Autonomic dysfunction in primary Sjögren's syndrome

Abstract: The aims of this work were to evaluate objective and subjective signs of autonomic dysfunction (AD) in patients with primary Sjögren’s syndrome (pSS) and to evaluate its clinical associations. In study I, 46 pSS patients and 56/80/238 autonomic nervous function test (ANT) controls participated. Objective signs of AD were evaluated using three different cardiovascular ANTs and exocrine function in pSS patients by the Schirmer I test, rose bengal staining, and unstimulated whole sialometry. pSS patients showed objective signs of a parasympathetic and sympathetic dysfunction and also an abnormal orthostatic blood pressure reaction. However, there was a poor association between cardiovascular autonomic and exocrine function tests. Study II comprised 31 patients with type I diabetes and 200 population-based controls. The Autonomic Symptom Profile (ASP), a questionnaire assessing autonomic symptoms, was translated into Swedish. The various scores were age-, sex-, height-, and weight-standardized and the reliability and validity of the ASP were assessed and considered acceptable or good. In study III, 38 pSS patients and 200 population-based controls participated. Subjective signs of AD were evaluated using the ASP. The pSS patients were found to have symptoms of both parasympathetic and sympathetic dysfunction. AD symptoms were, however, poorly associated with objective signs of AD and other clinical features of the disease. Study IV involved 20 consecutive pSS patients and 30 age- and sex-matched population-based controls. All study subjects were evaluated with a questionnaire on pharyngeal and esophageal symptoms and video-radiography and, in addition, pSS patients with two ANTs. Dysphagia and pharyngeal and esophageal symptoms were more common in pSS patients than in controls while objective signs of pharyngeal and esophageal dysmotility were not. Dysphagia in pSS patients was not related to video-radiographical signs of dysmotility but it was associated with objective signs of parasympathetic dysfunction.