Scleritis Clinical Presentation

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 of scleritis 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

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

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^[1] :
    • 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^[2] :
    • 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)^[3]