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The epidemiology of Sjögren syndrome (SS) has been extensively studied in America, Europe, and Asia.To summarize available data on the epidemiology of SS in Africa.MEDLINE, EMBASE, and African Journals Online were searched from inception up to May 17, 2020, to identify relevant articles. Data gleaned from these reports have been summarized narratively in this review.Twenty-one hospital-based studies were included. These studies reported 744 cases of SS. The mean age at diagnosis varied between 28 and 73.6 years, and the female proportion ranged from 83.3% to 100%. There was no population-based incidence or prevalence. Among people with autoimmune and other rheumatic conditions, the frequency of primary SS was in the range 1.9% to 47.6%, whereas that of rheumatoid arthritis-associated secondary SS was in the range 4.3% to 100%. Sicca symptoms were the commonest features, with most frequently involved organs being joints, lungs, and neurological structures. Main autoantibodies were anti-Ro/SS antigen A, anti-La/SS antigen B, and antinuclear antibodies.The epidemiology of SS is poorly characterized in Africa. Available data are broadly consistent with those from other populations. Extensive and high-quality research is urgently needed.

DrożakMartyna,DrożakPaulina,DziekońskaJustyna,NowińskaMartyna,GrabowyPaulina. Xerostomia among elderly patients – systematic review.Journal of Education, Health and Sport. 2021;11(9):289-297. eISSN 2391-8306. DOI http://dx.doi.org/10.12775/JEHS.2021.11.09.035 https://apcz.umk.pl/JEHS/article/view/JEHS.2021.11.09.035 https://zenodo.org/record/5515203 The journal has had 5 points in Ministry of Science and Higher Education parametric evaluation. § 8. 2) and § 12. 1. 2) 22.02.2019. © The Authors 2021; This article is published with open access at Licensee Open Journal Systems of Nicolaus Copernicus University in Torun, Poland Open Access. This article is distributed under the terms of the Creative Commons Attribution Noncommercial License which permits any noncommercial use, distribution, and reproduction in any medium, provided the original author (s) and source are credited. This is an open access article licensed under the terms of the Creative Commons Attribution Non commercial license Share alike. (http://creativecommons.org/licenses/by-nc-sa/4.0/) which permits unrestricted, non commercial use, distribution and reproduction in any medium, provided the work is properly cited. The authors declare that there is no conflict of interests regarding the publication of this paper. Received: 05.09.2021. Revised: 18.09.2021. Accepted: 18.09.2021. Xerostomia among elderly patients – systematic review Martyna Drożak1, Paulina Drożak1, Justyna Dziekońska2, Martyna Nowińska2, PaulinaGrabowy2 Students’ Scientific Association of Chair and Department of Family Medicine, Medical University of Lublin Students’ Research Group of Applied Psychology, Medical University of Lublin Abstract Introduction and purpose:Xerostomia is a salivary secretion malfunction that affects from 1 to 29% of the population. It is a common problem among elderly patients, however undeniably it still remains an underdiagnosed issue. Searching among available literature in the PubMed database with the following phrases: xerostomia; elderly, the aim of this article was to provide a broad review on the underdiagnosed problem which is xerostomia among elderly patients. Description:A group at risk of developing xerostomia are people over 65 years old and women in the perimenopausal period. Although dry mouth varies in etiology, geriatric patients mostly develop xerostomia as a result of head and neck radiotherapy, Sjögren syndrome or medication treatment. Untreated symptoms can lead to severe issues which heavily impact not only oral health of the patient, but also their everyday life quality, since xerostomia may lead into difficulty of speaking, swallowing and tasting. The plan of treatment is influenced by the etiology of the case, however it is aimed to stimulate salivary flow and eradicate the use of unnecessary medication which may cause dry mouth. Prevention of dry mouth is based on maintenance of good oral hygiene. Conclusions:Diagnosing the problem early can prevent patients from suffering the consequences of chronic xerostomia, therefore awareness should be brought to this issue. Dentist could also highly improve the quality of xerostomic patient’s life, if the chosen treatment significantly improved patient’s symptoms. Key words:xerostomia; elderly

