to hospital when perforation of a central corneal conjunctivitis sicca in 13 -4 per cent. of 440 rheuma- ulcer necessitated enucleation of the eye. One toid patients, and of Rosenberg (1949) who reported patient also had a small pannus involving the uveitis in 3 to 5 per cent. of cases. infero-lateral quadrant of the cornea. Of the thirty patients with kerato-conjunctivitis sicca in our series, all of whom had had ophthalmic Pathology.-Sjogren (1933) also described the symptoms, six had gross corneal and conjunctival histological features in the lacrimal and salivary lesions, eight had conjunctival lesions of moderate glands and in the conjunctiva. In the lacrimal severity and had only slight corneal changes in glands the changes consisted of round cell infiltra- addition, and sixteen had conjunctival lesions only, tions, atrophy and destruction of the acini, and and could be considered to be examples of con- replacement fibrosis in the later stages (Fig. 1). junctivitis sicca. Sex Ratio Sjogren (1933) stated that the syndrome occurred most commonly in middle-aged women. Holm (1949) found that female cases predominated, but that in his rheumatoid patients the sex incidence of kerato-conjunctivitis sicca paralleled the sex dis- tribution of the arthritis, being approximately 2 5 copyright. females to 1 male. Of the thirty patients in our series, 26 were females, making a ratio of 6- 5 females to one male. The sex ratio of the 210 patients studied was only 2-7 females to one male. Description classical symptoms Ophthalmic Features.-The http://ard.bmj.com/ and signs of kerato-conjunctivitis sicca are those of conjunctivitis associated with filamentary keratitis and reduction oflacrimal secretion, and the diagnosis can be substantiated by Schirmer's test for lacrimal secretion and the staining reaction with 1 per cent. aqueous Rose-Bengal solution. In the present series, several ophthalmic features of interest were noted. The ocular symptoms ran on September 30, 2021 by guest. Protected a course of remissions and exacerbations, but symptoms were rarely completely absent in severe cases of kerato-conjunctivitis sicca. The waxing and waning of the ophthalmic symptoms in those who also had rheumatoid arthritis often patients Fig. 1.-Lacrimal gland showing atrophy of acini and extensive occurred independently of any changes in the round cell infiltration ( x 80). -arthritic condition. Furthermore, the severity of the ocular changes in such patients was not propor- The lesions in the lacrimal glands were considered tional to the severity of the arthritis, some of the to be mainly responsible for the dryness of the eyes most marked examples of kerato-conjunctivitis and the subsequent epithelial changes, but primary sicca being encountered in patients with mild or degenerative changes in the conjunctival epithelium quiescent rheumatoid arthritis. could also contribute to the developed appearances Although ocular discomfort, photophobia, and in kerato-conjunctivitis sicca. The histological mild impairment of vision are well recognized as changes in the lacrimal glands are similar to those Ann Rheum Dis: first published as 10.1136/ard.15.1.21 on 1 March 1956. Downloaded from KERATO-CONJUNCTIVITIS SICCA AND RHEUMATOID ARTHRITIS 23 noted in the salivary glands, but one important evidence of arthritis comprised one man and three difference is that while secretary ducts may be women. In only two instances had ocular symptoms blocked in the salivary glands, this does not occur been noticed before the age of 40 years. The nail in the lacrimal glands (Morgan, 1954). This is in changes and otitis externa noted in the present keeping with the clinical observation that enlarge- series are worthy of special mention. ment of the salivary glands is not uncommon in The nail lesions, seen in both fingers and toes, Sjogren's syndrome, but that enlargement of the consisted of dry, longitudinally fissured nails lacrimal glands is never seen. (onychorrhexis), stained brownish-yellow. Those most severely involved were rough and irregularly Associated Clinical Features.-The principal
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