may later develop sensory ataxic neuropathy [46]. Sympathetic skin response, quantitative sensory testing, quantitative sudomotor axon reflex testing, and sensory evoked potentials are some of the methods used to assess small fiber function [49], but they are technically complex and not widely available. Skin biopsy, a simple outpatient procedure, is now used to assess epidermal nerve fiber density based on standardized methods of objective and reproducible quantification. Skin biopsy is the best and easiest method to diagnose small fiber neuropathy [24, 45]. We recommend it to establish the diagnosis and to exclude the possibility of psychosomatic complaints. Sural nerve biopsy, on the other hand, is not diagnostic and we do not recommend it [17, 46].

Treatment for small fiber neuropathy is mainly symptomatic, as described above. Of interest is a small uncontrolled study, which showed decrease of pain after IVIg [50]. However, the findings of this study need to be validated by further randomized controlled trials involving larger number of patients.

21.7Restless Leg Syndrome

Restless legs syndrome is a constellation of symptoms [51], the most important being an unpleasant sensation of the legs, which is relieved by movement and exacerbated by limb immobilization. Symptoms typically follow a circadian pattern of fluctuation, being more intense when at rest or falling asleep during the night. The symptoms result in sleep loss, disruption of normal activities, and depression. Iron deficiency, end-stage renal disease, and pregnancy are common causes of restless leg syndrome that should be excluded before the condition is attributed to Sjögren’s syndrome. The physical examination and laboratory evaluation are generally normal in this setting. Dopamine agonists, gabapentin, and the opiates methadone and oxycodone have been effective in the treatment of restless leg syndrome [52]. In a study focusing on sleep disorders, restless leg syndrome was more prevalent in patients with Sjögren’s syndrome compared to rheumatoid arthritis or healthy controls [53]; however, this association requires confirmation. In our experience, restless leg syndrome is an overlooked symptom in Sjögren’s syndrome patients and should be considered because the treatment is rewarding. Our experience suggests that dopamine agonists should be the treatment of choice.

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Chapter 22

Autonomic Neuropathy

Thomas Mandl and Lennart Jacobsson

Contents

22.1Introduction

Autonomic dysfunction (AD) is a feature of many different chronic diseases such as type I and II diabetes, rheumatoid arthritis, systemic lupus erythematosus, scleroderma, and inflammatory bowel disease. Patients with primary Sjögren’s syndrome (pSS) may also show various symptoms of impaired autonomic nervous function such as orthostatic intolerance [1]. The use of autonomic reflex tests (ARTs) in pSS has demonstrated evidence of parasympathetic and sympathetic dysfunction [2–6]. In contrast, investigations of heart rate variability and baroreflex sensitivity in pSS have yielded contradictory results [6–9] (Table 22.1). A variety of AD symptoms have been reported in pSS patients [9, 15]. However, because the degree of exocrine

T. Mandl (*) • L. Jacobsson

Department of Rheumatology, Skane University Hospital, Malmö, Sweden