Background/aim Sjögren’s syndrome (SS) is an autoimmune disease and its pathogenesis is still not completely clear. The wingless (Wnt)/β-catenin pathway has recently been shown to play an important role in inflammation. This study aims to determine the serum and saliva levels of Dickkopf (DKK)1 and sclerostin and to evaluate Wnt-1 and Wnt-3a expression in the salivary gland in patients with primary SS. Materials and methods This study included 30 patients diagnosed with SS, 30 patients diagnosed with systemic lupus erythematosus (SLE), and 29 healthy controls. Serum and saliva levels of DKK1 and sclerostin were measured and the expressions of Wnt1 and Wnt3a in the salivary gland were measured immunohistochemically. Results Serum DKK1 and sclerostin levels were lower in the SS and SLE groups compared to the control group (both p < 0.001). Saliva DKK1 levels were higher in the SS group compared to the control and SLE groups (p = 0.004 and p = 0.009, respectively). Wnt1 and Wnt3a expression were found in salivary gland tissue samples in 71.4% of primary SS patients and relatively frequent than control group. Conclusions Serum DKK1 and sclerostin levels in primary SS and SLE were decreased. Moreover, levels of Wnt1 and Wnt3a expression in the salivary gland were also elevated in primary SS. Therefore, it can be concluded that the Wnt/β-catenin pathway activities may be altered in case of glandular inflammation.

The aim of this study was to investigate the perspective of Romanian patients with Sjögren syndrome (SS) on various aspects of the disease during the SARS-CoV-2 outbreak, including both the impact of COVID-19 on the disease itself as well as the effects of vaccination against SARS-CoV-2 in this group of patients. The study is an online questionnaire-based survey. We received responses from 137 SS patients. Regarding the general emotional status, 33 patients (24.0%) and 47 patients (34.3%) declared to have been sadder/depressive and more agitated/anxious during the SARS-CoV2 outbreak, respectively. During the lockdown, 49 (33.7%) patients strictly and 77 patients (56.2%) did their best to respect the home isolation measures. The income was unchanged for most of the patients (94 patients, 68.6%). Regarding access to healthcare providers, 27 patients (18.7%) postponed the consultation for fear of getting SARS-CoV2. In our study group, 31 patients (22.6%) responded that they have had COVID-19. Only one patient was completely asymptomatic, while the most frequently declared symptom was weakness (84.0%). In 17 patients among the respondents (68%) the symptoms lasted for at least 2 weeks; the most frequent long-lasting symptoms were fatigue (40.0%) and weakness (36.0%). Out of all the respondents, 53 patients (41.4%) were vaccinated against SARS-CoV2 with at least one dose. After the first dose, the most prevalent side effect was pain at the site of injection (89.2%), followed by weakness (25.0%) and myalgias (21.4%). This information will be useful for developing special programs dedicated to SARS-CoV2 infection and vaccination in patients with SS and other autoimmune diseases. Supplementary Information The online version contains supplementary material available at 10.1007/s00296-021-04967-4.

To investigate the correlation between the duration of Sjogren syndrome (SS) and ocular surface parameters in patients with SS-related dry eye. We analyzed 108 eyes of 108 female patients with primary SS-related dry eye. All patients underwent rheumatoid serologic tests and ocular surface assessments. The ocular surface assessment included the Standard Patient Evaluation of Eye Dryness (SPEED) score, meibomian gland (MG) atrophy, lipid layer thickness (LLT), partial and total blinking, partial blinking rate, Schirmer’s I test, non-invasive tear break-up time, and ocular surface staining score. Correlations between the duration of SS and ocular surface assessments were calculated. The average age and SS duration of the participants were 56.7 ± 10.2 (range 21–78) years and 54.15 ± 41.10 (range 1–134) months, respectively. There was a strong positive correlation between SS duration and MG atrophy (r = 0.766, p

This clinical quandary details a Mexican man, aged 77 years, who presented to the oncology clinic with a sternal mass. Based on the results, the patient fulfilled the 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for Sjögren syndrome, thus the diagnosis triggered by immune checkpoint inhibitors was definitively established.

