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Scleritis
Authors

Amy Lagina1; Kamleshun Ramphul2.

Affiliations
1 University of Michigan
2 Shanghai Jiao Tong University

Last Update: June 27, 2022.

Continuing Education Activity

Scleritis is a severe ocular inflammatory condition affecting the outer covering of the eye. Scleritis has a high
association with systemic disease. This activity reviews the evaluation and treatment of scleritis and highlights the
role of the interprofessional team in caring for patients with this condition.

Objectives:

Describe the types of scleritis.

Explain the cause of scleritis.

Articulate the proper treatment protocols for each type of scleritis.

Employ interprofessional team strategies for improving care coordination and communication in the
management of scleritis.

Access free multiple choice questions on this topic.

Introduction
Scleritis is a severe ocular inflammatory condition affecting the sclera, the outer covering of the eye. It can be
categorized as anterior with diffuse, nodular, or necrotizing subtypes and posterior with diffuse or nodular subtypes.
Scleritis can be visually significant, depending on the severity and presentation and any associated systemic
conditions.[1] The presentation can be unilateral or bilateral.

Etiology
Scleritis can be idiopathic or caused by infectious or noninfectious conditions. Additionally, associations with
malignancy, autoimmune diseases, and surgically induced or medication side effects are causative factors. Viruses,
bacteria, fungi, and parasites can cause infectious scleritis and are reported to occur in 4% to 10% of all cases.[2]
Choroidal melanomas and conjunctival tumors can create ocular inflammation that can mimic the signs and symptoms
of scleritis. 50% of patients with scleritis will have an autoimmune condition, sometimes undiagnosed at the time of
presentation. Rheumatoid arthritis and systemic vasculitic conditions are most commonly associated with scleritis. [3]

Surgically induced scleritis has been associated with pterygium removal and scleral buckle procedures. Medications
used to treat osteoporosis such as bisphosphonates have been found to cause scleritis; however, reports of this
occurrence are rare.[2]

Epidemiology
Limited, population-based studies have reported 10,500 cases of scleritis in the United States per year or an estimated
four to six cases per 100,000 persons.[3]

It affects patients in middle age, commonly between 47 to 60 years.[2] Scleritis is more common in women, with a
60% to 74% predominance.[2] Limited information is known about the incidence of children besides case reports.

Pathophysiology
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The exact pathophysiology of scleritis is still under investigation. The anatomical structure of the sclera includes an
extracellular matrix of collagen, elastin, and proteoglycans that closely resemble the components of joints, causing it
to be susceptible to inflammatory conditions such as rheumatoid arthritis.[1] Additionally, the sclera is mostly
avascular and requires the transport of nutrients and the removal of cellular wastes on the surrounding episcleral and
choroidal vascular systems.

Histopathology
The disease's histopathological features are similar to those seen in vasculitic conditions. Scleral biopsies from
patients with severe scleritis or necrotizing scleritis demonstrated vascular occlusions and infiltration, necrosis, and
evidence of macrophages and T cells.[3]

History and Physical

The presentation can vary depending on the location and subtype of scleritis and can be unilateral or bilateral.[4] [5]

Anterior

Anterior from the rectus muscle's limbus-insertion

Occurs in 98% of cases

Mild to moderate pain and tenderness; worse at night

Pain with eye movement

A blue-violet hue of deep vessels; best seen in daylight or natural illumination

Photophobia, tearing, and possibly decreased vision

No vessel blanching with the installation of 2.5% topical phenylephrine

Diffuse

Most common (45% to 61%)

Extensive scleral edema; congestion of deep and superficial vessels

Can be localized or encompass the entire anterior sclera

Nodular

Multiple, well-defined, and non-moveable nodules

Scleral edema and congestion of vessels

Usually more localized

Necrotizing

With inflammation – intense congestion of vessels

Severe pain

Most severe form with the worst prognosis

Highest association with a systemic disease

Scleral thinning and exposure of choroid possible

Inflammation can spread to other ocular tissues such as the cornea, ciliary body, or trabecular meshwork

