Clinical manifestations and diagnosis of scleritis - Arthritis & Scleritis Diagnosis and Tests

Clinical manifestations and diagnosis of scleritis

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Last literature review version 16.3: October 2008 | This topic last updated: June 2, 2008 (More)

INTRODUCTION — Scleral inflammatory disease consists of episcleritis and scleritis. Episcleritis is typically self-limited or quickly responsive to topical therapies. In contrast, scleritis is a painful, destructive, and potentially blinding disorder that may also involve the cornea, adjacent episclera, and underlying uveal tract. Scleritis has a striking, highly symptomatic clinical presentation (show figure 1).

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 Wegener's granulomatosis [1]. 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 [2].

This topic will review the clinical manifestations and diagnosis of scleritis. The treatment of scleritis and issues related to other inflammatory disorders of the eye are presented separately. (See "Treatment of scleritis" and see "Episcleritis" and see "Uveitis: Etiology; clinical manifestations; and diagnosis" and see "Ocular manifestations of rheumatoid arthritis" and see "Retinal vasculitis associated with systemic disorders and infections").

ANATOMY — The sclera lies beneath the conjunctiva and episclera but above the choroid (show figure 2). 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 (show picture 1). (See "Posterior scleritis" below).

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