ABSTRACT

Central serous chorioretinopathy (CSCR) is characterized by a well-defined serous choroidal detachment of the retinal pigment epithelium with one or more focal lesions of the neurosensory retina. Risk factors for CSCR are psychosocial stress, increased endogenous catecholamine, and increased endogenous cortisol. Systemic steroids can cause ocular side effects such as cataract development, increased intraocular pressure, and less frequently the development of CSCR, which can resolve spontaneously with close follow-up and simple treatment modification. CSCR should be considered in patients who complain of worsening vision under steroid treatment for pathologies requiring steroid therapy. In this study we present two patients, one man and one woman, who developed acute CSCR while under systemic steroid treatment for Behçet’s disease.

Introduction

Behçet’s disease is a multisystemic condition of unknown etiology characterized by chronic, recurrent vasculitis. Ocular and systemic complications can be prevented by controlling the disease with early and effective treatment. Systemic steroids and immunomodulatory agents play an important role in controlling inflammation. Long-term steroid use can lead to systemic side effects such as osteoporosis, predisposition to infection, intestinal ulcers, hyperglycemia, and exacerbated hypertension, as well as severe ocular side effects such as cataract, elevated intraocular pressure and, less frequently, central serous chorioretinopathy (CSCR).¹

CSCR is characterized by well-defined serous detachment of the neurosensory retina at the macula that may be accompanied by retinal pigment epithelium (RPE) detachment.^2,3 The pathogenesis is believed to involve choroidal hyperperfusion and RPE barrier dysfunction. Different forms of steroid treatment, such as oral, inhaled, intranasal, intravitreal, and epidural, can cause CSCR.⁴ Here we present two cases, one woman and one man, who developed acute CSCR while under systemic steroid therapy for Behçet’s disease.

Discussion

CSCR commonly affects young/middle-aged men aged 25-55 years and is often unilateral and reversible. About 40% of patients have bilateral disease, though this rate is higher among patients with chronic CSCR.⁵ Similarly, our patients were 44 and 37 years old and represented both sexes. Only the first patient had bilateral CSCR. The primary symptom of CSCR is sudden blurred vision due to fluid leakage and serous detachment in the macula. Metamorphopsia, micropsia, central scotoma, or impaired color vision may also occur.

Risk factors include psychosocial stress, elevated endogenous catecholamine, hypertension, pregnancy, organ transplantation, and obstructive sleep apnea.⁶ Because psychosocial stress was identified as a risk factor in our patients, psychiatric consultation was requested for both of them. Corticosteroids are another important risk factor in the development of CSCR.^7,8 Steroid use is believed to increase the permeability of choroid capillaries and RPE by inhibiting collagen synthesis and disrupting ion pump function.^9,10 Glucocorticoids are also known to alter blood-aqueous barrier permeability and disrupt the external blood-retinal barrier by increasing cAMP levels in RPE cells.¹¹ Steroids administered by various routes (oral, inhaled, intranasal, intravitreal, epidural) contribute to the development CSCR.^4,12 In addition, the literature includes reports of a patient under systemic steroid therapy for “retrobulbar neuritis” developing multiple areas of serous retinal detachment in both eyes and a patient misdiagnosed with Vogt-Koyanagi-Harada disease and treated with corticosteroids who actually had atypical bullous CSCR.^13,14 Both of our patients were receiving oral steroid therapy for Behçet’s disease when they presented with CSCR.

Visual prognosis is generally very good in CSCR and follow-up alone is recommended if there is no underlying pathology. Most patients show spontaneous resolution within 3-4 months.¹⁵ Patients with steroid-associated CSCR should be followed with their steroid dosage reduced as low as permitted by their disease. In these patients, as in our 2 cases, clinical findings return to normal within a few months, resulting in good visual improvement. The rate of spontaneous resolution is higher in steroid-related cases than with other CSCR etiologies.¹⁶ Photodynamic therapy (PDT) is a treatment approach commonly used for patients who do not show spontaneous resolution within 3 months, have bilateral or recurrent disease, or who require rapid visual rehabilitation.¹⁷ However, the effectiveness of PDT may vary for steroid-associated CSCR.⁸ Furthermore, a meta-analysis evaluating the efficacy of anti-vascular endothelial growth factor (anti-VEGF) therapy in CSCR showed that anti-VEGF therapy was not superior to observation at 6-month follow-up in acute cases in which persistent subretinal fluid did not last longer than 3 months.¹⁸ In an evaluation of comparative studies of chronic cases, however, anti-VEGF therapy was not superior to observation in terms of best corrected visual acuity (BCVA), but there were significant differences between the two groups in terms of central macular thickness (CMT).¹⁸ In the same meta-analysis, it was reported that non-comparative studies demonstrated significant differences in BCVA and CMT after anti-VEGF therapy at 1, 6, and 12 months follow-up.¹⁸ Although results have been controversial, intravitreal anti-VEGF injection may be beneficial in chronic CSCR by reducing choroidal vascular hyperpermeability and obstruction.¹⁹ Both of our patients showed spontaneous resolution.

In conclusion, not only uveitis but also non-uveitic pathologies such as CSCR should be considered when patients present with reduced vision while taking steroids for pathologies such as Behçet’s disease that require steroid therapy. Otherwise, CSCR cases exhibiting atypical clinical features may be difficult to diagnose. CSCR can resolve spontaneously with close follow-up and simple treatment modifications.