Scleritis

What Is It, Causes, Symptoms, and More

Author: Lily Guo, MD

Editors: Alyssa Haag, MD, Emily Miao, MD, PharmD,Kelsey LaFayette, DNP, ARNP, FNP-

Illustrator: Abbey Richard, MSc

Modified: 7 Jun 2024

What is scleritis?

Scleritis is the inflammation of the sclera, the white part of the eye, and is a painful, destructive, and potentially sight-threatening condition. The sclera is situated beneath the conjunctiva (i.e., the eye's outer protective membrane) and the episclera (i.e., the outermost layer of the sclera), and overlies the choroid (i.e., the eye's vascular layer). Scleritis can be divided into a visible anterior portion and a posterior portion located behind the orbit (i.e., the bony socket in which the eyeball sits), with both segments susceptible to scleritis. 

What causes scleritis?

Scleritis may be idiopathic, or without a known cause, but is often associated with underlying inflammatory and autoimmune disorders, such as rheumatoid arthritis, vasculitides (e.g., granulomatosis with polyangiitis), connective tissue diseases (e.g., scleroderma, systemic lupus erythematosus), inflammatory bowel disease (e.g., Crohn disease, ulcerative colitis), and Sjögren syndrome. The inflammation in scleritis can also result from bacterial (e.g., Lyme disease) or viral (e.g, herpes zoster) infections. Trauma, eye injury, or surgical procedures may predispose individuals to bacterial infection, leading to infectious scleritis. Additionally, medications like bisphosphonates (e.g., pamidronate disodium, zoledronate, alendronate sodium) can induce drug-induced scleritis. 

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What are the signs and symptoms of scleritis?

Signs and symptoms of scleritis include severe, constant eye pain exacerbated by eye movement. In anterior scleritis, the eye's white part may appear violaceous and red, while this is less likely in posterior scleritis. Pain is typically worse at night or in the early morning, potentially disrupting sleep. The pain may radiate to the periorbital region and face, thereby causing restricted eye motion and potential vision loss. Additional symptoms include headaches, eye-watering, and photophobia. 

How is scleritis diagnosed?

Scleritis is diagnosed through patient history and physical examination. Patients may report eye pain, tenderness to palpation of the eyelid, and a violaceous redness, particularly seen in anterior scleritis. Healthcare providers may conduct slit-lamp examinations (i.e., a special microscope with a bright light) and ophthalmoscopy. Posterior scleritis, which poses more of a diagnostic challenge due to the potential lack of eye redness on clinical examination, may require imaging methods like B-scan ultrasonography and optical coherence tomography (OCT), which can both reveal scleral thickening. Computed tomography (CT) and magnetic resonance imaging (MRI) can help in identifying posterior inflammation. A biopsy may be pursued in select cases to rule out infiltrative processes (e.g., sarcoidosis, lymphoma) or infectious causes when not responding to empirical antibiotic therapy. 

Diagnostic evaluation for associated systemic disorders involves a review of symptoms, including joint pain, swelling, and gastrointestinal symptoms (e.g., abdominal pain, diarrhea, and blood in the stool). Laboratory testing may include complete blood count (CBC), serum chemistry profile, urinalysis, and acute phase reactants (e.g., C-reactive protein). Specialized serologic assays such as rheumatoid factor, anti-CCP antibodies, and antinuclear antibody testing may be performed depending on the suspected underlying cause. 

How is scleritis treated?

For mild cases of scleritis, treatment involves nonsteroidal anti-inflammatory medications (NSAIDs), such as ibuprofen. Cases unresponsive to oral NSAIDs may require high-dose systemic glucocorticoids (e.g., prednisone) or a combination with an immunosuppressive agent (e.g., methotrexate, mycophenolate mofetil) for disease control. Biologics like TNF inhibitors (e.g., adalimumab, infliximab) or rituximab may be necessary, especially in persistent, moderate to severe cases. Cyclophosphamide has shown efficacy in scleritis associated with granulomatosis with polyangiitis. Scleritis secondary to infection may be treated with antibiotic and antiviral medications. Finally, the offending medication should be discontinued in cases of drug-induced scleritis. 

What are the most important facts to know about scleritis?

Scleritis is an inflammatory condition affecting the sclera, the white part of the eye, characterized by pain, redness, and potential vision impairment. Scleritis is often associated with inflammatory and autoimmune diseases like rheumatoid arthritis, vasculitides, and connective tissue disorders. Eye infections, trauma, and medications can also trigger scleritis. The condition presents with severe, constant eye pain that worsens with movement, potentially causing disrupted sleep, headaches, and photophobia. Diagnosis involves comprehensive patient history, physical examinations, and if necessary, imaging methods. Treatment strategies include NSAIDs, systemic glucocorticoids, and, in some cases, immunosuppressive agents or biologics, depending on the severity and underlying cause. Early diagnosis and appropriate management are crucial to prevent potential vision-threatening complications. 

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