ABSTRACT

A 42-year-old man with undiagnosed Churg-Strauss syndrome (CSS) developed bilateral central retinal artery occlusion (CRAO). His medical history included bronchial asthma and irregular prednisolone usage but no atherosclerotic risk factors. At presentation, visual acuity (VA) was hand motion in the right eye and counting fingers in left eye. On fundoscopy, retinal whitening and a cherry red spot were observed in the right eye, while the fundus was normal in the left eye. After eyeball massage and systemic intraocular pressure lowering agents, his VA improved. On day 5 of treatment, he experienced right limb weakness and purpura on his right foot, and electromyography revealed mononeuritis multiplex. Laboratory tests indicated eosinophilia (52%). Based on the presence of hypereosinophilia, bronchial asthma, mononeuritis multiplex, vasculitis purpura, and sinusitis that was detected during etiological investigations, the patient was diagnosed as having CSS according to the American College of Rheumatology diagnostic criteria. Intravenous methylprednisolone 1 g/day was administrated for 3 consecutive days and 1 g cyclophosphamide was started and continued monthly for 6 months. Foot drop and vasculitic purpura improved after 7 days, but there was no further improvement in visual acuity. In conclusion, in the presence of bilateral CRAO and lack of atherosclerotic risk factors, CSS should be considered as a predisposing factor and investigations should be conducted accordingly.

Introduction

Central retinal artery occlusion (CRAO) is a devastating ocular emergency that usually occurs secondary to one or more serious systemic diseases such as carotid artery or cardiac valvular disease, hypercoagulability, atrial fibrillation, and autoimmune diseases.¹ According to pathophysiology, CRAO can be divided into two groups, arteritic and non-arteritic. The arteritic category comprises less than 5% of CRAO cases and is related to a vasculitic etiology.² Eosinophilic granulomatosis with polyangiitis (EGPA), also called Churg-Strauss syndrome (CSS), is known as a form of vasculitis characterized by inflammation of the blood vessels that can restrict blood flow and damage vital organs and tissues. Individuals diagnosed with EGPA usually have a history of asthma or allergies. Despite the presence of clear diagnostic criteria, the diagnosis of CSS can be delayed in the clinical setting. This is partly related to the sheer variety of clinical presentations of the disease.³ Regarding anti-neutrophil cytoplasmic antibody (ANCA) status, CSS can be divided into two major subsets: the ANCA-positive patients, who demonstrate clinical and histopathologic features of vasculitis, and the ANCA-negative patients, who exhibit tissue eosinophilic infiltration.⁴ Eosinophilic myocarditis⁵, neuroendocrine carcinoma⁶, eosinophilic vasculitic neuropathy⁷, multiple oral ulcerations⁸, and inflammatory pseudotumor of the anterior orbit⁹ have been reported as initial presentations of CSS in the literature. Herein, we describe a patient with undiagnosed CSS who developed simultaneous bilateral CRAO. To the best of our knowledge, this is a rare case of CSS with this clinical presentation, which we recognized during CRAO workup. This case report is of great importance, as the first presentation of CSS might be purely ocular.

Discussion

CSS is a rare disease with small vessel vasculitis characterized by eosinophilia and occurs almost exclusively in patients with asthma. The syndrome can vary in presentation and there is no pathognomonic ocular finding. Ocular presentations can be categorized into 2 groups, largely for prognostic explanations, as idiopathic orbital inflammation-type and ischemic vasculitis-type. Patients with ischemic vasculitis tend to be older than those with idiopathic orbital inflammation.¹⁰ According to a report by Takanashi¹¹, ANCA-positive patients are more likely to present clinically with classical small-vessel vasculitis, which affects the disease progression and outcomes. However, this was not confirmed in a study by Akella et al.¹⁰ that showed no statistically significant difference in ANCA positivity between the ischemic and inflammatory groups. In the literature, ischemic vasculitis presentations including retinal artery and vein occlusions have been reported in 12 patients. These presentations were mostly accrued in the known cases of CSS and were not the first presentations, and in two cases they were bilateral.¹⁰ However, our patient was ANCA-negative with bilateral CRAO as the first presenting clinical sign, and steroid pulse therapy resulted in no improvement in visual acuity. This result is consistent with the Akella¹⁰ study showing that ischemic vasculitis-type ophthalmic presentations have a less dramatic response to steroids. However, early diagnosis and timely treatment of CSS can prevent systemic life-threatening complications.

In the presence of bilateral CRAO and lack of atherosclerotic risk factors, it is important to rule out systemic vasculitis like CSS. Early diagnosis of the disease can be life-saving. However, the effect of systemic treatment on improving the patient’s vision is still unclear.