Scleritis – Causes, Symptoms, Diagnosis, Treatment and Ongoing care

Basics

Description

  • Inflammation of the sclera, part of the eye’s outer coat
  • System(s) affected: Ocular

Epidemiology

  • Predominant age: Most frequently occurs in 4th and 6th decades; mean age for all types of scleritis is 52 years
  • Predominant sex: Female > Male (1.6:1)

Incidence

In 1 study, 0.08% of new patients presenting to an ophthalmology department were diagnosed with scleritis. 94% of patients have anterior scleritis; the remaining 6% have posterior.

Prevalence

Uncommon in US

Risk Factors

Individuals with autoimmune disorders are most at risk.

Etiology

  • ∼50% of cases of scleritis are associated with autoimmune diseases such as rheumatoid arthritis. In 15% of cases, scleritis is the presenting manifestation of a systemic disorder, appearing 1 or more months before other symptoms of the condition.
  • Scleritis has been reported in patients taking bisphosphonate therapy.

Commonly Associated Conditions

  • Sjögren syndrome
  • Ankylosing spondylitis
  • Systemic lupus erythematosus
  • Reactive arthritis
  • Relapsing polychondritis
  • Polyarteritis nodosa
  • Wegener granulomatosis
  • Sarcoidosis
  • Inflammatory bowel disease
  • Varicella zoster virus
  • Syphilis
  • Lyme disease
  • Herpes zoster
  • Tuberculosis

Diagnosis

History

  • Redness and inflammation of the sclera
  • Can be bilateral in 1/3 of cases
  • Pain ranging from mild discomfort to extreme localized tenderness. May be described as constant, deep, boring, or pulsating.
  • Pain may be referred to the eyebrow, temple, or jaw.
  • Photophobia and lacrimation may occur, but discharge is uncommon.
  • Posterior scleritis tends to be less symptomatic, as is the variant scleromalacia perforans, due to associated pain fiber necrosis.

Physical Exam

  • Photophobia and tearing on exam
  • Examine for pain with consensual constriction, which suggests uveal involvement.
  • Engorged purplish-red blood vessels
  • Inspect for breadth and degree of injection.
  • A bluish hue may suggest thinning of the sclera.
  • Scleral edema is often present.
  • Deeper scleral blood vessels appear darker, follow a radial pattern, and do not move when manipulated with a cotton swab.
  • Check visual acuity.
  • A complete physical examination, particularly of the skin, joints, heart, and lungs, may be done to evaluate for associated conditions.

Diagnostic Tests & Interpretation

Lab

  • Consider further testing if history, physical examination, complete blood count, serum chemistry, urinalysis, erythrocyte sedimentation rate, and/or C-reactive protein suggest a systemic cause.
  • Rheumatoid factor, anticyclic citrullinated peptide antibodies, antineutrophil cytoplasmic antibody, and antinuclear antibody may aid in the diagnosis.
  • FTA-ABS, rapid plasma reagin, and Lyme titers

Imaging

Initial approach

  • Further imaging studies, such as CXR and/or chest CT and sacroiliac joint films, may be useful if specific systemic illnesses are suspected.
  • Ultrasound or CT scan of the orbit may be useful to determine the extent and location (especially posterior) of scleritis and the presence of local associated disease.

Diagnostic Procedures/Surgery

Biopsy not routinely required unless diagnosis remains uncertain after above investigations

Pathological Findings

  • Adjacent inflammation may or may not be present.
  • The scleritis may be diffuse, nodular, or necrotizing (with or without associated inflammation).
  • If the posterior region of the globe is involved, adjacent swelling of orbital tissues may occur.

Differential Diagnosis

  • Conjunctivitis
  • Episcleritis
  • Pink eye
  • Iritis (anterior uveitis)
  • Trauma
  • Ocular rosacea
  • Herpes zoster

Treatment

Medication

First Line

  • Glucocorticoids are the mainstay of treatment, including topical, periocular, and systemic.
  • Contraindications: Documented hypersensitivity. Active peptic ulcer disease. Untreated concomitant viral or bacterial infection.
  • Precautions: Scleritis can progress to ocular perforation, which may be hastened with periocular steroid injection.

Second Line

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  • The more clinically benign subtypes—diffuse and nodular anterior scleritis—may respond to nonsteroidal anti-inflammatory drugs (NSAIDs) alone.
  • Necrotizing or posterior scleritis, on the other hand, may require cyclophosphamide in addition to systemic steroids. Other immunosuppressive and immunomodulating agents may be indicated depending on the systemic condition.

Additional Treatment

Issues for Referral

  • All patients with scleritis should be managed with the help of an ophthalmologist familiar with this condition.
  • Rheumatology referral for coexistent systemic diseases is helpful for long-term success.

Additional Therapies

Immunosuppressants used for autoimmune and collagen vascular disorders may be of help in active scleritis.

Surgery/Other Procedures

In rare cases, scleral biopsy may be indicated. Ocular perforation requires scleral grafting.

Ongoing Care

Follow-Up Recommendations

No restrictions

Patient Monitoring

The patient should be followed very closely in the active stage of inflammation to assess the effectiveness of therapy. Medication use mandates close surveillance for adverse effects.

Diet

No special diet

Prognosis

  • Scleritis is indolent, chronic, and often progressive.
  • Recurrent bouts of inflammation may occur.

Complications

  • Increased intraocular pressure
  • Cataract and glaucoma can result from treatment.
  • Visual loss due to posterior extension or adjacent structural involvement
  • Ocular perforation can occur in severe stages.

Additional Reading

1. Fong LP, Sainz de la Maza M, Rice BA, et al. Immunopathology of scleritis. Ophthalmology. 1991;98:472–9.

2. Fraunfelder FW, Fraunfelder FT. Bisphosphonates and ocular inflammation. N Engl J Med. 2003;348:1187–8.

3. Smith JR, Mackensen F, Rosenbaum JT. Therapy Insight:scleritis and its relationship to systemic autoimmune disease. Nature Clinical Practice Rheumatology.2007;3:219–226.

Codes

ICD9

379.00 Scleritis, unspecified

Snomed

78370002 Scleritis (disorder)

Clinical Pearls

  • Scleritis involves a deeper layer of the eye than episcleritis and, therefore, can be a vision-threatening condition, more common in midlife. Episcleritis is less painful and occurs in younger patients.
  • Uveitis, by definition, involves some combination of the iris, ciliary body, and choroid membrane and thus, generally is associated with pain, with consensual constriction of the pupil. Scleritis can spread to involve these structures, but does not always. Both can be associated with underlying inflammatory diseases.

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