The aims of this pilot investigation were to calculate the levels of sensitivity and specificity of salivary glands ultrasonography (SGUS) in diagnosing Sjögren syndrome (SS) and to assess the ultrasonographic findings of parotid and submandibular glands.Patients diagnosed with SS or dry mouth and healthy controls were enrolled. Bilateral parotid and submandibular glands were assessed for (1) parenchymal inhomogeneity (PIH), (2) median size of the glands, (3) visibility of glandular posterior borders, and (4) size of sialolith, if present.This study included 34 female patients, of whom 12 had SS (35.3%), 12 had dry mouth (35.3%), and 10 were healthy controls (29.4%). Patients with SS showed higher PIH scores in all glands with the median differences being statistically higher in the right and left parotids and left submandibular glands (P.001, P = .012, and P.001, respectively). SGUS, with a PIH cutoff ≥2, showed a sensitivity of 100% and a specificity of 81.6% for detecting SS. The majority of SS had invisible glandular posterior borders (P.001). Median size of the glands and size of the sialolith did not show any statistically significant differences between groups.SGUS is a noninvasive imaging modality with good sensitivity and specificity that might be valuable as a diagnostic aid for SS.

Primary biliary cirrhosis is a rare progressive autoimmune liver disease that causes chronic cholestasis. Of patients with primary biliary cirrhosis, 75% develop secondary Sjogren syndrome and could develop vitamin A deficiency. Here, we report the case of a patient with primary biliary cirrhosis who developed a secondary Sjogren syndrome and vitamin A deficiency, which led to severe and unusual eye involvement with multiple and recurrent spontaneous corneal perforations. Corneal perforations in patients with primary biliary cirrhosis and secondary Sjogren syndrome are rare but devastating complications, in contrast to other eye clinical manifestations of the disease.

Calcific uremic arteriolopathy, termed calciphylaxis, was previously considered a condition that developed mostly in patients requiring dialysis. It has now been described in kidney transplant patients, in advanced chronic kidney disease (CKD) patients not requiring dialysis, and in individuals with maintained kidney function. We describe an individual with CKD stage 3b with hypercalcemia who presented with features highly specific for calciphylaxis based on results of a skin biopsy. The condition has high morbidity and mortality, and thus prompts immediate cessation of the offending agents or treatment of the cause. The following case and literature review demonstrates a need for a detailed assessment of patients' risks and exposures and expanding the differential diagnosis to include calciphylaxis in nonuremic patients with necrotic ulcers with a plan for early imaging and possible biopsy.

IgG4-related disease (IgG4-RD) is an immune-mediated disease condition that can affect almost any organ, including the head and neck. It is a chronic, systemic inflammation of unknown etiology. Tumor formation is the most common clinical symptom. Any tumor in the head and neck area is always a concern for an otorhinolaryngologist, head and neck surgeon, that we are dealing with malignant neoplastic growth. Ultrasound imaging, computer tomography, magnetic resonance imaging and even fine needle aspiration biopsy usually do not exclude neoplastic hyperplasia. Only open biopsy or excision biopsy followed by histopathological examination suggest the diagnosis of IgG4-RD, which requires further diagnosis, mainly serological. The authors present the most common IgG4-RD in the head and neck area, diagnostic criteria and their differentiation from apparently similar diseases.

Sjogren Syndrome (SS), a slowly Progressive disease that has unified, validated diagnostic criteria, with excellent evidence and performance in adults, but not in adolescents. We report a 17 year old teenager with a family history of SS in his sister and mother. He presented with fever and fatigue. He had an elevated C reactive protein, leukopenia, positive antinuclear and anti-Rho antibodies and rheumatoid factor. A scintigraphy showed a severe salivary gland dysfunction. The syndrome in this patient had a pediatric clinical behavior despite his proximity to adulthood.

