Cardiovascular autonomic dysfunction in primary Sjogren's syndrome

Kovács, László; Paprika, Dóra; Takács, Róbert; Kardos, Attila; Várkonyi, Tamás; Lengyel, Csaba; Kovács, Attila; Rudas, László; Pokorny, G.

doi:10.1093/rheumatology/keg468

Cited by 42 publications

(25 citation statements)

References 21 publications

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“…Recently, cardiovascular autonomic dysfunction in primary SS has been reported (46). Therefore, most likely in JHS as well as in primary SS, the decreased stimulation by the ANS of the salivary and lacrimal glands would produce xerophthalmia and xerostomia.…”

Section: Discussionmentioning

confidence: 99%

Clinical study of hereditary disorders of connective tissues in a Chilean population

Bravo¹,

Wolff²

2006

Arthritis & Rheumatism

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Objective. To demonstrate the high frequency and lack of diagnosis of joint hypermobility syndrome (JHS) and the seriousness of vascular Ehlers-Danlos syndrome (VEDS).Methods. Two hundred forty-nine Chilean patients with hereditary disorders of the connective tissues (CTDs) and 64 control subjects were evaluated for the diagnoses of JHS and VEDS using the validated Brighton criteria, as compared with the traditional Beighton score. In addition, the presence of blue sclera was determined, with the degree of intensity graded as mild, moderate, or marked.Results. The frequency of hereditary CTDs was 35%, with diagnoses of JHS in 92.4% of subjects, VEDS in 7.2%, and osteogenesis imperfecta in 0.4%. The Beighton score proved to be insufficient for the diagnosis of JHS (35% of subjects had a negative score), whereas the Brighton criteria yielded positive findings (a diagnosis of JHS) in 39% of control subjects. Blue sclera was frequent, being identified in 97% of JHS patients and 94% of VEDS patients. Moderate osteopenia/osteoporosis was observed in 50% of patients with VEDS and 26% of those with JHS. Dysautonomia, dyslipidemia, and scoliosis were more frequent in VEDS patients than in JHS patients. The typical JHS facial appearance and the "hand holding the head sign" were identified. Raynaud's phenomenon was extremely rare in JHS patients (2%). Ruptured uterus and cerebral aneurysm occurred in 12% and 6% of VEDS patients, respectively. Spontaneous pneumothorax was more frequent in VEDS patients (11%) than in JHS patients (0.9%).Conclusion. JHS is very frequent but usually undiagnosed. The Beighton score is an insufficient method for JHS diagnosis. We recommend that physicians learn to recognize the typical facial features of JHS and be able to identify blue sclera. We also propose that validated hypermobility criteria be routinely used. Further research is needed to determine why the prevalence of JHS is so high in Chile.Hippocrates first described hypermobile joints 2,400 years ago, but the condition of joint hypermobility was not described as a medical problem until 1967, by Kirk et al (1). Although many people have hypermobile joints, the general public as well as many physicians consider hypermobility to be a curiosity rather than a potentially serious medical problem (2,3). The prevalence of hypermobility is difficult to evaluate because it varies with age, sex, and ethnic background and because multiple criteria are being used. It is more frequent in female subjects and children, is more frequent in Asian than in black populations, and is more frequent in both Asian and black populations than in whites. It is generally agreed that it exists in ϳ10% of individuals in Western populations (4) and up to 25% in Iraqis (5). Most cases of joint hypermobility are pauciarticular rather than generalized, which makes the diagnosis more difficult. Because of this, many people are unaware of having lax joints (6). In spite of this laxity in the joints, most people with hypermobility do not experience joint or musculoskelet...

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Section: Discussionmentioning

confidence: 99%

Clinical study of hereditary disorders of connective tissues in a Chilean population

Bravo¹,

Wolff²

2006

Arthritis & Rheumatism

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“…Rarely, autonomic neuropathy can be the presenting symptom of PSS [21,22]. In one series, cardiovascular dysautonomia was observed in the majority of PSS patients [23]. In another series, 50% of patients had autonomic symptoms and 69% had autonomic signs on testing [24].…”

Section: Autonomic Neuropathymentioning

confidence: 95%

“…Previous studies have shown a wide variation in the prevalence of sensorimotor polyneuropathy -6 [3 ], 23 [2], and 68% [15], although the motor fiber involvement in some of these studies may be ascribed to subclinical abnormalities seen on needle electromyography (EMG) or motor nerve conduction testing. The same is true for the high prevalence of pure motor neuropathy reported in other studies [5,16].…”

Section: Sensorimotor Polyneuropathy and Polyradiculopathymentioning

confidence: 99%

Neurological manifestations of primary Sjogren's syndrome

Chai

Logigian

2010

Current Opinion in Neurology

136

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“…This is of clinical relevance because the diagnosis of small fibre neuropathy can usually only be proven by skin biopsies that show an increased intraepidermal nerve fiber density [47]. Most studies, with few exceptions [48], also depict an increased prevalence of autonomic symptoms in Sjögren's syndrome patients [46,[49][50][51].…”

Section: Sjö Gren's Syndromementioning

confidence: 99%