Scleritis Clinical Presentation

Updated: Nov 15, 2022
  • Author: Manolette R Roque, MD, MBA, FPAO; Chief Editor: Andrew A Dahl, MD, FACS  more...
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Presentation

History

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

Symptoms of scleritis can include pain, tearing or photophobia, ocular tenderness, and decreased visual acuity. Pain is the most common symptom for which patients seek medical assistance, and it is the best indicator of active inflammation. Pain results from direct stimulation and stretching of the nerve endings by 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; excessively tender
  • Only temporarily relieved by analgesics

The primary sign of scleritis is redness. Tearing or photophobia without mucopurulent discharge, usually mild or moderate, may occur in about 25% of patients with scleritis.

Unfortunately, many patients with scleritis first present to the emergency room or urgent care clinic, where a diagnosis of conjunctivitis is typically treated inappropriately with topical antibiotics. This practice generally leads to a significant delay in the initiation of anti-inflammatory therapy and a prolonged clinical course, and a more guarded prognosis.

Upon palpation, the patient may describe diffuse tenderness with possible radiation to other head parts.

Decreased visual acuity may be caused by the extension of scleritis to the adjacent structures, leading to reactive blepharitis, myositis, keratitis, uveitis, glaucoma, cataract, and fundus abnormalities.

Redness gradually increases over several days. It has a bluish-red, almost violaceous tinge, seen best when the examination is performed in natural light, not through the slit lamp. It may be localized in one sector or involve the whole sclera; most frequently, it is in the interpalpebral area. This discoloration does not blanch after topical applications of routine sympathomimetic dilating agents (Neo-Synephrine 2.5%).

Past medical and ocular histories may elucidate the following systemic diseases, trauma, drugs, or surgical procedures that might cause scleritis:

  • Connective-tissue or vasculitic diseases
  • Infectious diseases
  • Miscellaneous illnesses (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 might be significant, especially within a year before the onset of scleritis.

Past medical history is also necessary for discovering certain conditions (eg, gastric ulceration, diabetes, liver disease, anemia, 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
  • Hematologic and lymphatic
  • Pulmonary
  • Ear, nose, sinus, and throat
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Causes

Scleritis may occur in isolation (43%) or in association with several types of disorders (57%), as follows:

Autoimmune (48%)

Connective-tissue diseases and other inflammatory conditions include the following [6] :

Vasculitic diseases include the following [7] :

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) [8]

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Physical Examination

Examination of the head and extremities (e.g., nose, mouth, external ear, skin, joints) may reveal significant signs compatible with a particular underlying disease. The ocular examination includes a complete general eye examination focusing on the sclera that might detect and characterize the scleral disease.

Scleral examination

Daylight

Examination of the head and extremities (eg, nose, mouth, external ear, skin, joints) may reveal significant signs compatible with a particular underlying disease. The ocular examination includes a complete general eye examination focusing on the sclera that might detect and characterize the scleral disease.

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 occur, allowing the darkness of the uvea to be appreciated through the thin sclera.

A black, gray, or brown area surrounded by active scleral inflammation indicates a necrotizing process. If tissue necrosis progresses, the scleral area may become avascular, producing a central white sequestrum surrounded by a well-defined black or dark brown circle. The slough may be replaced gradually by granulation tissue, leaving the underlying uvea bare or covered by a thin layer of Tenon and 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 significantly affecting the deep episcleral network.

Red-free light

Red-free light is helpful in the following study areas:

  • Areas that have maximum vascular congestion
  • Sites that display new vascular channels
  • Completely avascular zones

General eye examination

Evaluate adjacent structures in scleritis at every follow-up visit since involvement is a significant reason for vision loss, as follows:

  • Extraocular muscles
  • Lids and orbit
  • Cornea
  • Uvea
  • Lens
  • Intraocular pressure (IOP)
  • Dilated fundus
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Complications

Potential complications include the following:

  • Keratopathy - Peripheral corneal thinning, acute stromal keratitis, sclerosing keratitis, interstitial keratitis, punctate keratopathy, or peripheral ulcerative keratitis
  • Uveitis - Anterior or posterior
  • Glaucoma
  • Cataract
  • Fundus abnormalities - Choroidal folds, subretinal mass, disk edema, macular edema, annular ciliochoroidal detachment, or serous retinal detachment
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