Autonomic Failure and Proximal Skeletal Myopathy in a Patient With Primary Sjögren Syndrome

Sorajja, Paul; Poirier, Maria K.; Bundrick, John B.; Matteson, Eric L.

doi:10.4065/74.7.695

Cited by 27 publications

(11 citation statements)

References 10 publications

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“…Peripheral neuropathy is an important component of the pathology of Sjogren's syndrome (Sorajja, Poirier et al 1999; Hocevar, Tomsic et al 2003). The trigeminal nerve is one of the most commonly affected cranial nerves in primary Sjogren's syndrome (Gemignani, Marbini et al 1994; Barendregt, van den Bent et al 2001).…”

Section: Neural Regulation Of Lacrimal Gland Function and Its Altmentioning

confidence: 99%

Neural regulation of lacrimal gland secretory processes: Relevance in dry eye diseases

Dartt

2009

Progress in Retinal and Eye Research

399

370

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The lacrimal gland is the major contributor to the aqueous layer of the tear film which consists of water, electrolytes and proteins. The amount and composition of this layer is critical for the health, maintenance, and protection of the cells of the cornea and conjunctiva (the ocular surface). Small changes in the concentration of tear electrolytes have been correlated with dry eye syndrome. While the mechanisms of secretion of water, electrolytes and proteins from the lacrimal gland differ, all three are under tight neural control. This allows for a rapid response to meet the needs of the cells of the ocular surface in response to environmental conditions. The neural response consists of the activation of the afferent sensory nerves in the cornea and conjunctiva to stimulate efferent parasympathetic and sympathetic nerves that innervate the lacrimal gland. Neurotransmitters are released from the stimulated parasympathetic and sympathetic nerves that cause secretion of water, electrolytes, and proteins from the lacrimal gland and onto the ocular surface. This review focuses on the neural regulation of lacrimal gland secretion under normal and dry eye conditions.

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Section: Neural Regulation Of Lacrimal Gland Function and Its Altmentioning

confidence: 99%

Neural regulation of lacrimal gland secretory processes: Relevance in dry eye diseases

Dartt

2009

Progress in Retinal and Eye Research

399

370

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“…In fact, approximately two‐thirds of patients with neuropathy from Sjögren's syndrome first note sensory symptoms in the feet. Primarily small‐fiber dysfunction may sometimes account for the sensory symptoms, and autonomic fibers may also be affected 18, 94. The presentation may be asymmetric 18.…”

Section: Causes Of Small‐fiber Neuropathymentioning

confidence: 99%

Small‐fiber neuropathy

2002

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Small-fiber neuropathy is a common disorder. It is often "idiopathic" and typically presents with painful feet in patients over the age of 60. Autoimmune mechanisms are often suspected, but rarely identified. Known causes of small-fiber neuropathy include diabetes mellitus, amyloidosis, toxins, and inherited sensory and autonomic neuropathies. Occasionally, small-fiber neuropathy is diffuse or multifocal. Depending on the type of small-fiber neuropathy, autonomic dysfunction can be significant or subclinical. Diagnosis is made on the basis of the clinical features, normal nerve conduction studies, and abnormal specialized tests of small-fiber function. These specialized studies include assessment of epidermal nerve fiber density as well as sudomotor, quantitative sensory, and cardiovagal testing. The sensitivities of these tests range from 59-88%. Each has certain advantages and disadvantages, and the tests may be complementary. Unless an underlying disease is identified, treatment is usually directed toward alleviation of neuropathic pain.

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“…Sorajja et al reported on a patient with recent onset primary SS, who developed myopathy and dysautonomia, manifesting as esophageal dysmotility, urinary retention and severe OH. This unusual constellation of symptoms and signs resolved promptly with steroid treatment [13]. Glucocorticoids were reportedly successful in resolving another case of incapacitating autonomic cardiovascular neuropathy that was the presenting complaint in a patient diagnosed as having primary SS [14].…”

Section: Ans Dysfunction In Ss: Preliminary Uncontrolled Studies and mentioning

confidence: 94%

Autonomic Nervous System Dysfunction in Sjogrens Syndrome

Liossis¹

2006

CRR

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Peripheral nervous system dysfunction is well documented in Sjögren's syndrome (SS), whereas central nervous system involvement is a matter of significant controversy. On the other hand, autonomic nervous system (ANS) dysfunction is not even mentioned in extensive reviews of the disease in classic rheumatology textbooks. Despite isolated reports, attention to such involvement in the rheumatologic literature has only been noted over the last six years, following a controlled prospective study that suggested significant autonomic cardiovascular neuropathy in these patients.Since then, few studies, on relatively small numbers of Sjögren's patients, have yielded conflicting results, including two that claim no abnormality in autonomic nervous system function. A variety of factors may be blamed for these discrepancies, including bias on patient selection but mainly differences in employed methodology, i.e. conventional cardiovascular reflex tests and baroreflex sensitivity vs 24 hour heart rate variability. In any case, besides isolated case reports describing severe autonomic dysfunction, usually manifested by postural hypotension, the majority of studies that indicate the presence of ANS abnormalities in SS, have detected that only by applying objective tests and specific questionnaires. On the other hand, ANS dysfunction, implying defective innervation of exocrine glands, and the presence of muscarinic receptor antibodies have been blamed, partially at least, for the decreased secretory function of exocrine glands in this disease.More importantly, ANS dysfunction has been reported to result in increased mortality in diabetics, post myocardial infarction patients and the elderly. If such a possibility exists for patients with SS, the need for prospective, controlled and long follow up studies on large cohorts is imperative.

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Autonomic Failure and Proximal Skeletal Myopathy in a Patient With Primary Sjögren Syndrome

Cited by 27 publications

References 10 publications

Neural regulation of lacrimal gland secretory processes: Relevance in dry eye diseases

Neural regulation of lacrimal gland secretory processes: Relevance in dry eye diseases

Small‐fiber neuropathy

Autonomic Nervous System Dysfunction in Sjogrens Syndrome

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