The first case of a patient with bilateral retinal vasculitis associated with cold agglutinin disease (CAD) has been reported. The case, published in the American Journal of Ophthalmological Case Reports, notes the patient was successfully treated with obinutuzumab and infliximab.

“To the best of our knowledge, there are no published reports of bilateral retinal vasculitis in CAD patients,” the authors wrote. “We herein report the clinical course and management of bilateral retinal vasculitis associated with CAD that demonstrated minimal response to anti-CD20 monoclonal antibody obinutuzumab and significant improvement following treatment with intravenous [IV] anti-tumor necrosis factor [TNF] infliximab and methylprednisolone.”

The patient was a 69-year-old Hispanic woman with worsening blurred vision. She had a history of epiretinal membranes in both eyes, cholecystectomy, oophorectomy, nonspecific joint disease, and pulmonary symptoms. She was being treated with low doses of prednisone and methotrexate.


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The initial studies performed revealed positive antinuclear antibody of 1:80, leukocytosis, and reduced hemoglobin. She was administered 1000 mg mycophenolate mofetil twice per day and 20 mg prednisone daily. She showed little improvement, and additional tests revealed CAD. A bone marrow evaluation showed chronic lymphocytic leukemia-like monoclonal B-cell lymphocytosis, and she was started on therapy with obinutuzumab in weekly infusions.

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After 8 cycles, her blood tests had returned mostly to normal, but after 6 months she still had diffuse perivascular leakage. Her medical team switched her infusions to infliximab and methylprednisolone, and after 2 cycles her fluorescein angiography showed significant improvement.

The authors are uncertain whether the CAD caused the patient’s retinal vasculitis. They noted that treatment with infliximab and anti-TNF agents such as methylprednisolone may be required to treat patients with ocular manifestations of CAD. They recommend further studies on possible associations between CAD and retinal vasculitis to improve patient management.

Reference

Tuong Than NT, Yasar C, Huy Pham B, et al. Bilateral retinal vasculitis associated with cold agglutinin disease treated with obinutuzumab and infliximab. Am J Ophthal Case Rep. December 2022. doi:10.1016/j.ajoc.2022.101752