Purpose: The aim of this study was to present a patient with systemic lupus erythematosus on longstanding hydroxychloroquine (HCQ) use for whom HCQ was stopped because of signs of toxicity and then resumed four years later because of dire systemic need.
Methods: This is a long-term retrospective study. Humphrey visual fields (10-2 and 24-2), fundus autofluorescence imaging, and spectral domain optical coherence tomography (OCT) were used to follow progression over time.
Results: The patient was on HCQ for 26 years, with a cumulative dose over 3,000 g. HCQ was stopped in 2011 because of macular toxicity. She remained off HCQ for four years, during which time she developed type 1 diabetes due to an immunologic attack on the pancreas and then JC (John Cunningham) viremia after a period of treatment with mycophenolate, which put her at risk for progressive multifocal leukoencephalopathy. Mycophenolate was discontinued, and HCQ was resumed with careful follow-up over the next 7 years. The toxic maculopathy showed only mild slow progression since HCQ was resumed.
Conclusion: Careful annual monitoring using Humphrey visual field 10-2 and spectral domain OCT imaging remains the standard of care for the patients on HCQ. However, it may be possible with close monitoring when there is compelling systemic need to resume HCQ after it has been stopped, with only slow progression of the retinopathy. This allowed the patient to have an improved quality of life and reduced the risk of severe morbidity and mortality.