Background The aim of this study was to evaluate the in vivo confocal microscopic morphology of corneal subbasal nerves and its relationship with clinical parameters in patients with primary Sjögren’s syndrome in China. Methods This was a case control study of 22 dry eye disease (DED) patients with primary Sjögren’s syndrome (pSS) and 20 control subjects with non-Sjögren dry eye disease (NSDE). Each patient underwent an evaluation of ocular surface disease using the tear film break-up time (TBUT), noninvasive tear film break-up time (NIKBUT), noninvasive tear meniscus height (NIKTMH), corneal staining (National Eye Institute scale, NEI), Schirmer I test, meibography, and corneal subbasal nerve analysis with in vivo confocal microscopy (IVCM). The right eye of each subject was included in this study. Results SS patients showed a shorter TBUT (P = 0.009) and Schirmer I test results (P = 0.028) than the NSDE group. However, there was no significant difference in NIKBUT between the two groups (P = 0.393). The nerve density of subbasal nerves, number of nerves and tortuosity of the SS group were significantly lower than those of the NSDE group (P = 0.001, P P = 0.039, respectively). In the SS group, the mean nerve length was correlated with age and the Schirmer I test (r = − 0.519, P = 0.013 and r = 0.463, P = 0.035, respectively). Corneal staining was correlated with nerve density and the number of nerves (r = − 0.534, P = 0.013 and r = − 0.487, P = 0.025, respectively). Conclusions Sjögren syndrome dry eye (SSDE) patients have more severe clinical dry eye parameters than non-Sjögren dry eye disease (NSDE) patients. Compared with NSDE patients, we found that SSDE patients showed decreased corneal subbasal nerve density and numbers.

Although the physiological control of salivary secretion has been well studied, the impact of disease on salivary gland function and how this changes the composition and function of saliva is less well understood and is considered in this review. Secretion of saliva is dependent upon nerve-mediated stimuli, which activate glandular fluid and protein secretory mechanisms. The volume of saliva secreted by salivary glands depends upon the frequency and intensity of nerve-mediated stimuli, which increase dramatically with food intake and are subject to facilitatory or inhibitory influences within the central nervous system. Longer-term changes in saliva secretion have been found to occur in response to dietary change and aging, and these physiological influences can alter the composition and function of saliva in the mouth. Salivary gland dysfunction is associated with different diseases, including Sjögren syndrome, sialadenitis, and iatrogenic disease, due to radiotherapy and medications and is usually reported as a loss of secretory volume, which can range in severity. Defining salivary gland dysfunction by measuring salivary flow rates can be difficult since these vary widely in the healthy population. However, saliva can be sampled noninvasively and repeatedly, which facilitates longitudinal studies of subjects, providing a clearer picture of altered function. The application of omics technologies has revealed changes in saliva composition in many systemic diseases, offering disease biomarkers, but these compositional changes may not be related to salivary gland dysfunction. In Sjögren syndrome, there appears to be a change in the rheology of saliva due to altered mucin glycosylation. Analysis of glandular saliva in diseases or therapeutic interventions causing salivary gland inflammation frequently shows increased electrolyte concentrations and increased presence of innate immune proteins, most notably lactoferrin. Altering nerve-mediated signaling of salivary gland secretion contributes to medication-induced dysfunction and may also contribute to altered saliva composition in neurodegenerative disease.

Sjogren syndrome is a chronic autoimmune inflammatory disease characterized by lymphocytic infiltration of exocrine glands, predominantly the parotid and lacrimal glands, thereby resulting in oral and ocular dryness. It has been reported to occur most frequently in women between 40 and 50 years of age. Sjogren syndrome has an insidious onset, is slowly progressive, and presents a wide range of clinical manifestations, leading to delays or challenges in the diagnosis. Early diagnosis of this condition is essential to prevent the associated complications that affect patients' quality of life. This report presents 3 cases of Sjogren syndrome in female patients aged between 40 and 75 years who presented with complaints of persistent dry mouth and burning sensation. The cases highlight the diagnostic value of 3-dimensional cone-beam computed tomographic sialography in the detection of salivary gland pathologies at an early stage.

