What is your diagnosis?
A 50-year-old woman with lupus presents with slowly decreasing vision in both eyes.
The diagnosis is...
The image is consistent with bull's-eye maculopathy, for which the differential diagnosis includes hydroxychloroquine retinopathy:
- Hydroxychloroquine is a medication used to treat many dermatologic and rheumatologic conditions.
- Long duration of use (>5 years) and high dosage (>5 mg/kg of body weight daily) can damage macular rods and cones and lead to the classic appearance of the bull's eye in the macula.
- Cases of retinopathy tend to be bilateral, symmetric, and irreversible, so early, frequent screening and early detection are important to prevent sequelae.
- The differential diagnosis of bull's-eye maculopathy includes diseases such as age-related macular degeneration, cone dystrophy, cone-rod dystrophy, and Stargardt disease.
What is the role of the primary care or emergency medicine physician?
Patients who develop symptoms of hydroxychloroquine retinopathy should be referred urgently to follow-up with their ophthalmologist.
What is the role of the ophthalmologist?
The ophthalmologist should offer counseling to patients about the risk of toxicity and the importance of regular screening. Patients who are on hydroxychloroquine should be screened by an ophthalmologist as follows:
- Screening should occur within 1 year of starting hydroxychloroquine, and it should be performed annually after 5 years of use.
- Patients with certain risk factors (eg, excessive dosing, kidney or liver disease, macular disease, older age) should be screened more often.
Screening, at minimum, includes the following:
What is the treatment?
If hydroxychloroquine retinopathy is detected, the medication should be discontinued immediately, and the patient's rheumatologist should be involved to determine alternative treatments for systemic disease.
No treatments are currently available to reverse the retinopathy. Retinopathy progression may continue following cessation of the drug, so the ophthalmologist should consider monitoring the patient closely.
Learn more: Ophthalmology resources for medical students