Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by multisystemic manifestations. Central nervous system (CNS) involvement has been reported to occur in 6-95% of SLE patients, depending on the criteria applied,1,2 however, the exact prevalence is probably between 6% and 12%.3
Autonomic disorder is one of the 19 clinical syndromes in the American College of Rheumatology (ACR) classification of neuropsychiatric syndrome of systemic lupus erythematosus (NPSLE).4 The reported prevalence of autonomic neuropathy in SLE varies from no difference compared with controls5 to a high prevalence6-8 based on small case series and case-control studies. The majority of patients in these studies had mild subclinical dysautonomia in the presence of one or more abnormal cardiovascular reflex tests.7 Reports on SLE with acute pan-dysautonomia are rare. In addition to neurological diseases, such as Shy-Drager and Guillain-Barre syndromes, autonomic neuropathy also includes symptomatic (secondary) autonomic neuropathy associated with diabetes, virus infections, hereditary diseases, and tumors (including paraneoplastic syndrome), as well as acute idiopathic autonomic neuropathy (AIAN) of unknown cause initially reported by Young et al. in 1969.9, 10 As a result of constitutional, broad dysautonomia, various autonomic nerve signs, such as orthostatic hypotension, ileus, and dysuria, and a catatonic pupil usually occur in AIAN, the degree is severe,11 and motor, sensory, and CNS disorders are not present in principle.12
We encountered a patient who acutely developed SLE with broad and advanced autonomic symptoms, exhibited various neuropathologies, such as central nervous system and peripheral neuropathy, and responded well to intravenous cyclophosphamide (IVCY).