Autoimmune-Related Retinopathy Presenting As Plaquenil Toxicity in Patients With Systemic Lupus Erythematosus

Abstract

Purpose: To report 3 cases of autoimmune retinopathy (AIR) in patients with systemic lupus erythematosus (SLE) to explore the association between these conditions and highlight additional clinical consideration of AIR in patients presenting with atypical retinopathy in the context of hydroxychloroquine use. Methods: The medical and clinical follow-up records of 3 clinical cases were reviewed. The eligibility criteria were the absence of other retinopathy or systemic autoimmune diseases. Results: All patients had a long-standing diagnosis of SLE and had been taking hydroxychloroquine at a dose exceeding the American Academy of Ophthalmology recommendations. All 3 patients had extensive retinal degeneration atypical in appearance for drug toxicity alone. Examination, imaging, electroretinograms, and autoantibody assays eventually led to the diagnosis of AIR. Conclusions: Further study of the AIR and SLE may reveal an association between these conditions. In patients with SLE presenting with retinal degeneration, AIR may be underdiagnosed.

From the Editor-in-Chief

“…Ma et al 8 begin our case-focused manuscripts, describing a small case series of patients masquerading as Plaquenil (hydroxychloroquine sulfate) toxicity in the setting of systemic lupus erythematosus on high-dose oral hydroxychloroquine usage. Like virtually all of our rare diseases, a high threshold of suspicion is necessary to establish the correct diagnosis and guide appropriate treatment.…”