eMedicine Specialties > Ophthalmology > Sclera

Scleritis

Author: Maite Sainz de la Maza, MD, PhD, Associate Professor, Division of Ocular Immunology and Uveitis, Department of Ophthalmology, Hospital Clinico y Provincial, Barcelona, Spain
Contributor Information and Disclosures

Updated: Nov 8, 2007

Introduction

Background

Scleritis is a chronic, painful, and potentially blinding inflammatory disease that is characterized by edema and cellular infiltration of the scleral and episcleral tissues. Scleritis is commonly associated with systemic autoimmune disorders, including rheumatoid arthritis, systemic lupus erythematosus, relapsing polychondritis, spondyloarthropathies, Wegener granulomatosis, polyarteritis nodosa, and giant cell arteritis. Scleritis may be the initial or only presenting clinical manifestation of these potentially lethal disorders. The correct and rapid diagnosis and the appropriate systemic therapy can halt the relentless progression of both ocular and systemic processes, thus preventing destruction of the globe and prolonging survival.

Scleritis may be classified into anterior and posterior. Anterior scleritis can be diffuse, nodular, necrotizing with inflammation (necrotizing), and necrotizing without inflammation (scleromalacia perforans). The most common clinical forms are diffuse scleritis and nodular scleritis. Necrotizing scleritis with or without inflammation is much less frequent, more ominous, and frequently associated with systemic autoimmune disorders. Posterior scleritis is characterized by flattening of the posterior aspect of the globe, thickening of the posterior coats of the eye (choroid and sclera), and retrobulbar edema.

Pathophysiology

An autoimmune dysregulation in a genetically predisposed host is presumed to cause scleritis. Inciting factors may include infectious organisms, endogenous substances, or trauma. The inflammatory process may be caused by immune complex–related vascular damage (type III hypersensitivity) and subsequent chronic granulomatous response (type IV hypersensitivity).

The following interact as part of the activated immune network, which can lead to scleral destruction: immune complex vessel deposition in episcleral- and scleral-perforating capillary and postcapillary venules (inflammatory microangiopathy) and cell-mediated immune responses. The autoimmune nature of scleritis also is supported by the frequent association with systemic autoimmune disorders and by the favorable response to immunosuppressive therapy.

Frequency

United States

The exact incidence is uncertain, although scleritis is not common. The reported prevalence is skewed by the somewhat selected referrals of the reporting institutions. Approximately 2.6% of patients who were referred to the Immunology Service at the Massachusetts Eye and Ear Infirmary Hospital of Boston during an 11-year period had scleritis.

International

Approximately 0.08% of patients who were referred to the Department of Ophthalmology of Southern General Hospital and Victoria Infirmary of Glasgow during an 8-year period had scleritis.

Mortality/Morbidity

Patients with scleritis are at risk for ocular complications and systemic disease association.

• Ocular complications of scleritis, which cause vision loss and eye destruction, appear as a result of the extending scleral inflammation. Peripheral ulcerative keratitis (13-14%), uveitis (about 42%), glaucoma (12-13%), cataract (6-17%), and fundus abnormalities (about 6.4%). These complications are most common in necrotizing scleritis, the most destructive type of scleritis.
• Disease association may be found in about 57% of patients with scleritis. Up to 48% of patients with scleritis present with a known connective tissue or vasculitic disease. Some of these diseases are potentially lethal unless treated with prompt and aggressive therapy. Other patients may present with concomitant trauma, infection, or postsurgical inflammation. Systemic disease association is most common in cases of necrotizing scleritis. Scleritis may be the first manifestation of a potentially lethal systemic disease.

Sex

Women are more likely to have scleritis than men (1.6:1).

Age

Scleritis is most common in the fourth to sixth decades of life. The peak incidence is in the fifth decade.

Clinical

History

When interviewing the patient, investigate the following: the major complaint; a history of the present illness; the past history, including infection, injury, or surgery; and the review of systems.

