Purpose: To report a case of uveitis and papillitis in a patient with cryopyrin-associated periodic syndrome.
Methods: Case report. A 44-year-old white woman with a long history of inflammatory arthritis, diffuse erythematous rashes and hives, and hearing loss was referred to the Massachusetts Eye Research and Surgery Institution for an evaluation of chronic ocular inflammation. She was diagnosed with bilateral papillitis and uveitis after a comprehensive eye examination, which included dilated fundoscopy, optic nerve ocular coherence tomography, fluorescein angiography, indocyanine green angiography, and B-scan ultrasonography. She was later diagnosed with cryopyrin-associated periodic syndrome, an interleukin-1-driven autoimmune disease, as confirmed by genetic testing. Soon after starting treatment with anakinra, a human interleukin-1 receptor antagonist, she experienced rapid improvement of her ocular and systemic symptoms, including rash, uveitis, and arthritis.
Results: Cryopyrin-associated periodic syndrome-associated ocular inflammation in remission with anakinra 100-mg daily subcutaneous injection.
Conclusion: Anakinra is a very effective treatment for both cryopyrin-associated periodic syndrome and cryopyrin-associated periodic syndrome-associated ocular inflammation.