Appendix A: Case Definitions for Neuropsychiatric Syndromes in Systemic Lupus Erythematosus

Autonomic Disorder

Disorder of the autonomic nervous system with orthostatic hypotension, sphincteric erectile/ejaculatory dysfunction, anhidrosis, heat intolerance, constipation.

Diagnostic criteria:

Symptoms and abnormal response to provocative tests:

Test Normal range

• Blood pressure response to standing Fall in BP £ 30/15 mm Hg
  or vertical tilt (systolic/diastolic)
• Heart rate response to standing Increase 11-29 beats/minute
• Heart rate variation with respiration Max. - min. heart rate
  ³15 beats/min.; E:I ratio ³1.2
• Valsalva ratio³ 1.4
• Sweat test Sweating over all body and limbs

E:I ratio = ratio of heart rate during expiration and inspiration

Exclusions:

• Autonomic dysfunction with Lambert-Eaton syndrome
• Medications: tricyclic antidepressants
• Poisons: organophosphates
• Shy-Drager syndrome

Associations:

• Diabetic neuropathy and peripheral neuropathy of other causes
• Autonomic failure in elderly

Ascertainment:

History, physical examination, and provocative tests

Record:

• Basic descriptors
• Type of disturbance

(References: 23, 28, 29, 114-118)

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