Hyperadrenergic Orthostatic Hypotension With Pure Peripheral Sympathetic Denervation Associated With Sjogren's Syndrome.

Hyperadrenergic orthostatic hypertension (hyper OH), defined as OH with elevated levels of upright plasma norepinephrine (NE), is assumed to be a clinical expression of peripheral sympathetic denervation (PSD). Primary Sjogren's syndrome (pSS) is an autoimmune disease that not only affects the exocrine glands but also develops autonomic neuropathy with PSD. We present a hyper OH with PSD possibly associated with pSS and successfully treated with intravenous immunoglobulin (IVIg). The case was a 60-year-old man who developed recurrent syncope on rapid standing from a sitting position. Head-up tilt test and NE infusion test showed hyper OH with PSD. This case report raises the possible associations between hyper OH and autonomic neuropathy as pSS developed.