Necrotizing Without inflammation (Scleromalacia perforans)

Severe scleral thinning- with visible choroid

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Can be asymptomatic or can have reduced acuity resulting from corneal astigmatism caused by the scleral
thinning

Posterior

Behind the insertion of the rectus muscles

Occurs in 2% of cases

Can occur in conjunction with anterior scleritis

May present with decreased vision, with or without ocular pain

Retinal detachments, optic nerve swelling, and cotton wools spots can be seen on dilated examination

Diffuse or nodular subtypes can be differentiated with B-scan, CT), or MRI

B-scan ultrasound displays thickening of the posterior sclera (greater than 2millimeters); retrobulbar thickening
and fluid surrounding the optic nerve create a characteristic “T” sign

Diffuse - Diffuse thickening of sclera and choroid

Nodular- Scleral nodules seen on B-scan

Evaluation
The diagnosis is based on the clinical presentation and ocular exam with a detailed history and review of systems,
targeted laboratory tests, and imaging studies.[2]

Common Lab Tests

Antineutrophil cytoplasmic antibodies (ANCAs) – specific for granulomatosis with polyangiitis (Wegener
granulomatosis)

Antinuclear Antibodies (ANA) – suggestive of systemic lupus erythematosus and other autoimmune conditions

Complete blood count(CBC) with differential – identify infectious or inflammatory processes

C-reactive protein – elevated in inflammatory conditions

ESR – suggestive of giant cell arteritis, inflammatory or infectious processes

HLA-B27 - more useful in cases of uveitis; rare association with scleritis

Lyme serology – rule out Lyme Disease, presents with recurrent diffuse anterior scleritis

Rheumatoid factor – suspect for rheumatoid arthritis

Angiotensin-converting enzyme (ACE) - suggestive of sarcoidosis

Serum lysozyme – suggestive of sarcoidosis

Rapid plasma reagin (RPR) – suggestive for syphilis

Fluorescent treponemal antibody absorption test (FTA-ABS) – suggestive for syphilis

Imaging

Chest X-Ray – rule out sarcoidosis

B-scan – assess for posterior scleritis

MRI – can be used if B-scan is non-conclusive

CT – can be used if MRI is non-indicated (posterior scleritis)

Ultrasound biomicroscopy (UBM) – can be used to differentiate episcleritis from scleritis

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Optical coherence tomography (OCT) – can display thickened sclera on anterior segment OCT and thickened
choroidal tissue

Anterior segment fluorescein and indocyanine green angiography (ICGA) – useful in visualizing necrosis

Treatment / Management
The treatment and management of scleritis are designed to determine any causative factor, manage ocular
inflammation, control ocular pain and symptoms, prevent sequela, and reduce recurrences.[2][4] [5]

Anterior Infectious

Treatment tailored to the infectious cause

Anterior Noninfectious

First-line treatments

Topical corticosteroid eyedrops

Can be used in mild cases; some report very limited success

Prednisolone acetate 1.0% or difluprednate 0.05% four times per day are options

Oral NSAIDs

Can start with one agent and switch to another agent if not effective

Indomethacin, commonly used, 50 mg, 3 times per day

Ibuprofen 600 mg, 3 times per day

Naproxen 500 mg, 2 times per day

Second-line treatments

Oral corticosteroids

Prescribed when oral NSAID treatment fails

Oral prednisone 1 mg/kg/day; 60 to 80 mg per day

Dose continued until one month after the resolution of scleritis, then tapered accordingly

Subconjunctival corticosteroid injection

Can be used when steroids are contraindicated

Controversial use with previous studies of scleral necrosis/perforation

More recent reports have indicated improved safety and efficacy of injections

Third line treatments

Immunosuppressive agents (steroid-sparing)

Reserved for more severe cases of scleritis, with oral steroid treatment failure or concern for side effects
with long-term oral steroid use

Methotrexate is the most commonly prescribed agent; starting dose of 0.15 mg/kg per week in
combination with folic acid supplements

Other agents include azathioprine, mycophenolate, and cyclophosphamide

Fourth line treatments

Biologics

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Ongoing research with the use of biologics such as infliximab and rituximab has been shown to have a
positive treatment response

