Therapy Insight: Scleritis And Its Relationship To Systemic Autoimmune Disease

Justine R Smith; Friederike Mackensen; James T Rosenbaum

Disclosures

Nat Clin Pract Rheumatol. 2007;3(4):219-226. 

In This Article

Summary and Introduction

The term scleritis describes a chronic inflammation that involves the outermost coat and skeleton of the eye. Disease can be isolated to the eye, but in up to half of affected individuals it occurs in the context of an immune-mediated systemic inflammatory condition, such as rheumatoid arthritis or Wegener's granulomatosis. Although uncommon, scleritis is often extremely painful, can lead to vision-threatening complications (and involvement of other ocular tissues), and is considered to confer an increased risk of mortality in patients with rheumatoid arthritis. Pathogenic mechanisms in scleritis are poorly understood, but enzymatic degradation of collagen fibrils by resident cells and infiltrating leukocytes seems to be a key feature. Several forms of inflammation can be distinguished histologically; interestingly, although the disease typically presents with engorgement of scleral vessels, vasculitis is not universally present at the microscopic level. Although some patients with scleritis respond well to treatment with NSAIDs, aggressive systemic therapy is often required to obtain a favorable outcome, particularly when systemic disease coexists. The mainstay of treatment is oral prednisone, but this agent is usually combined with a steroid-sparing immunosuppressive drug. New therapies presently under investigation for scleritis include local corticosteroid injections and various biologic agents.

The sclera is the white external tunic that envelops the eye from the limbus or corneoscleral junction (anterior), and to the cribriform plate of the optic nerve head (posterior) (Figure 1). In a healthy eye the scleral vessels are not prominent, but in patients with scleritis this vasculature is engorged. If the anterior aspect of the eye is involved (which is the most common situation) the external surface of the eye becomes red and tender, which is the result of inflammation. Sometimes the redness is widely diffuse, and in other situations it covers a pie-shaped area. Scleritis can occur as a condition confined to the eye or it might be associated with a systemic, immune-mediated disease; uncommonly, scleritis might be caused by an infection or drug reaction, or by an occult (i.e. hidden) tumor, or complications following surgery. This form of ocular inflammation is usually a chronic disease that can be extremely painful and can lead to vision-threatening complications that include uveitis, cataract and glaucoma, as well as various retinal pathologies (Box 1 contains definitions of ophthalmologic terms). In this paper, we will review current knowledge of the epidemiology, clinical presentation, course, etiology and treatment of this disease.

Diagrammatic representation of the eye shown in cross section. The sclera forms the outermost coat of the eye. The episclera is a thin layer between the sclera and the conjunctiva.

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