Bilateral Occlusive Retinal Vasculitis in a Patient with Dermatomyositis

Abstract

A 48-year-old woman with a history of dermatomyositis (DMS) presented with 2 weeks of worsening myalgias, weakness, and diffuse edema following cessation of her systemic immunosuppression and subsequently developed severe bilateral vision loss consistent with bilateral frosted branch angiitis. Multimodal imaging was performed, and the patient was successfully treated with pulse-dose steroids and intravenous immunoglobulin, as well as intravitreal aflibercept. Ophthalmic involvement of DMS is typically limited to episcleritis, conjunctivitis, and uveitis. We present an uncommon case of bilateral occlusive retinal vasculitis with frosted branch angiitis in a patient with DMS. The significant improvement anatomically and in visual acuity in our patient suggests a role of combined anti-vascular endothelial growth factor and systemic immunosuppression in the management of DMS -related frosted branch angiitis. We suggest that retinal vasculitis should be considered in patients with known DMS and acute vision loss, with prompt referral for ophthalmologic evaluation.

Introduction

Dermatomyositis (DMS) is a rare idiopathic inflammatory myopathy that presents with characteristic dermatologic and muscular findings.[1] Ophthalmic involvement is typically limited to episcleritis, conjunctivitis, and uveitis. We report the first documented case of bilateral occlusive retinal vasculitis with frosted branch angiitis in a patient with DMS. We suggest that retinal vasculitis should be considered in patients with known DMS and acute vision loss, with prompt referral for ophthalmologic evaluation.

Case Report

A 48-year-old woman with a history of DMS presented with 2 weeks of worsening myalgias, weakness, and diffuse edema. She had been diagnosed 5 months previously with DMS based on classic clinical findings (Gottron’s papules, heliotrope rash, and V-sign) and bilateral proximal muscle weakness. She was initially managed with oral methylprednisolone and azathioprine, but due to multiple infections and leukopenia, these medications were held for several weeks. Her myalgias returned shortly before the presentation, and the medications were restarted. She was admitted for worsening symptoms, and on the 3^rd day of admission, she developed severe bilateral vision loss.

On initial ophthalmologic examination, her visual acuity was count fingers in each eye with a normal anterior segment. Fundus examination demonstrated severe, bilateral, and symmetric retinal vascular tortuosity with extensive diffuse hemorrhagic perivascular sheathing and macular edema [Figure 1]. Intravenous fluorescein angiography demonstrated diffuse bilateral perivascular leakage consistent with vasculitis, macular leakage, and peripheral capillary nonperfusion [Figure 1].

Figure 1.

Composite color fundus photographs and fluorescein angiography at presentation. Note the diffuse retinal hemorrhages following the retinal vasculature in the classic “frosted branch” pattern. The corresponding fluorescein angiogram demonstrates corresponding perivascular leakage, blocked fluorescence from hemorrhaging, and peripheral ischemia

Intravenous ganciclovir was initiated for empiric treatment of herpetic retinitis, and she underwent vitreous biopsy. Extensive further infectious, hematologic, and rheumatologic workup was unremarkable aside from a positive anti-ribonucleoprotein (RNP) and a muscle biopsy consistent with DMS. She was treated with pulse-dose steroids and intravenous immunoglobulin, as well as intravitreal aflibercept. Her systemic symptoms improved, and her vision gradually began to recover. Eight weeks later, her (uncorrected) vision improved to 20/150 in the right eye and 20/125 in the left eye. Follow-up examination demonstrated improvement in the vasculitis with resolution of retinal hemorrhages [Figure 2a and b].

Figure 2.

(a) Composite color fundus photographs at 8 weeks. These photos show significant improvement of the vasculitis as well as retinal hemorrhaging. Of note, a boat-shaped preretinal hemorrhage is visible inferiorly in each image. (b) Composite color fundus photographs at 15 weeks. The vasculitis is significantly improved with almost complete resolution of retinal hemorrhaging. There is no residual vasculitis and the macula is clear in both eyes

Discussion

DMS is a rare idiopathic inflammatory myopathy that presents with characteristic dermatologic and muscular findings. Ophthalmic involvement is typically limited to episcleritis, conjunctivitis, and uveitis. Rarely, myositis can involve the extraocular muscles, causing pain and exophthalmos. DMS-related retinopathy, first described by Bruce[1] in 1938, is also rare and is typically characterized by cotton-wool spots, intraretinal hemorrhage, and macular edema or exudates. These findings usually resolve completely without lasting visual impairment but rarely can cause persistent and profound vision loss.[2] DMS has previously been associated with central retinal vein occlusion and occlusive retinopathy, presumably either due to a systemic thrombotic thrombocytopenia or phlebitis.[3]

The term frosted branch angiitis can be descriptive of a distinct clinical syndrome in its idiopathic form or a clinical sign associated with neoplastic, infectious, or autoimmune disease. It is characterized by severe vasculitis with perivascular sheathing affecting the entire retina, resembling the frosted branches of a tree. Although the pathogenesis of frosted branch angiitis is unknown, histopathologically this sheathing represents the invasion of the perivascular space with lymphoplasmacytic infiltrates.[4]

To our knowledge, this is the first reported case of DMS associated with severe, bilateral occlusive retinal vasculitis with a frosted branch angiitis appearance. Lee et al.[5] described a similar case of unilateral frosted branch angiitis associated with DMS. Our case differs in that it was bilateral, accompanied by extensive preretinal and intraretinal hemorrhages consistent with occlusive disease, and demonstrated peripheral ischemia on fluorescein angiography. Our patient had a positive anti-RNP, which has been associated with mixed connective tissue disease, an overlap syndrome with features of systemic lupus erythematosus, systemic sclerosis, and polymyositis.[6] We hypothesize that this may have contributed to the severity of our patient’s retinal vasculitis. Our case adds to the growing body of evidence that DMS can cause retinal vasculitis with a frosted branch appearance. We suggest that retinal vasculitis should be considered in patients with known DMS and acute vision loss, with prompt referral for ophthalmologic evaluation.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.