A case of pan-dysautonomia associated with systemic lupus erythematosus / 대한내과학회지

ABSTRACT

Autonomic neuropathy can occur as a neurologic manifestation in patients with systemic lupus erythematosus (SLE). Its precise prevalence and pathogenesis were not fully evaluated. Recent studies reported that about half of patients with SLE had autonomic neuropathy. Autonomic neuropathies include cardiovascular, gastrointestinal, genitourinary, sudomotor, lacrimal, and pupillary dysfunction. Autonomic nerve dysfunction significantly affects clinical course of the disease, and especially cardiovascular autonomic dysfunction may cause arrhythmias increasing the risk of sudden death. Pan-dysautonomia has been rarely reported as a neurologic complication of SLE. We experienced a patient with SLE presenting pan-dysautonomic manifestations. A 23-year-old man was admitted due to dizziness and syncopal attack. He complained various autonomic symptoms, such as orthostatic syncope, dysphagia, severe constipation, indigestion, and anhidrosis. Autonomic nerve function tests and the clinical manifestations revealed that he had pan-autonomic dysfunction. During hospitalization, respiratory and cardiac arrest developed soon after syncopal attack. He recovered after prompt cardiopulmonary resuscitation. But his autonomic dysfunctions improved slightly after 7 months of therapy. Early detection and aggressive treatment are needed to prevent potentially fatal dysautonomic attack in patient with SLE.