scholarly journals EP33 Pronounced dys-autonomic symptoms announcing a primary Sjögren’s syndrome

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_2) ◽  
Author(s):  
Francesco Masini ◽  
Klodian Gjeloshi ◽  
Fiammetta Danzo ◽  
Emanuele Pinotti ◽  
Lucio Monaco ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Orthostatic Hypotension ◽  
Autonomic Neuropathy ◽  
Sinus Bradycardia ◽  
Primary Sjögren’S Syndrome ◽  
Sicca Syndrome ◽  
Dry Mouth ◽  
Primary Sjogren’S Syndrome ◽  
Autonomic Symptoms ◽  
History Of

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 >20 mmHg and diastolic reduction >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 (>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.

scholarly journals Clinical and Cost Burden of Primary Sjögren’s Syndrome: Descriptive Analysis Using a US Administrative Claims Database

10.36469/9807 ◽  
2018 ◽  
Vol 5 (2) ◽  
pp. 150-161 ◽  
Author(s):  
Sue Perera ◽  
Liyuan Ma ◽  
Raj Punwaney ◽  
Sulabha Ramachandran
Keyword(s):  
Resource Utilization ◽  
Clinical Characteristics ◽  
Disease Burden ◽  
Healthcare Resource ◽  
Healthcare Resource Utilization ◽  
Primary Sjögren’S Syndrome ◽  
Sicca Syndrome ◽  
Primary Sjogren’S Syndrome ◽  
Primary Sjögren's Syndrome ◽  
Sicca Symptoms

Background: Current knowledge of the disease burden of primary Sjögren’s syndrome (pSS) is limited. Objectives: The primary objective of this study was to describe the demographic and clinical characteristics of patients with pSS. The secondary objective was to describe the treatment patterns and healthcare resource utilization of patients with pSS. Furthermore, clinical characteristics of interest were described and the proportions of patients with glandular versus extra-glandular disease were reported. Methods: This was a retrospective cohort study (HO-15-16077) conducted in the US Truven MarketScan Commercial database. We report descriptive data from employees and their families, as covered by employer-sponsored medical insurance. The primary cohort comprised patients with pSS (with ≥1 diagnosis of sicca syndrome prior to January 1, 2013), with continuous enrollment for ≥24 months (January 1, 2012–December 31, 2013). Patients with conditions mimicking sicca symptoms not due to SS were excluded, as were those with connective tissue disease that may have suggested secondary SS. To compare the healthcare burden of patients with and without sicca symptoms, a 1:1 matched comparator population comprising subjects without a previous diagnosis of sicca syndrome (sicca-free) was also identified. Results: There were 12 717 eligible patients in the primary cohort. The majority (86%) was female and the mean age was 51 years. Overall, 60.7% of patients had claims associated with pSS extra glandular disease manifestations. These patients experienced a higher disease burden, and more commonly reported pain, fatigue or insomnia and any combination of pain, fatigue or insomnia (41.3%) compared with patients with glandular disease only (12.4%). Patients in the primary cohort incurred greater annual healthcare service costs (1.6 times greater, all causes) and healthcare resource utilization compared with the sicca-free comparator cohort. Patients with extra glandular disease also incurred greater average annual costs (2.9 times) contributing to ≥2 times/year more resource use for outpatient services than patients with glandular disease only. Conclusion: Patients with pSS experience a high disease burden despite treatment. This study provides novel insights in to the extent of the burden on healthcare resources among patients with pSS, in particular for patients with extra-glandular disease manifestations, when compared with sicca-free subjects.

Fatigue and blood pressure in primary Sjögren's syndrome

2008 ◽  
Vol 37 (4) ◽  
pp. 284-292 ◽  
Author(s):  
H. Forsblad d'Elia ◽  
E. Rehnberg ◽  
G. Kvist ◽  
A. Ericsson ◽  
Y. T. Konttinen ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Primary Sjögren’S Syndrome ◽  
Primary Sjogren’S Syndrome ◽  
Primary Sjögren's Syndrome ◽  
Sjögren‘S Syndrome

scholarly journals Expression of IL-17, IL-23 and Their Receptors in Minor Salivary Glands of Patients with Primary Sjögren's Syndrome

2012 ◽  
Vol 2012 ◽  
pp. 1-8 ◽  
Author(s):  
Diana Mieliauskaite ◽  
Irena Dumalakiene ◽  
Rita Rugiene ◽  
Zygmunt Mackiewicz
Keyword(s):  
Salivary Glands ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Primary Sjögren’S Syndrome ◽  
Sicca Syndrome ◽  
Minor Salivary Glands ◽  
Primary Sjogren’S Syndrome ◽  
Primary Sjögren's Syndrome ◽  
Sjögren‘S Syndrome

The main purpose of this study was to determine the expression of interleukins-17/-23 (ILs-17/-23) and receptors of interleukins-17/-23 (IL-17R, IL-23R) in minor salivary glands (MSGs) of patients with primary Sjögren's syndrome (pSS). Expression of IL-17, IL-23 and receptors of IL-17/-23 was analyzed in MSGs from 25 patients with pSS, 25 patients with probable preclinical pSS, and 25 patients with nonautoimmune sicca syndrome by immunohistochemistry. Comparison of the expression of IL-17, IL-23 and receptors of IL-17, IL-23 in MSG of patients with pSS with probable preclinical pSS, and with nonautoimmune sicca syndrome showed significant differences between three groups. However, the expression of IL-17, IL-23 and receptors of IL-17/-23 in MSG was comparable in pSS and probable preclinical pSS patients. We did not find correlation between the expression of IL-17 and IL-23 and of IL-17R and IL-23R in patients with pSS. These results demonstrate an involvement of IL-17/-23 system in the early pSS pathogenesis.

