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A 46 year-old female presented with dry eyes and a dry mouth which she had been experiencing for about 15 years. She also began to notice dizziness (orthostatic hypotension) during the last 5 years. The symptoms gradually increased whereupon she began to have polyarthralgia, facial flashing, hyper and hyposweating in some areas on the face and trunk. Her sialography showed a damaged parotid gland. Minor salivary gland biopsy revealed chronic sialoadenitis. The Sirmer test was low, and the Rosebengal test indicated keratoconjunctivitis sicca. Her serological tests showed hypocomplementemia and were positive for antinuclear antibody and SS-A antibody. The diagnosis of primary Sjögren's syndrome (SjS) was made based on these findings. Prednisolone, at a dose of 15 mg per day, was given orally. As a result of this therapy, arthralgia disappeared immediately, although it had no effect on the neuropathy found in this patient. This is a rare case of SjS associated with peripheral neuropathy and severe dysautonomia.

起立性低血圧，発汗異常，Ａｄｉｅ瞳孔などの自律神経障害を伴った原発性シェーグレン症候群の１例