Optic Nerve Involvement with Panuveitis in Sweet Syndrome

Abstract

Sweet syndrome can present with optic nerve involvement. Screening for underlying etiologies, including autoimmune disease, malignancy, and offending drugs, is important for targeted therapy.

Neuro-Ophthalmic Presentation of Neuro-Sweet Disease

“…These occur due to inflammation of the ocular, orbital, and periorbital tissues leading to dacryoadenitis, conjunctivitis, episcleritis, scleritis, limbal nodules, keratitis, uveitis (anterior, posterior and panuveitis), glaucoma, retinal vasculitis, and optic neuropathy. 1,8,9 Our case report highlights the diagnostic difficulty for rare presentations of systemic conditions. Presentation with papilloedema and elevated CSF pressure with normal contents led to the falsely reassuring diagnosis of idiopathic intracranial hypertension and treatment with acetazolamide, despite tell-tale signs of a systemic inflammatory process-constitutional symptoms, headache, skin rash, uveitis, and elevated inflammatory markers.…”

“…10 Several cases of SS in literature describe simultaneous occurrence of intraocular and central nervous system (CNS) inflammation, which suggests that SS may be a cause of uveo-meningeal syndrome. 8 We believe that our patient most likely had uveo-meningitis at presentation. Although the initial CSF results were "normal", we believe that CSF pressure was elevated due to aseptic meningitis.…”

“…Neurological signs and symptoms include headache and altered consciousness and may precede development of skin lesions. 8 Ophthalmic manifestations may be seen in approximately a third of SS. These occur due to inflammation of the ocular, orbital, and periorbital tissues leading to dacryoadenitis, conjunctivitis, episcleritis, scleritis, limbal nodules, keratitis, uveitis (anterior, posterior and panuveitis), glaucoma, retinal vasculitis, and optic neuropathy.…”

Sweet Syndrome in a Patient With Systemic Lupus Erythematosus Flare.

“…The diagnosis of Sweet syndrome is based on two major and two of four minor criteria's (Table 1) [13,14,15]. Our patient met almost all of the major and minor criteria required for the diagnosis of Sweet syndrome.…”

Surgical results of strabismus correction in patients with myelomeningocele

“…Of all cases 15%-20% are associated with hematological malignancies (most commonly acute myelogenous leukemia) and solid tumors (most commonly carcinomas of the genitourinary organs, breast, and gastrointestinal tract). Ocular involvement has been reported in 4%-72% of patients with Sweet syndrome and ocular manifestations include conjunctivitis, episcleritis, subconjunctival hemorrhage, scleritis, inflammatory glaucoma, choroiditis, iritis, optic nerve involvement, limbal nodules, periorbital eruption, or orbital and eyelid inflammation with vesicular lesions (5,7,8) . Systemic corticosteroids (oral prednisone or prednisolone) are the mainstays of treatment for Sweet syndrome (9) .…”