Bilateral episcleritis followed by right optic perineuritis with severe visual loss: a case report.

Abstract

Persistent red eye may result in severe visual impairment. Scleritis is one possible cause of red eye that is considered to pose a significant threat to vision [1]. In contrast, episcleritis is confined to the superficial episcleral tissue and is thought to represent a mild, non-vision-threatening form of ocular inflammation, usually idiopathic in nature, that does not generally involve other ocular structures [1]. Idiopathic optic perineuritis has been reported as a type of orbital inflammatory pseudotumor [2, 3] with slow onset and often poor recovery in the event of delayed treatment [4]. Comorbid sclerotic inflammation and optic perineuritis have rarely been reported [5]. Here we describe a patient presenting with severe visual loss caused by bilateral episcleritis followed by right optic perineuritis. An 85-year-old woman was referred to our hospital with right visual loss. She had a past history of acute left visual loss and recovery with oral steroid therapy in her 50s, of which the details were unknown. Prior to referral, she had developed upper respiratory tract inflammation and visited her ophthalmologist 2 weeks later complaining of bilateral red eyes without ocular pain. At this time, her visual acuity was 20/40 with correction in each eye. She was diagnosed with episcleritis and treated with eye lotion containing corticosteroids. However, her eyes remained red 3 months later, and she noticed mild right ocular pain and decreased visual acuity in her right eye. She was finally referred to our department 2 months after the onset of visual impairment. On admission, her right visual acuity with correction was reduced to 20/200 and papilledema was detected in her right eye. No abnormal ocular movements were observed and her visual fields were normal. Fundus fluorescein angiography showed no apparent abnormalities. No other neurological deficit which might suggest multiple sclerosis or neuromyelitis optica spectrum disorders was detected. Other physical examinations were normal and there was no lymphadenopathy. Blood tests showed an erythrocyte sedimentation rate of 23 mm in the first hour, a white blood cell count of 7000/ll, C-reactive protein of 0.01 mg/dl, an angiotensin-converting enzyme level of 12.6 IU/l (normal 8.3–21.4 IU/l), a lysozyme level of 4.9 lg/ml (normal 5.0–10.2 lg/ml), Treponema pallidum hemagglutination assay and fluorescent treponemal antibody negativity. Antinuclear antibody was positive but rheumatoid factor and other specific autoantibodies including anti-aquaporin 4 antibody, anti-DNA antibody, and anti-neutrophil cytoplasmic antibodies were negative. Tests for bacterial, viral, fungal and tuberculous infections were all negative. Other blood studies, including renal and liver function tests, thyroid function tests, soluble interleukin-2 receptor, and adenosine deaminase, were all within the normal ranges. Chest X-ray findings were normal. Magnetic resonance imaging (MRI) of the orbit revealed high intensity at the swollen right optic nerve sheath in coronal short tau inversion recovery sequence (STIR) (Fig. 1a). The soft tissues around the right optic nerve sheath and right posterior sclera also showed high intensity in coronal and axial STIR images (Fig. 1a, b). The right optic nerve sheath and right posterior sclera were slightly enhanced in axial gadolinium-DTPA T1-weighted images using a fat-suppression technique (Fig. 1c). Systemic examination with whole-body computed tomography and gallium-scan & Haruo Nishijima hnishijima-tky@umin.ac.jp