Ross syndrome on a background of Sjogren’s syndrome

<h3></h3> We report the case of a 60 year old woman seen in the autonomic clinic. She had previously been diagnosed with Sjogren’s syndrome, limited cutaneous systemic sclerosis, mild hypermobility, and a longstanding sensory peripheral neuropathy. Her autonomic symptoms started aged 45 when her optician identified asymmetrical pupils and she was diagnosed with Holmes-Adie syndrome. Her autonomic symptoms include heat intolerance, increased sweating on the head and trunk and reduced sweating in the limbs. <h3>Examination and Investigations</h3> Physical examination revealed hyporeflexia and small, irregularly shaped pupils with light- near dissociation and abnormal dilation with apraclonidine (Adie’s pupil). A sweat test revealed generalized anhidrosis in the limbs. Pupillometry assessments indicated bilateral and symmetric parasympathetic and sympathetic paresis, suggestive of ciliary ganglionopathy. <h3>Diagnosis and Discussion</h3> The combined clinical picture of tonic pupils, hyporeflexia and anhidrosis suggest a unique syndrome known as Ross syndrome. The pathogenesis of this condition is not known. However, given that Sjogren’s syndrome can cause an autoimmune ganglionopathy, and the presence of Holmes-Adie syndrome has been well reported in this condition, we propose that in this case it may have an autoimmune basis linked to her Sjogren’s syndrome.