Clinical manifestations and diagnosis of scleritis
- Reza Dana, MD, MPH, MSc
Reza Dana, MD, MPH, MSc
- Claes Dohlman Professor of Ophthalmology
- Harvard Medical School
Scleritis is a painful, destructive, and potentially blinding inflammatory disorder of the sclera that may also involve the cornea, adjacent episclera, and underlying uveal tract. Scleritis has a striking, highly symptomatic clinical presentation (picture 1) (see 'Clinical features' below). By contrast, episcleritis is typically self-limited or quickly responsive to topical therapies. (See "Episcleritis".)
Scleritis sometimes occurs in an isolated fashion, without evidence of inflammation in other organs. However, in up to 50 percent of patients, scleritis is associated with an underlying systemic illness such as rheumatoid arthritis or granulomatosis with polyangiitis (Wegener's)  (see 'Systemic disease associations' below). Two-thirds of patients with scleritis require high-dose glucocorticoids or the combination of high-dose glucocorticoids and another immunosuppressive agent to achieve disease control . (See "Treatment of scleritis".)
This topic will review the clinical manifestations and diagnosis of scleritis. The treatment of scleritis, episcleritis, and issues related to other inflammatory disorders of the eye are presented separately. (See "Treatment of scleritis" and "Episcleritis" and "Uveitis: Etiology, clinical manifestations, and diagnosis" and "Ocular manifestations of rheumatoid arthritis" and "Retinal vasculitis associated with systemic disorders and infections".)
The sclera lies beneath the conjunctiva and episclera but above the choroid (figure 1). The opaque scleral tissue is composed of collagen fibrils arranged in a precise, interlacing manner that enhances rigidity and stability. Although the sclera itself is avascular, the tissue derives its metabolic requirements by diffusion from the episclera and choroid, both of which are highly vascularized.
The sclera comprises 90 percent of the outer coat of the eye. Scleral tissue begins at the limbus (the outer edge of the cornea) and terminates posteriorly at the optic canal. At the posterior pole of the eye, the sclera fuses with the dura mater and arachnoid sheaths of the optic nerve. These anatomic relationships explain why optic nerve edema and visual compromise are common complications of posterior scleritis (picture 2). (See 'Clinical features' below.)To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
- Okhravi N, Odufuwa B, McCluskey P, Lightman S. Scleritis. Surv Ophthalmol 2005; 50:351.
- Jabs DA, Mudun A, Dunn JP, Marsh MJ. Episcleritis and scleritis: clinical features and treatment results. Am J Ophthalmol 2000; 130:469.
- McCluskey PJ, Watson PG, Lightman S, et al. Posterior scleritis: clinical features, systemic associations, and outcome in a large series of patients. Ophthalmology 1999; 106:2380.
- Horo S, Sudharshan S, Biswas J. Recurrent posterior scleritis--report of a case. Ocul Immunol Inflamm 2006; 14:51.
- Fong LP, Sainz de la Maza M, Rice BA, et al. Immunopathology of scleritis. Ophthalmology 1991; 98:472.
- Scott DG, Bacon PA, Tribe CR. Systemic rheumatoid vasculitis: a clinical and laboratory study of 50 cases. Medicine (Baltimore) 1981; 60:288.
- Hoang LT, Lim LL, Vaillant B, et al. Antineutrophil cytoplasmic antibody-associated active scleritis. Arch Ophthalmol 2008; 126:651.
- Sainz de la Maza M, Foster CS. Necrotizing scleritis after ocular surgery. A clinicopathologic study. Ophthalmology 1991; 98:1720.
- Díaz-Valle D, Benítez del Castillo JM, Castillo A, et al. Immunologic and clinical evaluation of postsurgical necrotizing sclerocorneal ulceration. Cornea 1998; 17:371.
- Scott JA, Clearkin LG. Surgically induced diffuse scleritis following cataract surgery. Eye (Lond) 1994; 8 ( Pt 3):292.
- Squirrell DM, Winfield J, Amos RS. Peripheral ulcerative keratitis 'corneal melt' and rheumatoid arthritis: a case series. Rheumatology (Oxford) 1999; 38:1245.
- Pakrou N, Selva D, Leibovitch I. Wegener's granulomatosis: ophthalmic manifestations and management. Semin Arthritis Rheum 2006; 35:284.
- Lin P, Bhullar SS, Tessler HH, Goldstein DA. Immunologic markers as potential predictors of systemic autoimmune disease in patients with idiopathic scleritis. Am J Ophthalmol 2008; 145:463.
- SCLERITIS SUBTYPES
- Anterior scleritis
- Posterior scleritis
- Systemic disease associations
- CLINICAL FEATURES
- Ocular symptoms
- Ocular examination
- Involvement of other ocular structures
- Features of systemic disease
- Diagnosis of scleritis
- Diagnostic evaluation for systemic disease
- DIFFERENTIAL DIAGNOSIS