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

  • F. Masini Internal Medicine, Department of Advanced Medical and Surgical Sciences, University of Campania “Luigi Vanvitelli”, Napoli, Italy. https://orcid.org/0000-0002-4184-2808
  • L. Monaco Internal Medicine, Department of Advanced Medical and Surgical Sciences, University of Campania “Luigi Vanvitelli”, Napoli, Italy.
  • K. Gjeloshi Internal Medicine, Department of Advanced Medical and Surgical Sciences, University of Campania “Luigi Vanvitelli”, Napoli, Italy. https://orcid.org/0000-0003-0874-9692
  • E. Pinotti Internal Medicine, Department of Advanced Medical and Surgical Sciences, University of Campania “Luigi Vanvitelli”, Napoli, Italy. https://orcid.org/0000-0002-1275-9079
  • R. Ferrrara Internal Medicine, Department of Advanced Medical and Surgical Sciences, University of Campania “Luigi Vanvitelli”, Napoli, Italy.
  • P.C. Pafundi Internal Medicine, Department of Advanced Medical and Surgical Sciences, University of Campania “Luigi Vanvitelli”, Napoli, Italy. https://orcid.org/0000-0002-0310-3529
  • M. Santini Internal Medicine, Department of Advanced Medical and Surgical Sciences, University of Campania “Luigi Vanvitelli”, Napoli, Italy. https://orcid.org/0000-0003-1162-7805
  • T. Salvatore Internal Medicine, Department of Advanced Medical and Surgical Sciences, University of Campania “Luigi Vanvitelli”, Napoli, Italy.
  • G. Cuomo | giovanna.cuomo@unicampania.it Internal Medicine, Department of Precision Medicine, University of Campania “Luigi Vanvitelli”, Napoli, Italy. https://orcid.org/0000-0002-4292-3589

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.

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Published
2020-07-23
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Issue
Section
Case Reports
Keywords:
Primary Sjögren’s syndrome, dys-autonomic symptoms
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How to Cite
Masini, F., Monaco, L., Gjeloshi, K., Pinotti, E., Ferrrara, R., Pafundi, P., Santini, M., Salvatore, T., & Cuomo, G. (2020). Pronounced dys-autonomic symptoms announcing a primary Sjögren’s syndrome. Reumatismo, 72(2), 111-114. https://doi.org/10.4081/reumatismo.2020.1241