Rheumatologische Erkrankungen gehoren zu den weltweit haufigsten Ursachen fur eine krankheitsbedingte signifikante Einschrankung der Lebensqualitat und Lebenserwartung und verursachen signifikante volkswirtschaftliche Kosten. Gerade bei der rheumatoiden Arthritis, beim systemischen Lupus erythematodes, beim Sjogren-Syndrom und der ankylosierenden Spondylitis sind neurologische Begleiterkrankungen sehr haufig. Dabei sind alle Bereiche vom ZNS uber das periphere Nervensystem bis hin zur Muskulatur betroffen. Am haufigsten sind sicherlich neben einer krankheitsbegleitenden Depression Polyneuropathien in sehr unterschiedlicher Auspragung. Subklinisch sind nach Studien z. B. bis zu 85% der Patienten mit einer rheumatoiden Arthritis von einer PNP betroffen. Daruber hinaus konnen in der Therapie rheumatologischer Erkrankungen verwandte Medikamente wie vor allem monoklonale Antikorper zu neurologischen Nebenwirkungen inklusive der Entwicklung einer progressiven multifokalen Leukoenzephalopathie fuhren. Vor diesem Hintergrund ist es wichtig, rheumatologische Erkrankungen in die Differenzialdiagnose bei neu aufgetretenen neurologischen Erkrankungen einzubeziehen.

Chikungunya virus (CHIKV) infection is caused by an arbovirus prevalent in various parts of the world. The virus can induce autoantibodies and rheumatic diseases, such as rheumatoid arthritis and spondylarthritis. However, until now, no case of Sjögren syndrome (SS) was described associated with CHIKV. In this article, we describe a 49-year-old female with polyarthralgia and a temporary rash on her trunk and arms. Her physical examination showed polyarthritis of her ankles and wrists. Serologies for CHIKV were interpreted as positive with IgM 6.5 (normal range < 0.8) and negative for IgG. Antinuclear antibodies were positive at a titer of 1:640 as well as anti-Ro/SS-A. The diagnosis of subacute CHIKV infection was determined. The Schirmer test, Rose Bengal, and salivary scintigraphy were positive and the diagnosis of SS was confirmed. She was treated with hydroxychloroquine, methotrexate, and a single dose of betamethasone depot. This is the first report on CHIKV associated with SS. Sequence analysis of the CHIKV proteome versus SS autoantigens showed an extensive peptide sharing between the virus and numerous SS autoantigens, thus supporting the hypothesis that autoimmune cross-reactivity might causally link CHIKV to SS.

Introduction Oral and ocular dryness are the most common symptoms reported during ophthalmological and dental examinations. It is becoming a serious and growing problem due to the huge variety of affecting factors and with population aging. Objectives The purpose was to demonstrate an application of the Schirmer test for xerostomia. Subsequently, to compare and correlate the results achieved from the lacrimal Schirmer test and salivary Schirmer test in non-Sjögren patients. Methods Study group consisted of 642 patients with/without subjective or/and objective symptoms of dry eye or mouth who did not fulfill the criteria for diagnosis of Sjögren syndrome. The lacrimal Schirmer test (lST) and the salivary Schirmer tests (sST) were performed (sSTm was put on the floor of the mouth, sSTp in front of the parotid gland duct). The results were recorded after 1 min (sSTm), 3 min (sSTp), and 5 min (lST). Results The lST and sST test scores were considerably higher in the healthy group than in others, p < 0.001. The results of sST1 and sST2 decreased with the appearance of subjective and objective symptoms, p < 0.001. There were positive correlations between lST and sSTm outcomes between the groups, p < 0.001. Conclusions We present the Schirmer test adapted to measure salivary gland hypofunction that is a time-saving tool in our daily practice. Results of this study reveal an excellent correlation between the eye Schirmer test and the salivary Schirmer tests. Clinical relevance The salivary Schirmer tests seem to be rapid, convenient, and reliable objective screening tools for salivary gland hypofunction in non-Sjögren patients.