Posterior

Requiring intense and immediate treatment

First-line treatments

Oral NSAIDs

Commonly used: indomethacin, naproxen, ibuprofen

Second-line treatments

Oral corticosteroids

- Prescribed when oral NSAID treatment fails

- Oral prednisone of 1 mg/kg/day; 60 to 80 mg per day

Third line treatments

Immunosuppressive/antimetabolites agents

Reserved for severe cases of scleritis and with high dose oral steroid treatment failure after 1 month

Commonly prescribed agents include methotrexate, azathioprine, and mycophenolate

Fourth line treatments

Biologics

Infliximab and rituximab

Differential Diagnosis
The main differential diagnosis of scleritis is episcleritis. Episcleritis is defined as inflammation of the superficial
episcleral tissues and blood vessels. Patients can present with mild pain, redness, foreign body sensation, and tearing.
Upon installation of 2.5% topical phenylephrine in the affected eye, the blood vessels will blanch with episcleritis but
will not blanch with scleritis.[2] Episcleritis is usually idiopathic and resolves without treatment.

Prognosis
Visual prognosis is relatively good for patients with mild or moderate scleritis that respond well to the appropriate
medical treatment and management of any underlying systemic condition.[6] Necrotizing and posterior scleritis cases
pose a higher risk of visual loss to the extent of the inflammation and involvement of other ocular structures.

Complications
Sequela resulting from scleritis can vary depending on the severity of presentation and any associated autoimmune
conditions and can include decreased vision, cataracts, increased intraocular pressure, scleral thinning or melting, and
corneal thinning. [6]

Deterrence and Patient Education

it is important to discuss the risk of associated systemic conditions with patients with scleritis. Emphasis on proper
pain management and control of inflammation is essential for visual recovery and improved prognosis.

Pearls and Other Issues

At least half of scleritis cases can be linked to an autoimmune condition. Order specific laboratory tests and imaging
according to patient demographics, detailed history, and physical examination. Investigation for an associated
autoimmune condition must be completed at first presentation of scleritis if the patient is unaware of any systemic
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health problems. Instill one drop of 2.5% topical phenylephrine to observe any vessel blanching and to distinguish
between episcleritis versus scleritis.[2]

Enhancing Healthcare Team Outcomes

Essential communication with internal medicine, eye care providers, and medical specialists (rheumatology) is
required for proper medical management and to improve patient outcomes.

Review Questions

Access free multiple choice questions on this topic.

Comment on this article.

References
1. Okhravi N, Odufuwa B, McCluskey P, Lightman S. Scleritis. Surv Ophthalmol. 2005 Jul-Aug;50(4):351-63.
[PubMed: 15967190]
2. Daniel Diaz J, Sobol EK, Gritz DC. Treatment and management of scleral disorders. Surv Ophthalmol. 2016 Nov-
Dec;61(6):702-717. [PubMed: 27318032]
3. Artifoni M, Rothschild PR, Brézin A, Guillevin L, Puéchal X. Ocular inflammatory diseases associated with
rheumatoid arthritis. Nat Rev Rheumatol. 2014 Feb;10(2):108-16. [PubMed: 24323074]
4. Sims J. Scleritis: presentations, disease associations and management. Postgrad Med J. 2012 Dec;88(1046):713-8.
[PubMed: 22977282]
5. Oray M, Meese H, Foster CS. Diagnosis and management of non-infectious immune-mediated scleritis: current
status and future prospects. Expert Rev Clin Immunol. 2016 Aug;12(8):827-37. [PubMed: 27055583]
6. Wieringa WG, Wieringa JE, ten Dam-van Loon NH, Los LI. Visual outcome, treatment results, and prognostic
factors in patients with scleritis. Ophthalmology. 2013 Feb;120(2):379-86. [PubMed: 23177360]
Disclosure: Amy Lagina declares no relevant financial relationships with ineligible companies.

Disclosure: Kamleshun Ramphul declares no relevant financial relationships with ineligible companies.

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