Sclerouveitis as Part of Multiple Autoimmune Syndrome in a Patient with Alkaptonuria: A Case Report

To present a novel association of multiple autoimmune syndrome (MAS) in a patient with sclerouveitis and alkaptonuria.

A 68-year-old female with alkaptonuria, Hashimoto's thyroiditis, and familial autoimmunity presented with decreased VA, red eye, foreign body sensation, and ocular pain. Ophthalmological examination: OD conjunctival hyperemia, ochronosis, a reddish-violet scleral nodule, keratic precipitates, 2+ cells in the anterior chamber, 0.5+ vitreous cells, and mild vitreous haze. The patient was diagnosed with anterior uveitis and anterior nodular scleritis. Due to the associated sicca symptoms, a salivary gland biopsy was ordered, confirming Sjögren's syndrome. Then, MAS was diagnosed, and immunomodulatory medications were started; however, as she was refractory to more than two of them, it was suggested to start biological treatment.

We present a novel MAS-type 2 pattern consisting of Hashimoto's thyroiditis, sclerouveitis, and Sjögren's syndrome. Its diagnosis and management represent a challenge, so a multidisciplinary approach should be provided.