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

Purpose: To present a novel association of multiple autoimmune syndrome (MAS) in a patient with sclerouveitis and alkaptonuria.Case report: 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 Sjogren'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.Conclusion: We present a novel MAS-type 2 pattern consisting of Hashimoto's thyroiditis, sclerouveitis, and Sjogren's syndrome. Its diagnosis and management represent a challenge, so a multidisciplinary approach should be provided.

Automated summary

This case report presents a patient with sclerouveitis and alkaptonuria who developed multiple autoimmune syndrome (MAS) including Hashimoto's thyroiditis and Sjogren's syndrome. The patient had ocular symptoms such as decreased visual acuity, red eye, foreign body sensation, and ocular pain. Ophthalmological examination revealed conjunctival hyperemia, ochronosis, scleral nodule, keratic precipitates, anterior chamber cells, vitreous cells, and vitreous haze. In addition to immunomodulatory medications, biological treatment was suggested due to refractory response.