The Food and Drug Administration has recently approved the off-label use of hydroxychloroquine (HCQ) for the treatment of coronavirus disease 2019 (COVID-19) infections. However, a recent study not only failed to demonstrate HCQ efficacy but also documented a serious side effect of COVID-19 therapy with HCQ: QT prolongation and secondary arrhythmia. HCQ has been used as an off-label drug and deemed safe and effective for the treatment of oral lesions, such as Sjogren syndrome (SS), chronic ulcerative stomatitis (CUS), and oral lichen planus (OLP). Because HCQ may be appropriately used for the off-label treatment of SS, CUS, and OLP, relevant safety concerns regarding HCQ therapy with regard to dosage, drug-to-drug interactions, and QT prolongation and secondary arrhythmia are discussed here. Because of the possibility of decreased pharmacy supplies of HCQ, replacement drugs with respect to patients with SS, CUS, and OLP being successfully treated with HCQ are also discussed.

Objectives The aim of this study was to find out the correlation and evaluate the accuracy of labial minor salivary gland biopsy as a diagnostic tool in the multidisciplinary management of patients with Sjogren syndrome. Materials and methods Patients, referred to our outpatient office between January 2014 and December 2018 from a rheumatologist for biopsy examination, as part of the complex diagnostic plan for suspected Sjogren syndrome, were included in the current study. Each specimen was examined histomorphometrically by the pathologist to calculate the focus score describing the degree of salivary gland inflammatory infiltration. Results Fifty patients met the inclusion criteria. From the total number of patients, 39 presented with an established Sjogren syndrome by fulfilling the revised American-European criteria. From those, 27 had a positive lip biopsy. The remaining 12 patients from the total group, who were diagnosed with Sjogren syndrome based on the same criteria, had a negative lip biopsy. Conclusion The labial minor salivary gland biopsy is a valuable diagnostic tool to establish the diagnosis of Sjogren syndrome. However, a positive biopsy result must always be correlated with all the other diagnostic criteria to prove the exact diagnosis (Tab. 1, Fig. 4, Ref. 49). Text in PDF www.elis.sk Keywords: Sjogren syndrome, keratoconjunctivitis sicca, xerostomia, labial minor salivary glands, biopsy, focal lymphocytic infiltration, focus score.

Purpose To compare the burden related to dry eye with systemic symptoms of Sjogren syndrome; to estimate the burden related to ocular treatments; and to compare the impact of dry eye and extraocular manifestations of Sjogren syndrome on various aspects of patient life. Design Cross-sectional study. Methods We conducted a postal survey of adult patients with a history of physician-diagnosed Sjogren syndrome. Results The survey was completed by 2,961 patients (mean age 65.1 years, standard deviation 11.7 years), most of whom were women (96%) and white (94%). Forty-one patients younger than 18 years of age were excluded. More than half (53%) experienced severe dry eye (ie, dry eye daily/almost daily with major impact on their life). Corresponding proportions for dry mouth and fatigue were 48% and 45%, respectively. Almost all patients (97%) had used nonprescription eye drops/artificial tears/ointments. Compared with patients who did not experience dry eye, those who experienced significant dry eye (ie, daily/almost daily dry eye) more often agreed that living with Sjogren syndrome made every day a challenge (adjusted odds ratio [OR] 3.81, 95% confidence interval [CI] 2.49 to 5.86) and added a significant emotional burden (adjusted OR 2.22, 95% CI 1.49 to 3.31). Adjusted ORs for the impact of dry eye were generally lower than those for fatigue, but were similar to dry mouth and considerably higher than use of systemic treatments for serious manifestations of the disease and diagnosis of lymphoma. Conclusions Sjogren-related dry eye is more burdensome than systemic manifestations of the disease. While fatigue has the greatest impact on patient life, the impact of dry eye is comparable to that of other systemic manifestations.

Pulmonary involvement in Sjogren syndrome (SS) could manifest as cystic lung disease (CLD). CLD in SS includes lymphocytic interstitial pneumonia (LIP) and pulmonary amyloidosis. Differential diagnosis usually requires surgical lung biopsy, whereas CT-guided percutaneous fine needle aspiration biopsy (CT-FNAB) has not yet explored. We describe the case of a 63-year-old never smoker Caucasian female with a SS diagnosis who displayed a newly detected diffuse CLD at high-resolution computed tomography, though totally asymptomatic. Given the favorable location of one big lesion at the superior left lobe, a CT-FNAB was proposed instead of a more invasive SLB. At histology examination a diagnosis of pulmonary nodular AL kappa amyloidosis in the context of SS was established. In conclusion, CT-FNAB might represent an alternative and less invasive diagnostic procedure than SLB in the differential diagnosis of CLD, even if further research is needed. Moreover, this case presents an unusual association between SS and pulmonary nodular AL kappa amyloidosis, with pulmonary nodules and cysts without systemic manifestations.

