Research in Review

Neurally mediated hypotension in systemic lupus erythematosus patients with fibromyalgia.

Rheumatology ( Oxford ). 2004 Feb 24

Tang S, Calkins H, Petri M.

Department of Medicine & Geriatrics, Princess Margaret Hospital, Hong Kong .

OBJECTIVE: Fibromyalgia (FM) is a major determinant of poor health status in systemic lupus erythematosus (SLE). FM has been shown to be associated with neurally mediated hypotension (NMH) in the general population, in which effective treatments exist. We explored whether NMH was more common in SLE patients with FM than those without.

METHODS: Seventy-six SLE patients (4 male, 72 female; 1 ethnic Indian, 28 African American, 47 Caucasian; mean age 40.2 +/- 9.4 yr) were recruited and their FM status ascertained using American College of Rheumatology (ACR) classification criteria. Patients who were pregnant or deconditioned were excluded. A two-stage tilt-table test was used to detect NMH. All patients completed the SF-36 Heath Status Inventory (SF-36) and the Krupp Fatigue Severity Scale (KFSS) to evaluate their quality of life. Serological markers and the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) were recorded. Medications that might interfere with testing were temporarily withheld before the tilt-table test.

RESULTS: The prevalence of NMH (first stage) in SLE patients was 47.9%. Seven patients had both NMH and postural orthostatic tachycardia syndrome. Two patients had borderline results with symptoms reproduced but an inadequate drop in systolic blood pressure by definition (a drop of at least 25 mmHg defines an abnormal response). Eighteen (23.7%) SLE patients had FM and 51 (67.1%) had at least one tender point (TP). The frequency of NMH (first or second stage) in SLE with FM was 58.3% compared with 69.4% in SLE without FM (odds ratio 0.62, 95% CI 0.16-2.37). SF-36 and KFSS scores were not significantly different in SLE patients with and without NMH. However, both scores were found to be associated with FM status (P<0.001 and P = 0.014), reflecting poor health status in the FM group. No confounding variable was found to be significantly associated with both NMH and FM.

CONCLUSION: NMH is common in SLE patients with a high prevalence rate. The large increase in NMH, a form of autonomic neuropathy, in SLE, has not been explained. However, NMH has no impact on quality of life above that determined by FM, and has no statistically significant association with FM status. Identification of NMH may be important in selected patients with SLE who have chronic fatigue, but NMH cannot explain the increased prevalence of FM in SLE.

Pacing for Vasovagal Syncope After the Second Vasovagal Pacemaker Study (VPS II): A Matter of Judgement.

Card Electrophysiol Rev. 2003 Dec;7(4):416-20.

Trim GM, Krahn AD, Klein GJ, Skanes AC, Yee R.

Division of Cardiology, University of Western Ontario, London, Ontario, Canada.

Vasovagal or neurocardiogenic syncope is a common benign condition. In the majority of patients it regresses naturally, or can be controlled by conservative therapy. However there is a group of patients who remain severely affected despite lifestyle measures, counselling and medication. Pacing has been considered in these patients as a result of logic, observational studies, and three randomised but unblinded studies, VPS, VASIS and SYDIT. A randomised and blinded study, VPS II, was recently published, the results of which undermined the results of these preceding studies: despite a 30% trend towards reduced syncope in patients with active pacing, the result was not statistically significant. This left clinicians with a dilemma, whether or not to pace in patients with disabling syncope despite conservative therapy. We believe, based on a review of all currently available evidence, that there remains a role for pacing in the patient with evidence of significant cardioinhibition, particularly severe bradycardia or asystole, and ongoing disabling syncope in spite of conservative measures. When to pace in these patients is a matter of clinical judgement. The threshold for pacing should remain high, however, with extensive attempts of conservative and pharmacological measures and with appropriate discussions with patients prior to instituting pacing, regarding the risks and long-term implications of a pacemaker. More needs to be learned about optimal pacing modalities.

Autonomic failure in neurodegenerative disorders.

Semin Neurol. 2003 Dec;23(4):351-63.

Kaufmann H, Biaggioni I.

Department of Neurology, Mount Sinai School of Medicine, New York, New York 10029, USA .

Autonomic failure with orthostatic and postprandial hypotension, bowel and bladder disturbances, and sexual dysfunction are frequent, disabling features in patients with the three most prevalent neurodegenerative movement disorders: Parkinson's disease (PD), dementia with Lewy bodies and multiple system atrophy (MSA), and the related neurodegenerative Lewy-body disorder characterized by isolated severe autonomic failure (pure autonomic failure, PAF). All of these disorders have in common the presence of alpha-synuclein in the cytoplasmic precipitates found in neurons in Lewy body disorders or glia in MSA. Autonomic failure with disabling orthostatic hypotension is the clinical hallmark of PAF. It may also be the initial presentation of MSA, making diagnosis difficult. Within a few years, however, MSA patients develop movement disorders, which are differentiated from PD by the paucity of unilateral resting tremor, the lack of response to levodopa, and their rapidly progressive nature, resulting in disability and death in 7 to 8 years. Moderately effective treatment is available for autonomic symptoms, but management of movement disorders remains unsuccessful. Discoveries relevant to physiology and common pathological conditions were initially made in patients with autonomic failure. Meals induce profound hypotension in these patients. Conversely, commonly used nasal decongestants can produce substantial pressor effects. Even 500 mL of water can increase blood pressure by a previously unrecognized sympathetic reflex. Residual sympathetic tone is able to induce sustained supine hypertension in MSA, because it is resolved after ganglionic blockade. These phenomena were not previously recognized because of the buffering capacity of the baroreflex, but were unmasked in autonomic failure patients.

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