Long-term management of recurrent uveitis associated with autoimmune lymphoproliferative syndrome.

Abstract

Background: The purpose of this case report is to describe the long-term findings and management of recurrent uveitis in a patient with autoimmune lymphoproliferative syndrome (ALPS).

Case observation: A 25-year-old female with a history of ALPS and previous uveitis flares presented with reduced vision and pain in the left eye. Uveitis flares began at age 4 and continued into adulthood prompting multiple medical regimen changes. Ocular involvement included anterior uveitis, posterior uveitis, and panuveitis. The laterality of uveitis flares favored the left eye, however bilateral episodes were documented. The patient was diagnosed with recurrent anterior uveitis and treated with topical corticosteroids with subsequent resolution of her symptoms. However, the patient continued to develop recurrent uveitis prompting low-dose long-term maintenance treatment with topical corticosteroids. Additionally, methotrexate was added which led to uveitis resolution and inactive disease for over a year on this treatment regimen.

Conclusions: Uveitis secondary to chronic autoimmune disease, such as ALPS, offer long-term challenges for ocular health management. Due to the recurrent nature of uveitis flares in patients with ALPS, long-term ophthalmic and systemic management may be warranted with low-dose maintenance topical corticosteroid or systemic immunomodulation. Response to topical and systemic therapy can be highly patient specific. Complex side effect profiles of many systemic immunotherapies make drug tolerability an important treatment consideration. Multidisciplinary coordination of systemic immunosuppression should be tailored to the patient's anti-inflammatory needs, as well as careful monitoring of adverse events.