Afferent baroreflex dysfunction and age-related orthostatic hypotension

1991 ◽  
Vol 81 (s25) ◽  
pp. 531-538 ◽  
Author(s):  
A. L. Tonkin ◽  
L. M. H. Wing ◽  
M. J. Morris ◽  
V. Kapoor
Keyword(s):  
Blood Pressure ◽  
Heart Rate ◽  
Cold Stress ◽  
Orthostatic Hypotension ◽  
Autonomic Neuropathy ◽  
Isometric Exercise ◽  
Control Subjects ◽  
Age Related ◽  
Head Up Tilt ◽  
Elderly Control

1. To test the hypothesis that in apparently healthy elderly subjects with orthostatic hypotension there is afferent baroreflex dysfunction, cardiovascular and neurohumoral responses were measured after separate stimuli which activated baroreceptor (head-up tilt) and non-baroreceptor (cold stress, isometric exercise) afferent pathways. 2. In 15 healthy elderly control subjects blood pressure did not change with 60° head-up tilting and there was a moderate increase in heart rate, whereas in 13 subjects with age-related orthostatic hypotension head-up tilting was associated with a marked fall in blood pressure but a similar heart rate response to that in the elderly control group. In contrast, both groups of subjects had similar blood pressure and heart rate responses to cold stress and sustained isometric exercise. 3. Nine subjects with autonomic neuropathy also showed a marked hypotensive response to head-up tilt, but produced no pressor response to cold stress or isometric exercise. 4. The plasma concentrations of noradrenaline, adrenaline and neuropeptide-Y-like immunoreactivity rose and that of atrial natriuretic peptide fell after head-up tilt in the study population as a whole. There were no significant differences between groups despite the much greater blood pressure drops in the subjects with autonomic neuropathy and in those with age-associated orthostatic hypotension. 5. The aorto-iliac pulse wave velocity index was significantly higher in subjects with age-associated orthostatic hypotension compared with that in control subjects. 6. The pattern of responses to the separate stresses observed in the group with age-associated orthostatic hypotension is characteristic and different from that in the elderly control subjects and the subjects with autonomic neuropathy. It suggests that age-associated orthostatic hypotension is related predominantly to dysfunction in the afferent limb of the baroreflex arc, possibly partially caused by a splinting of arterial baroreceptors by non-compliant arterial walls.

scholarly journals Prevalence and coincidence of hypertension and orthostatic hypotension in pre- and centenarians and their effect on mortality: preliminary results of Moscow centenarian study

2020 ◽  
Vol 26 (1) ◽  
pp. 37-42
Author(s):  
K. A. Eruslanova ◽  
L. V. Matchekhina ◽  
Y. V. Kotovskaya ◽  
N. K. Runikhina ◽  
O. N. Tkacheva
Keyword(s):  
Blood Pressure ◽  
Orthostatic Hypotension ◽  
Antihypertensive Drugs ◽  
Factors Affecting ◽  
Wide Range ◽  
Moscow City ◽  
Minimum Age ◽  
History Of ◽  
Study Participants ◽  
Design And Methods

Objective. To estimate the prevalence and impact on mortality of arterial hypertension (HTN) and orthostatic hypotension (OH) in centenarians (95 years and older) in Moscow.Design and methods. The study participants were 82 super-long-livers of Moscow city aged 95 years and older (minimum age of 95 years, maximum 105 years), who underwent a comprehensive geriatric assessment at home by a multidisciplinary team (geriatrician, nurse and social worker). The following prospective observation lasted for three years (36 months).Results. Past medical history of HTN was noted in 78 %. The mean systolic blood pressure (SBP) in the supine position was 151 ± 27,9 mm Hg (100–216 mm Hg), and the diastolic blood pressure (DBP) 74 ± 12,8 mm Hg (44–197 mm Hg). OH was detected in 31 % of 61 long-livers who was able to perform an orthostatic test. The presence of OH was not associated with the higher intake of antihypertensive drugs. Within three years, 44 study participants died. The level of blood pressure (BP), history of HTN, and the presence of OH did not affect mortality (p > 0,05).Conclusions. Centenarians have a wide range of SBP and DBP, high prevalence of HTN and OH. BP level, presence of HTN and OH did not affect mortality over 3 year period. Further investigation is needed to understand better the health status of long-livers and factors affecting the prognosis.

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

Reumatismo ◽  
2020 ◽  
Vol 72 (2) ◽  
pp. 111-114
Author(s):  
F. Masini ◽  
L. Monaco ◽  
K. Gjeloshi ◽  
E. Pinotti ◽  
R. Ferrrara ◽  
...  
Keyword(s):  
Autoimmune Disease ◽  
Physical Examination ◽  
Orthostatic Hypotension ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sinus Bradycardia ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
History Of ◽  
Sjögren‘S Syndrome

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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