Scleritis


Article Author:
Amy Lagina


Article Editor:
Kamleshun Ramphul


Editors In Chief:
Kranthi Sitammagari
Mayank Singhal


Managing Editors:
Avais Raja
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Khalid Alsayouri
Trevor Nezwek
Radia Jamil
Patrick Le
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Hassam Zulfiqar
Hussain Sajjad
Steve Bhimji
Muhammad Hashmi
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Sarosh Vaqar
Mark Pellegrini
James Hughes
Beata Beatty
Beenish Sohail
Nazia Sadiq
Hajira Basit
Phillip Hynes


Updated:
6/3/2019 3:05:16 PM

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 incidence of children besides case reports.

Pathophysiology

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 transport of nutrients and 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
  • 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
  • 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 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 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
    • 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 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. 


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Scleritis - Questions

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A 55-year-old female with long-standing rheumatoid arthritis presents with complaints of photophobia, tearing, and constant, severe left eye pain that started last night. She denies any fever, but there is significant redness that does not respond to the administration of 2.5% topical phenylephrine. Gentle palpation of the affected eye through the eyelid causes a marked increase in pain. The vision in the left eye is slightly diminished. Her medications include artificial tears, prednisone, atenolol, and methotrexate. Which of the following is the most likely diagnosis?



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A 65-year-old female has been diagnosed with scleritis and has been taking 600 mg of ibuprofen three times per day for the last week. She presented for a follow-up and stated she had not had any improvement in her symptoms. Which would the next best treatment option?



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Which of the following features is characteristic of rheumatoid factor positive juvenile idiopathic arthritis (JIA)?



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Which of the following ocular conditions is commonly associated with rheumatoid arthritis?



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A 31-year-old white woman presents with redness, tearing, photophobia, eyelid swelling, and pain in her left eye for ten days. She was referred by her primary care provider for evaluation and treatment of conjunctivitis. She denies any change in vision, itchiness, or discharge. Her medical history is positive for hypothyroidism and migraines. On examination, the left eye has diffuse scleral edema and redness of superficial and deep episcleral vascular plexus. The vessels do not blanch with the installation of 2.5% phenylephrine. Which of the following is the most appropriate initial or next step in patient care?



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Scleritis - References

References

Scleritis., Okhravi N,Odufuwa B,McCluskey P,Lightman S,, Survey of ophthalmology, 2005 Jul-Aug     [PubMed]
Daniel Diaz J,Sobol EK,Gritz DC, Treatment and management of scleral disorders. Survey of ophthalmology. 2016 Nov - Dec     [PubMed]
Artifoni M,Rothschild PR,Brézin A,Guillevin L,Puéchal X, Ocular inflammatory diseases associated with rheumatoid arthritis. Nature reviews. Rheumatology. 2014 Feb     [PubMed]
Sims J, Scleritis: presentations, disease associations and management. Postgraduate medical journal. 2012 Dec     [PubMed]
Oray M,Meese H,Foster CS, Diagnosis and management of non-infectious immune-mediated scleritis: current status and future prospects. Expert review of clinical immunology. 2016 Aug     [PubMed]
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     [PubMed]

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