Posterior Scleritis

-Posterior scleritis is a rare inflammatory condition of the eye which is a diagnostic challenge because of its diverse clinical presentation

-Female predominance

-Mean age of presentation is around 45 years

-Unilateral but in a third of patients, is bilateral

-Symptoms: eye pain, headache, pain with ocular movements, and loss of vision if not treated timely

-Clinical signs are nonspecific: conjunctival chemosis, hyperemia, and optic nerve swelling

-The eye may also be without visible redness if inflammation is only posterior, making the diagnosis even more challenging

-The condition may masquerade as other ocular pathologies, emphasizing the need for a keen clinical eye to identify its distinct features.

D/D: Acute angle closure glaucoma, orbital/pre-septal cellulitis, orbital masses such as orbital pseudotumor also known as idiopathic orbital inflammation, rhabdomyosarcoma, leukemia, and neuroblastoma

-About 40-50% of scleritis are associated with systemic diseases

-The inflammatory condition most commonly associated is RA, but SLE and GPA are commonly linked

-Other etiologies of scleritis include infections (namely, varicella zoster virus (VZV) and herpes zoster virus (HZV)), orbital trauma, and medications such as bisphosphonates

-These patients are usually co-managed with a rheumatologist and an ophthalmologist

DIAGNOSIS:

-Slit lamp exam: inflammation of the anterior sclera with conjunctival chemosis, vessel dilation, and tortuosity

-Fundus: Optic nerve edema, serous RD, choroidal thickening and chorioretinal folds

-OCT may show choroidal thickening in active disease and is a useful tool to track disease course, with a reduction of thickening when treated effectively

-B-scan ultrasonography shows choroidal and scleral thickening greater than 2.5 mm, as well as fluid in sub-Tenon's space known as the pathognomonic T-sign

-MRI further compliments the diagnostic arsenal offering a more detailed anatomical outline and is useful when ultrasound is non-conclusive

-Certain laboratory tests can aid in the diagnosis of this condition, such as a serologic test for rheumatic diseases such as an anti-nuclear antibody (ANA), rheumatoid factor antibody (RF), anti-citrullinated antibody (CCP), and ANCA

MANAGEMENT:

-Oral corticosteroids: prednisolone is 1 mg/kg/day with weekly tapering depending on the clinical response

-Pulse IV corticosteroid in vision-threatening cases

-Methotrexate: first-choice steroid-sparing therapy

-Other steroid-sparing agents are azathioprine and mycophenolate for their tolerability and more rapid steroid-sparing effect when compared to methotrexate

-Cyclophosphamide is another agent used, but primarily in patients with systemic vasculitis such as GPA

-Biologic therapy: TNF-alpha inhibitors, Rituximab, newer biologic agents such as tocilizumab an interleukin-6 inhibitor

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Image from Rajan Eye Care Hospital

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