EP33 Pronounced dys-autonomic symptoms announcing a primary Sjögren’s syndrome
Authors: Masini Francesco , Gjeloshi Klodian , Danzo Fiammetta , Pinotti Emanuele , Monaco Lucio , Cuomo Giovanna 
Published: April 2020

Abstract

Background
Sjogren syndrome (SS) is an autoimmune disease which involves the nervous system in about 20% of SS cases, whilst in 25-92% neurological symptoms may precede the onset of sicca syndrome.

Methods
A 65-year-old male came to our observation due to a 7-month history of near-syncope episodes, constipation, hypohidrosis, fatigue and asthenia. Past history revealed hypothyroidism due to Hashimoto's thyroiditis, under treatment with levothyroxine. Physical examination showed: temperature 36.5 °C, blood pressure 90/60 mmHg and respiration rate 20 breaths per minute. The ECG revealed sinus bradycardia with 55 beats per minute (bpm) and right bundle-branch block (RBBB). Laboratory exams showed: lymphocytopenia (WBC 9008/μL, with 90% granulocytes and 3% lymphocytes); CRP (0.3 mg/dL), ESR (20mm/hr). In order to investigate endocrine disorders, hormone tests were also performed, showing: euthyroidism (FT3 2.0 pg/mL, FT4 12.6 pg/mL, TSH 1.685 µUI/mL, Ab anti-thyroglobulin 1630.0 UI/mL, Ab anti-thyreoperoxidase 592.5 UI/mL), hypogonadotropic hypogonadism (LH 1.6 mIU/mL, FSH 2.7 mIU/mL, prolactin 15.9 ng/mL, testosterone 133 ng/dL) and normal adrenocortical function (serum cortisol 8 am 17.5 µg/dL, aldosterone 6.8 pg/dL, ACTH 8.5 pg/mL). Brain-MRI showed anterior pituitary hypoplasia.

Results
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 (at 120 seconds systolic reduction was &gt;20 mmHg and diastolic reduction &gt;10 mmHg) were performed, all resulting pathological. ECG Holter monitoring revealed sinus bradycardia, with a mean heart rate of 55 bpm and RBBB. Holter blood pressure 24-hour monitoring showed a diurnal hypotensive profile. The echocardiogram revealed a mild mitral and tricuspid insufficiency. A paraneoplastic syndrome was also excluded by total body-CT with mean of contrast and tumor markers, which resulted normal. On a further assessment, the patient reported a 3-month history of worsening dry mouth and persistent dehydrated tongue. Signs of anisocoria in response to light stimulation, were observed as well. Tests for autoimmune diseases showed ANA (1:320 speckled pattern), Ro/SS-a (&gt;240U/l) and La/SS-b (162 U/ml) antibodies. Though symptoms such as dry eyes were not reported, “Shirmer test” revealed an insufficient tear production in both eyes (RE 5 mm, LE 3 mm). The patient was discharged with the diagnosis of autonomic neuropathy most likely due to primary Sjögren’s syndrome and started therapy with Prednisone 25 mg/die and Hydroxychloroquine 200 m/bid o.d. After one month, he reported a significant improvement in symptoms such as asthenia, fatigue and dry mouth. Orthostatic hypotension symptoms were also significantly decreased and no further near-syncopal events occurred.

Conclusion
This case underlines the clinically importance of dys-autonomic symptoms, antecedent to the onset of sicca syndrome, and highlights the favorable response to immune-modulating therapy in the treatment of autonomic dysfunction arising from SS.

Disclosures
F. Masini None. K. Gjeloshi None. F. Danzo None. E. Pinotti None. L. Monaco None. G. Cuomo None.