Vasculitic central retinal vein occlusion: The presenting sign of seronegative rheumatoid arthritis

Abstract

Purpose: To report the case of a patient who presented with a vasculitic central retinal vein occlusion (CRVO), which was the result of an undiagnosed systemic inflammatory condition, seronegative rheumatoid arthritis (RA).

Observations: The patient presented with reduced vision in the left eye and polyarthralgia. Fundoscopic examination revealed a central retinal vein occlusion (CRVO) with concurrent evidence of vasculitis. Work-up for polyarthralgia included comprehensive serologic testing for connective tissue disease, including Vectra^® disease activity (DA) testing. Results of these studies confirmed the diagnosis of seronegative rheumatoid arthritis (RA). Systemic steroid therapy was initiated with subsequent anatomic and visual improvement.

Conclusions and importance: We hypothesize that the systemic inflammation-a hallmark of RA-led to the development of a vasculitic CRVO and, thus, the retinal manifestations served as the disease marker that prompted thorough work-up of the patient's disease, even in the face of initial seronegativity. This case serves as a reminder that, in the setting of CRVO and polyarthralgia, systemic inflammatory conditions must be considered as the underlying etiology. Further, this case report highlights our evolving understanding of the role that serologic markers play in the diagnosis and monitoring of RA.

Keywords: Central retinal vein occlusion (CRVO); Rheumatoid arthritis (RA); Seronegative rheumatoid arthritis; Vasculitic CRVO; Vectra DA biomarker assay.

Fig. 1

Widefield color fundus photograph shows dilated and tortuous veins with scattered flame-shaped hemorrhages, cotton-wool spots and perivascular exudation.

Fig. 2

(A) Early phase widefield fluorescein angiography showed capillary non-perfusion. (B) Late phase widefield fluorescein angiography showed staining of the vessel wall and perivascular leakage.

Fig. 3

(A) Spectral-domain optical coherence tomography (SD-OCT) at the time of presentation showed significant macular edema and vitreous cell; (B) On day three of the initial topical steroid therapy, SD-OCT showed marked improvement of the intraretinal fluid; (C) On day ten, the macular edema recurred despite continued topical therapy. Intravitreal ranibizumab was administered at this time. (D) At 3.5 months of monthly ranibizumab treatment, the patient's visual acuity improved to 20/30 and there was a resolution of the macular edema.