Pronounced dys-autonomic symptoms announcing a primary Sjögren’s syndrome

Abstract

Sjögren’s syndrome (SS) is an autoimmune disease that involves the nervous system in about 20% of cases. In 25-92% of patients affected by Sjögren’s syndrome, neurological symptoms may precede the sicca syndrome. A 65-year-old male presented with a seven-month history of episodes of near-syncope, constipation, anhidrosis, disabling fatigue and asthenia. Physical examination was unremarkable, whilst the ECG revealed sinus bradycardia. Laboratory tests showed lymphopenia and normal inflammatory markers. In order to assess a potential autonomic neuropathy, “Deep Breathing Test” (E/I 1.02), “Lying to Standing Test” (R/R’ 0.95), and “Orthostatic Hypotension Tests” (T 120s Systolic reduction >20 mmHg and Diastolic reduction >10 mmHg) were performed, all of which were abnormal. ECG Holter monitoring revealed sinus bradycardia, and right bundle branch block with 24-h blood pressure monitoring revealing a diurnal hypotensive profile. The patient reported a three-month history of worsening dry mouth. On physical examination, the patient had anisocoria in response to light stimulation. Auto-antibody testing was performed to evaluate the presence of any autoimmune disease. The results of these studies included an abnormal elevation of ANA (1:320 speckled pattern), Ro/SS-a (>240U/l), and La/SS-b (162 U/ml) antibodies. The patient was discharged with a diagnosis of “Autonomic Neuropathy Most Likely Due to Primary Sjögren’s Syndrome (SS)” and started the immunotherapy. After one month, he reported a significant improvement in his symptoms with a concomitant normalization of his “Orthostatic Hypotension Tests.” This case underlines the potential for dys-autonomic symptoms to precede the onset of sicca syndrome in patients with Sjogren’s Syndrome.

Keywords

• Primary Sjögren’s syndrome
• dys-autonomic symptoms