[A case of Sjögren syndrome associated with multiple mononeuritis and dysautonomia including bilateral tonic pupils]

Sjögren syndrome (SjS) is a glandular disease characterized by dry eyes and dry mouth. Extraglandular manifestations in SjS are also common, and peripheral nerve involvement has been reported in 10-20% of cases. We report a case of Sjögren syndrome with bilateral tonic pupils, dysautonomia, and multiple mononeuritis. The fact that sural nerve sections, in addition to marked loss of myelinated and unmyelinated fibers, showed an increased number of infiltrating macrophages without lymphocytes and aberrant expression of HLA-DR (class II) antigen in Schwann cells was an especially interesting finding. No evidence of active vasculitis was detected. The patient was treated with corticosteroids and her condition gradually improved, as confirmed by thermography. Our findings suggested the presence of specific immunological abnormalities simultaneously involving the ciliary ganglia, autonomic ganglia, and dorsal root ganglia in this peculiar form of SjS.