Dysautonomia as a form of presentation of primary Sjögren's disease: a case series of six patients.

Abstract

We present six patients with dysautonomia secondary to primary Sjögren's disease (pSjD) and analyze the literature regarding this topic. Case series. Patients were retrospectively recruited from a tertiary center in Mexico from 2001 to 2022 and included if they met 2016 ACR/EULAR criteria for SjD. Dysautonomia was diagnosed by the presence of autonomic symptoms and at least one of Ewin's battery tests. We scored the Composite Autonomic Severity Scale (CASS), and the Composite Autonomic Symptom Score (COMPASS-31). Patients (n = 6) were primarily females (83%), with a range of 23 to 60 years at onset of neurological manifestations. Dysautonomia preceded 2-10 years the diagnosis of pSjD in five patients. The clinical manifestations varied, but all patients had orthostatic hypotension and syncope. Patients had variability regarding non-neurological disease activity (ESSDAI 0-15) at the onset of neurological manifestations. Treatment for dysautonomia included prednisone, hydroxychloroquine, DMARDs, rituximab, or their combinations. Overall, we observed partial improvement in most of the cases and only one patient had remission of her symptoms of dysautonomia. Two patients discontinued treatment and lost follow-up. Few cases of dysautonomia and biopsy proven pSjD were identified in the literature, but they shared clinical features with ours. Dysautonomia is a rare and challenging neurological manifestation attributed with pSjD. The main symptoms in our patients were orthostatic hypotension and syncope; and most of the times preceded SjD diagnosis. Partial improvement was achieved in the majority of patients following treatment.