Autonomic neuropathy associated with sicca complex

We retrospectively studied 52 patients with sicca complex who presented pri marily with peripheral neuropathic symptoms to assess the degree of autonom ic involvement and its relationship to somatic neuropathy, serological test s and extraglandular disease. One patient presented with severe panautonomi c dysfunction, with another six patients reporting prominent autonomic symp toms, and a further 40 patients having mild or asymptomatic disease. Eighty -seven percent of patients showed abnormalities on an autonomic reflex scre en (ARS). Fifty-eight percent of patients showed a mixed pattern of autonom ic dysfunction with abnormalities in two or more ARS subgroups of sudomotor , cardiovagal and adrenergic function. Three patients had a cholinergic aut onomic neuropathy with sparing of adrenergic function. Tonic pupils were pr esent in 13 patients. Autonomic neuropathy occurred in patients with a vari ety of somatic neuropathy subtypes, without a clear relationship between th e type of autonomic neuropathy and the type of somatic peripheral neuropath y. Extraglandular disease was uncommon, occurring in only four patients. Se rological abnormalities (extractable nuclear antigen [ENA], SS-A, SS-B) occ urred in 20% of patients. We conclude that: (1) A wide spectrum of autonomi c dysfunction is seen in sicca complex. (2) Severe autonomic involvement is uncommon, with most patients showing mildly symptomatic or asymptomatic di sease. (3) Overall, characteristic patterns of autonomic dysfunction did no t predominate, and then were no clear relationships with the different type s of somatic peripheral neuropathy. (4) Clinical and serological evidence o f non-neurological extraglandular disease is uncommon in this group of pati ents. (C) 1999 Elsevier Science B.V. All rights reserved.