Bilateral occlusive retinal vasculitis associated with primary sclerosing cholangitis

Abstract

We present the case of a 46-year-old man who presented with bilateral panuveitis and occlusive retinal vasculitis 6 months after being acutely admitted with abnormal liver function and diagnosed with primary sclerosing cholangitis (PSC). Initial investigations by the medical and ophthalmic departments including all autoimmune investigations were within normal parameters. Of particular interest was the high likelihood of inadvertent androgenic-anabolic steroid self-suppression of disease. As a lifelong bodybuilder, the patient had been taking oral and intramuscular steroids for years. He became symptomatic upon cessation of these recreational medications. There remains a significant paucity of information describing the relationship between uveitis and PSC. Given the poorly understood aetiology of this rare cholestatic disease, we review the current literature and highlight the diagnostic and therapeutic challenges for such a patient. PSC may predispose patients to an occlusive panuveitis with androgenic-anabolic steroids suppressing ocular autoimmune disease.

Keywords: Primary sclerosing cholangitis; uveitis; vasculitis.

Figure 1a

Left eye fundal images showing retinal hemorrhages and cotton wool spots along the superior temporal arcade

Figure 1b

Left eye fundal image showing hemorrhages and cotton wool spots of the superior retinal vasculature extending into the periphery