Elsevier

Ophthalmology

Volume 106, Issue 7, 1 July 1999, Pages 1328-1333
Ophthalmology

Scleritis: A clinicopathologic study of 55 cases

Presented in part as a poster at the American Academy of Ophthalmology annual meeting, San Francisco, California, October 1997.
https://doi.org/10.1016/S0161-6420(99)00719-8Get rights and content

Abstract

Objective

By a clinicopathologic study, to evaluate the histopathologic features associated with various causes of scleritis.

Design

Retrospective observational case series.

Participants

Enucleated globes or biopsy specimens obtained from 55 cases of clinically diagnosed necrotizing scleritis.

Methods

On the basis of their histologic appearance, these cases were divided into four morphologic groups: (1) zonal necrotizing granulomatous scleral inflammation; (2) nonzonal diffuse scleral inflammation, with or without granulomatous process; (3) necrotizing inflammation with microabscesses, with or without evidence of micro-organisms in the section studied; and (4) sarcoidal granulomatous inflammation. The clinical charts were reviewed for the presence of any associated disease.

Results

There were 14 (25.4%) cases in the first group; 12 had clinical evidence of systemic autoimmune diseases, including 8 cases of rheumatoid arthritis and 1 each of polychondritis, Goodpasture syndrome, Wegener granulomatosis, and collagen vascular disease; of the remaining 2 cases, 1 patient had a history of herpes zoster ophthalmicus, and the other had no history of any systemic autoimmune or infectious disease. None of the 19 (34.5%) patients characteristic of group 2 had any history of systemic autoimmune or infectious disease. Eleven of the 21 (38.2%) patients in group 3 had infections, including Pseudomonas spp., gram-positive cocci, Haemophilus spp., Actinomyces spp., and fungi; in the 10 remaining cases, no micro-organisms could be detected. The one case in group 4 was diagnosed as sarcoidosis.

Conclusions

On the basis of their histologic features, rheumatoid scleritis and related systemic autoimmune-mediated necrotizing scleral inflammations could be differentiated from either idiopathic or infectious scleritis; however, the histologic features of rheumatoid scleritis were similar to those of necrotizing scleritis associated with other systemic autoimmune diseases.

Section snippets

Materials and methods

A total of 55 cases of scleritis were included in this study (Table 1). Thirty-eight cases were obtained from the files of the A. Ray Irvine Ocular Pathology Laboratory of the Doheny Eye Institute; 3 of these had been brought back from the Verhoeff Society Meeting (1 case was presented by Peter R. Egbert, MD, 1 by Robert A. Levine, MD, and 1 by Alan M. Roth, MD). The remaining 17 cases were obtained from the Armed Forces Institute of Pathology (AFIP). Eleven of the Doheny Eye Institute cases

Results

The results are summarized in Table 1. The specimens submitted for histopathologic examination consisted of 46 enucleated eyes and 9 scleral biopsy specimens. All nine biopsy specimens were obtained from the anterior sclera of patients with a clinical diagnosis of anterior scleritis; five of these patients belonged to group 2 and four to group 3. The patients ranged in age from 22 to 95 years (mean = 61.6 ± 19.7 years, median = 68 years). Twenty-eight patients were women; 25 were men. In two

Discussion

The systemic immune-mediated disease most frequently associated with scleritis is rheumatoid arthritis.1, 5 In the previous clinicopathologic study of necrotizing scleritis,6 rheumatoid scleritis could be differentiated histologically from idiopathic scleritis. On the basis of such histologic criteria, 14 (25.4%) of the 55 cases in the present study showed histologic features of zonal necrotizing granulomatous inflammation (Fig 1). Twelve of these 14 cases showed clinical evidence of systemic

Acknowledgements

The authors thank John A. Irvine, MD, for providing the clinical photograph of culture-proven Pseudomonas scleritis.

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