• Symptoms can include pain, tearing or photophobia, tenderness, and decreased visual acuity. The primary sign is redness.
  • Pain is the most common symptom for which patients seek medical assistance, and it is the best indicator of active inflammation. Pain results from both direct stimulation and stretching of the nerve endings by the inflammation.
  • The following pain descriptions are characteristic of scleritis:
    • Severe, penetrating pain that radiates to the forehead, brow, jaw, or sinuses
    • Awakens the patient during the night
    • Exacerbated by touch; extremely tender
    • Only temporarily relieved by analgesics
  • Tearing or photophobia without mucopurulent discharge, which is usually mild or moderate, may occur in about 25% of patients with scleritis.
  • Upon palpation, the patient may describe tenderness that is diffuse with possible radiation to other parts of the head.
  • Decreased visual acuity may be caused by extension of scleritis to the adjacent structures, leading to keratitis, uveitis, glaucoma, cataract, and fundus abnormalities.
  • Redness gradually increases over several days. It has a bluish red tinge, which is seen best when the examination is performed in natural light. It may be localized in one sector or involve the whole sclera; most frequently, it is in the interpalpebral area. This discoloration does not blanche after topical applications of routine sympathomimetic dilating agents (Neo-Synephrine 2.5%).
• Past medical and ocular histories may elucidate systemic diseases, trauma, drugs, or surgical procedures that might cause scleritis:
  • Connective tissue or vasculitic diseases
  • Infectious diseases
  • Miscellaneous diseases (eg, atopy, rosacea, gout, chemical injuries)
  • Blunt or penetrating ocular trauma
  • Drugs, such as pamidronate (Aredia), alendronate (Fosamax), risedronate (Actonel), zoledronic acid (Zometa), and ibandronate (Boniva)
  • Past ocular surgical procedures, especially within a year prior to the onset of scleritis, might be significant.
• Past medical history is also important for discovering certain conditions (eg, gastric ulceration, diabetes, liver disease, renal disease, hypertension) that eventually might modify future therapy.
• Past and present therapies and responses to these interventions should be investigated.
• Because scleritis can be associated with systemic disorders, make a routine inquiry that covers various bodily systems, as follows:
  • Dermatologic (eg, skin, hair, nails)
  • Respiratory
  • Cardiac
  • Genitourinary
  • Rheumatologic
  • Gastrointestinal
  • Neurologic
  • Ear, nose, sinus, and throat

Physical

The head and extremities (eg, nose, mouth, external ear, skin, joints) examinations may reveal significant signs, which might be compatible with a particular underlying disease. An eye examination might detect and characterize scleral disease. Include scleral and general eye examinations.

• Scleral examination
  • Daylight
    • The sclera may appear diffuse, deep bluish red, or violaceous. After several attacks of scleral inflammation, areas of scleral thinning and translucency may appear, allowing the dark uvea to show.
    • A black, grey, or brown area that is surrounded by active scleral inflammation indicates a necrotizing process. If tissue necrosis progresses, the scleral area may become avascular, producing a white sequestrum in the center that is surrounded by a well-defined black or dark brown circle. The slough may be removed gradually by granulation tissue, leaving the underlying uvea bare or covered by a thin layer of conjunctiva.
  • Slit lamp light
    • In scleritis, maximum congestion is in the deep episcleral network with some congestion in the superficial episcleral network. The posterior and anterior edges of the slit lamp beam are displaced forward because of underlying scleral and episcleral edema.
    • In scleritis, topical application of 2.5% or 10% phenylephrine only blanches the superficial episcleral network without significant effect on the deep episcleral network.
  • Red-free light is helpful to the following study areas:
    • Areas that have maximum vascular congestion
    • Areas that display new vascular channels
    • Areas that are totally avascular
• General eye examination: Evaluate adjacent structures in scleritis at every follow-up visit, since involvement is an important reason for vision loss.
  • Extraocular muscles
  • Cornea
  • Uvea
  • Lens
  • Intraocular pressure
  • Dilated fundus

Causes

Scleritis may occur isolated (43%) or in association with several types of disorders (57%).

• Autoimmune (48%)
  • Connective tissue diseases and other inflammatory conditions include the following:
    • Rheumatoid arthritis
    • Systemic lupus erythematosus
    • Ankylosing spondylitis
    • Reactive arthritis
    • Psoriatic arthritis
    • Gouty arthritis
    • Inflammatory bowel diseases
    • Relapsing polychondritis
    • Polymyositis
    • Sjögren syndrome
    • Mixed connective tissue disease
    • Progressive systemic sclerosis
  • Vasculitic diseases include the following:
    • Polyarteritis nodosa
    • Allergic angiitis of Churg-Strauss syndrome
    • Wegener granulomatosis
    • Behçet disease
    • Giant cell arteritis
    • Cogan syndrome
• Infectious (7%) - Bacterial, fungal, viral, or parasitic
• Miscellaneous (2%) - Atopy; rosacea; or secondary to foreign bodies, chemical injuries, or drugs (eg, pamidronate, alendronate, risedronate, zoledronic acid, ibandronate)

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