A rare case of bilateral sequential posterior scleritis in an elderly woman

Abstract

A 63-year-old woman came with complaints of decreased vision and minimal pain on extraocular movements in the left eye and, after investigations, was diagnosed with posterior scleritis. Systemic steroid treatment helped resolve the symptoms and signs. One year later, she presented with right eye involvement. She was evaluated for systemic illness and, based on clinical and investigational findings, it was deduced that probably a sarcoidosis was predisposing her to the eye problem. She was provisionally diagnosed with bilateral sequential posterior scleritis secondary to sarcoidosis. She was prescribed oral steroids, which helped resolve her eye problem. She was next referred to a rheumatologist and a pulmonologist for further evaluation and treatment.

Background

Posterior scleritis is a rare but potentially vision-threatening condition. It is often underdiagnosed due to varied clinical presentation. Nearly 80% of patients are in the 30–60 years age group, nearly 70% of them are women and 15% are bilateral at presentation.¹ We present a rare case of bilateral sequential posterior scleritis occurring in an elderly woman.

Case presentation

A 63-year-old elderly woman came with decreased vision in the left eye and mild pain on extraocular movements from 1 week. Her right eye best-corrected visual acuity (BCVA) was 20/20, N6 and left eye BCVA was 20/100, N12. Left eye fundus showed hyperemic disc with a pale subretinal lesion temporal to the fovea with surrounding exudative retinal detachment involving the macula (figure 1A). No vitreous cells were seen.

Figure 1.

(A) (left eye): Fundus photo showing a hyperemic disc with arrow pointing at pale subretinal fluid accumulation in macula temporal to fovea with exudative retinal detachment of macula. (B) (left eye): Fundus fluorescein angiography showing disc oedema and arrow pointing at exudative retinal detachment with pinpoint hyperfluorescence suggestive of active inflammation. (C) (left eye): Optical coherence tomography showing neurosensory detachment at fovea with fibrinous-like subretinal material temporal to fovea causing exudative retinal detachment with elevated choroid. (D) (left eye): B scan showing diffuse thickening of the choroid with low acoustic reflectivity. The sclera showed thickening and there was evidence of fluid in the subtenon space shown by the arrow.

Fundus fluorescein angiography (FFA) showed disc oedema and exudative retinal detachment with hyperfluorescent pinpoints suggestive of areas of maximal inflammatory activity (figure 1B). Optical coherence tomography (OCT) showed subretinal fluid accumulation with neurosensory detachment at fovea and subretinal fibrinous-like material temporal to the fovea causing exudative detachment with elevated choroidal layer (figure 1C). The left eye B scan showed diffuse thickening of the choroid with low acoustic reflectivity. The sclera showed thickening and there was evidence of fluid in the subtenon space (figure 1D). Oral steroid treatment helped resolve the disease activity, demonstrated by regression of pinpoint foci of hyperfluorescence on FFA. One year later, during sequential involvement of her right eye, the BCVA had deteriorated to 20/125, N18 and the fundus showed mild disc hyperemia and pale subretinal lesion temporal to the fovea with surrounding exudative retinal detachment involving the macula (figure 2A). The right eye FFA, OCT and B scan showed similarities to the left eye (figure 2B–D). She was a known case of type 2 diabetes and hypertension, both of which were under control. She had arthralgia in both her knees. Other systemic illness were ruled out as she did not have evening rise of temperature, weight loss, cough or arthralgia of small joints.

Figure 2.

(A) (right eye): Fundus photo showing a hyperemic disc with arrow pointing at pale subretinal fluid accumulation in macula temporal to fovea with exudative retinal detachment of macula. (B) (right eye): Fundus fluorescein angiography showing disc oedema and arrow pointing at exudative retinal detachment with pinpoint hyperfluorescence suggestive of active inflammation. (C) (right eye): Optical coherence tomography showing neurosensory detachment at fovea with fibrinous-like subretinal material temporal to fovea causing exudative retinal detachment with elevated choroid. (D) (right eye): B scan showing diffuse thickening of the choroid with low acoustic reflectivity. The sclera showed thickening and there was evidence of fluid in the subtenon space shown by the arrow.

Laboratory investigations

The blood counts indicated she had lymphopenia. Besides, her raised erythrocyte sedimentation rate (ESR) of 52 and raised C reactive protein (CRP) of 7.5 suggested active underlying inflammatory disease. Serum ACE levels were elevated at 46. The chest X-ray showed prominent bronchovascular markings near the hilar lymph nodes. The Monteux test and TB QuantiFERON gold assay were negative. Given the high ESR, CRP and serum ACE levels and low lymphocyte count and prominent bronchovascular markings, a provisional diagnosis of sarcoidosis was made. The high-resolution computerised tomography (HRCT) of chest showed ground glass haziness of lungs with mediastinal lymphadenopathy for which she was referred to a pulmonologist and a rheumatologist.

