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Primary Sjogren's syndrome presenting with generalized autonomic failure
Author(s) -
Sakakibara R.,
Hirano S.,
Asahina M.,
Sawai S.,
Nemoto Y.,
Hiraga A.,
Uchiyama T.,
Hattori T.
Publication year - 2004
Publication title -
european journal of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.881
H-Index - 124
eISSN - 1468-1331
pISSN - 1351-5101
DOI - 10.1111/j.1468-1331.2004.00846.x
Subject(s) - medicine , pure autonomic failure , orthostatic vital signs , fibromyalgia , cardiology , physical examination , blood pressure , dermatology
A 64 year‐old woman developed Raynaud's phenomenon and dry eyes/mouth. Laboratory examination revealed positive Schirmer's test, rheumatoid factor and anti‐nuclear antibody, and lymphocytic sialoadenitis on salivary gland biopsy. These features strongly suggested the diagnosis of primary Sjogren's syndrome. Three years later, she gradually developed generalized autonomic failure without apparent sensory neuropathy on nerve conduction study. She had systolic pressure fall of 51 mmHg on head‐up tilt test, cardiovascular supersensitivity to diluted norepinephrine infusion, cardiac denervation in [123I]‐MIBG scintigraphy, impaired R‐R variability, decreased sweating and prolonged colonic transit time. Autoimmune autonomic ganglionopathy was mostly responsible for her autonomic failure.