Novel strategies for the diagnosis and treatment of scleritis

ABSTRACT

Introduction

Scleritis is a severe, vision-threatening ocular inflammation often associated with systemic diseases that carry high risks of morbidity. Timely diagnosis and management are crucial to prevent sequela.

Areas Covered

This review encompasses the etiology, pathophysiology, classification, diagnosis, and treatment of scleritis with an emphasis on novel diagnostic and treatment modalities. A review of the literature was performed using the keywords ‘scleritis,’ ‘autoimmune diseases,’ ‘non-steroidal anti-inflammatory drugs,’ ‘antimetabolite,’ ‘immunomodulatory therapy,’ ‘tumor necrosis factor alpha inhibitors,’ biologic response modifier’ and ‘optical coherence tomography.’

Expert Opinion

Scleritis is mainly a clinical diagnosis. Nevertheless, multimodal imaging may be used in diagnostically challenging cases. New approaches such as anterior segment optical coherence tomography (OCT), OCT angiography (OCTA), and ultrasound biomicroscopy (UBM) may serve as more objective measures to diagnose and monitor scleritis patients. The etiology, severity, anatomical location, and associated systemic inflammatory diseases should be considered when determining scleritis treatment. Currently, first-line treatment in low grade non-necrotizing anterior scleritis is usually non-steroidal anti-inflammatory drugs (NSAIDs) and/or topical/systemic corticosteroids. In non-responsive cases, systemic immunomodulatory therapy (IMT) is recommended. Novel treatment with biologic response modifiers (BRMs) is promising in refractory scleritis. Further studies are needed to evaluate efficiency and safety of BRMs.

KEYWORDS:

• Antimetabolite
• autoimmune disease
• biologic response modifier
• immunomodulatory therapy
• non-steroidal anti-inflammatory drugs
• optical coherence tomography
• scleritis
• tumor necrosis factor alpha inhibitors

Article highlights

• Scleritis is often associated with inflammatory systemic disease that can have significant extraocular morbidity if not correctly diagnosed or treated. Timely diagnosis and management of the scleritis and any associated systemic disease is critical.

• Ocular complications of scleritis include anterior uveitis, decreased visual acuity, cataract, peripheral ulcerative keratitis, glaucoma, vitreitis, cystoid macular edema and exudative retinal detachment. Complications are more common in necrotizing scleritis and posterior scleritis.

• Scleritis can usually be diagnosed after careful history taking, macroscopic and biomicroscopic examination, but it can be more challenging to identify posterior scleritis in the absence of anterior segment involvement. Multimodal imaging such as B scan ultrasonography, OCT, FFA/ICGA, MRI/CT may be helpful in these cases.

• Novel diagnostic strategies are evolving in scleritis. Anterior segment OCT imaging demonstrates increased scleral thickness and intrascleral hyporeflective areas of edema in active anterior scleritis. OCTA has been used to measure scleral area vessel density and correlate with scleritis activity. UBM was found to be superior to slit-lamp examination in detecting scleral necrosis, scleral thinning, and defining the type of scleritis. A study with thermographic camera images revealed high temperatures in scleritis patients compared to control.

• Treatment regimens for scleritis vary depending on the location, severity, and any associated ocular complications or systemic conditions. First-line treatment is usually systemic NSAIDs, followed by corticosteroids and/or corticosteroid-sparing immunomodulatory medication. In general, the majority of scleritis patients with associated systemic inflammatory disease or those with necrotizing anterior or posterior scleritis will require systemic immunosuppressive medication.

• Novel topical/regional treatment modalities include topical tacrolimus, subconjunctival sirolimus, topical erythropoietin, and iontophoresis corticosteroid delivery system.

• BRMs are increasingly used to treat scleritis recalcitrant to other regimens. TNF-α inhibitors are the most used class of BRMs. Another promising option is rituximab.

• Further randomized, prospective clinical trials are needed to evaluate efficiency and safety of new treatment modalities in scleritis.

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.