Differential diagnosis

When posterior scleritis is bilateral, it can be potentially mistaken for Vogt-Koyanagi-Harada’s (VKH) disease, which classically presents as a granulomatous panuveitis.² Proteinaceous fluid exudates produced in VKH accumulate in the choroidal stroma and may obscure the choroidal vessel, which appears as diffuse, homogenous and hyporeflective choroid on OCT.³ Dense hyporeflectivity of choroid, convolution of choroid, Retinal Pigment Epithelium (RPE) undulations, subretinal membranous structures and subretinal hyperreflective dots are the specific OCT markers for acute VKH.^{3 4} The other differentials of posterior scleritis with exudative retinal detachment are malignant melanoma, choroidal hemangioma, uveal metastasis, pseudotumors and orbital neoplasm can be lucidly delineated using clinical and imaging techniques.⁵ The commonly associated autoimmune conditions are rheumatoid arthritis, systemic lupus erythematosus, Wegener’s granulomatosis, polyangiitis, sarcoidosis, inflammatory bowel disease and giant cell arthritis.¹

Other causes include infections like TB, syphilis, toxoplasmosis and herpes. Other miscellaneous causes include malignancies, monoclonal gammopathy and myeloma. Hilar lymphadenopathy and granulomas on histopathology are confirmatory of sarcoidosis.⁶ High serum ACE levels and lymphopenia are positive predictors for sarcoidosis.⁷ As she had osteoarthritis of knee without small joint involvement, it clinically ruled out rheumatoid arthritis, which is the most common autoimmune aetiology in elderly patients.

Laboratory investigations show active underlying inflammatory disease with raised ESR and CRP. Lymphopenia and mild elevation of ACE levels are additional favouring factors for sarcoidosis.

Treatment

Both episodes of posterior scleritis were managed by oral steroid therapy. The patient was started on oral steroids, with weekly tapering by 10 mg doses of tablet Wysolone starting with dosage of 60 mg (1 mg/kg body weight) over for a period of 2 months. She was given oral calcium (tablet Shelcal 500 mg once a day) during oral steroid therapy to prevent steroid induced complications like osteopenia.

Outcome and follow-up

After starting oral steroid therapy, there was good resolution of symptoms and signs in both eyes.

Left eye fundus and OCT show complete resolution of fluid and recovery of visual acuity to 20/20, N6 at 2 months follow-up period (figure 3C, D). One year later, when she presented with right eye posterior scelritis, she showed good response to oral steroid therapy with resolution of exudative detachment and resolving fluid in the OCT (figure 3A, B). Visual acuity in the right eye was recovered to 20/30, N9 at 1 month follow-up. She was referred for rheumatology and pulmonology opinion for further evaluation, confirmation and management of our diagnosis of systemic sarcoidosis.

Figure 3.

(A) (right eye) and (B) (left eye): Fundus photo showing resolution of exudative retinal detachment in response to therapy. (C) (right eye) and (D) (left eye): Optical coherence tomography with complete resolution of subretinal fluid and attached neurosensory retina after treatment. The right eye with decrease in subretinal fluid and decrease in size of neurosensory detachment, 2 weeks after initiating oral steroid treatment.

Discussion

In the literature, the most common aetiology of bilateral posterior recurrent scleritis was idiopathic.² Tuberculosis (TB) and giant cell arthritis are described in some case reports for bilateral recurrent aetiology.^{8 9} Our patient showed features of both ocular and systemic sarcoidosis. These patients are often known to present with headache and disc oedema.¹⁰ Oral corticosteroids are widely used, effective first-line agents for inflammatory scleritis, with refractory and recurrent cases alone requiring antimetabolites, T-cell inhibitors or biologic response modifiers.^{10 11} Early treatment for these patients can prevent potential vision threatening sequelae.

Our patient was elderly and showed sequential involvement of the left eye followed by the right eye, responding well to oral steroid treatment. Our patient symptomatology of pain on extraocular movements, unilateral presentation, with ocular features of disc oedema, neurosensory detachment was characteristic of posterior scleritis. Even through FFA, OCT findings may mimic other disease findings, ultrasound showing subtenons fluid often clinches the diagnosis of posterior scleritis, which was seen in our case as well.¹² The uniqueness of our case is in its bilateral sequential presentation. Horo et al also reported a patient with posterior scleritis who had one episode in the right eye and four episodes in the left eye over a 35-month period.¹³

Our patient’s FFA features indicated stippled pinpoint hyperfluorescence and OCT features of neurosensory detachment with subretinal fibrinous material without the presence of RPE undulations or subretinal septae. These are typical features of posterior scleritis differentiating from VKH.^{3 4}

Posterior scleritis is generally autoimmune in origin and systemic disease can be an associating feature in more than 40% of cases especially in patients who are more than 50 years of age.¹ Bilateral hilar lymphadenopathy is the most typical sign at chest radiography in sarcoidosis. HRCT evidence of hilar lymphadenopathy and biopsy proven evidence of granuloma are confirmatory investigations for the diagnosis of sarcoidosis.¹⁴ Our case is an elderly woman who showed ground glass haziness of lungs with mediastinal lymphadenopathy a typical feature of systemic sarcoidosis. Hence, the patient is referred to pulmonology and rheumatology for further evaluation and management to prevent recurrences. Immunomodulatory therapy will be required in certain cases to prevent recurrences.

Learning points.

• Multimodal imaging plays a pivotal role in accurately diagnosing and managing posterior scleritis.

• Autoimmune disease can still be a probable diagnosis in elderly patients, presenting with the first episode of bilateral sequential posterior scleritis.

• Immediate treatment with systemic steroids or immunosuppressants helps in rapid resolution of vision threatening sequelae.