Autonomic neuropathy in systemic sclerosis: a case report and evaluation of six patients.

Abstract

SUMMARY We describe a case of systemic sclerosis with sympathetic and parasympathetic neuropathy and detail autonomic testing in six further patients. Of these six patients, three showed early parasympathetic damage. None of the patients had evidence of peripheral neuropathy, and there was no correlation between the presence of autonomic dysfunction and the severity of Raynaud's phenomenon.Autonomic neuropathy has not previously been reported in systemic sclerosis to our knowledge. Autonomic neuropathy has been described in rheumatoid arthritis1 2 and systemic lupus erythematosus,3 and impotence was recently described in systemic sclerosis4 but without formal studies of autonomic function. Other neurological manifestations of systemic sclerosis have been described but are rare. A mild peripheral neuropathy has been reported.5 6 as has trigeminal neuralgia.7-9Subacute combined degeneration of the cord following vitamin B12 deficiency secondary to sclerodermatous involvement of the small intestine has also been recorded.10We describe a patient with systemic sclerosis and autonomic neuropathy and detail our investigation of the autonomic nervous system in six other patients with scleroderma and eight control patients.

Transcutaneous Electrical Nerve Stimulation (TENS) Improves Upper GI Symptoms and Balances the Sympathovagal Activity in Scleroderma Patients

“…The exact etiology of SSc is unknown but most studies support the hypothesis that it is an underlying vascular or vasospastic disorder, with a strong neuropathic influence. Autonomic dysfunction is believed to play a role in the pathogenesis of the disease, increasing both GI and cardiovascular morbidity [4].…”

Autonomic nervous dysfunction in systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA): possible pathogenic role of autoantibodies to autonomic nervous structures

“…Autonomic neuropathy has been described, though less frequently than peripheral somatic neuropathy, in connective tissue diseases, such as SLE [1][2][3], RA [4][5][6] and systemic sclerosis [7][8][9]. Reports on the prevalence of autonomic dysfunction are discordant, ranging from 0% to over 90% according to different authors.…”

Bladder Involvement in Systemic Sclerosis: Urodynamic and Histological Evaluation in 23 Patients

“…Certain features, particularly those related to the gastrointestinal system, such as abnormal esophageal motility, constipation, diarrhea and Raynaud’s phenomenon, have suggested an autonomic dysfunction [5, 6, 19], but some investigators disagree with regard to the results [20]. …”

“…Although neurologic manifestations in SSc have been previously considered uncommon, more recent observations indicate a much higher prevalence of neurologic manifestations (about 40%) that may involve all levels of the neuraxis [4] and the autonomic nervous system [5, 6]. …”