Objectives: This study aims to assess the reliability and validity of the Turkish version of the European League Against Rheumatism (EULAR) Sjögren Syndrome Patient Reported Index (ESSPRI) (TR) in patients with primary Sjögren syndrome (pSS). Materials and methods: A cross-sectional survey study design and analysis were used to assess the reliability and validity of the ESSPRI (TR) between March 2019 and July 2019. A total of 30 patients (5 males, 25 females; mean age 54.1±10.5 years; range, 18 to 75 years) diagnosed as pSS according to revised American College of Rheumatology (ACR)/EULAR classification criteria were included. ESSPRI (TR) was applied to the patients with face-to-face interviews twice: on their first visit and after an interval of 15 days. The test-retest reliability was assessed with the intraclass correlation coefficient (ICC), and the internal consistency of the multi-item subscales by calculating Cronbach alpha values. The correlations between basal and stimulated salivary flow (BSF and SSF), Oral Health Impact Profile-14 (OHIP-14) and Oral Health-Related Quality of Life-UK (OHRQOL-UK) questionnaires were evaluated to determine the construct validity. Results: The ICC value for the test/retest reliability of ESSPRI (TR) was 0.925. The internal consistency was 0.682. There were low to moderate correlations between the ESSPRI (TR) total score and BSF (-0.39), SSF (-0.50), OHIP-14 total (0.57) and OHRQOL-UK total (-0.67). Conclusion: The Turkish version of the ESSPRI was found to be clinically valid and reliable to be used in clinical evaluations and rehabilitation interventions in patients with pSS.

RESUMEN El síndrome de Sjögren primario (SSp) es una enfermedad autoinmune que afecta principalmente al tejido glandular. A pesar de ello, puede involucrar otros sistemas, siendo el compromiso neuropsiquiátrico una manifestación extraglandular común. Su presentación clínica varía ampliamente según el dominio que se encuentre afectado, y por tanto puede dividirse en tres grandes categorías: sistema nervioso central, sistema nervioso periférico y psiquiátrico. Algunas de estas complicaciones comparten mecanismos fisiopatológicos comunes, entre los principales la vasculitis/vasculopatía, la infiltración linfocítica y la presencia de anticuerpos antineuronales. La diversidad en la presentación clínica de esta entidad impide hacer una aproximación diagnóstica común, por lo cual la utilización de estudios específicos depende de un adecuado reconocimiento y de la localización por parte del clínico. El tratamiento debe dirigirse al mecanismo fisiopatológico implicado y, de acuerdo con el tipo de manifestación, puede incluso estar limitado al manejo sintomático. ABSTRACT Primary Sjögren's syndrome is an autoimmune disease that mainly involves glandular tissue. Despite this, it can potentially develop systemic involvement, within which neuropsychiatric manifestations are common. The clinical presentation may vary widely depending on the domain affected, and may thus be classified into three categories: central nervous system, peripheral nervous system, and psychiatric. Some of these complications share a common pathophysiology, amongst which are vasculitis/ vasculopathy, lymphocytic infiltration and positive antineuronal antibodies. The wide clinical presentation makes it difficult to establish a common diagnostic approach, making it essential for the clinician to recognise and localise the type of compromise, so that diagnostic tools can be more advantageously employed. Treatment must be directed towards the underlying pathophysiology, and depending on the type of compromise, it can even be limited solely to the management of symptoms.

Purpose of review This article discusses peripheral neuropathies associated with vasculitis (isolated or in the setting of systemic vasculitis) and autoimmune connective tissue disease and provides a brief overview of their diagnostic evaluation and management. Recent findings The classification of systemic vasculitic neuropathy and nonsystemic vasculitic neuropathy continues to evolve. Classification according to the presence of antineutrophil cytoplasmic antibodies and their subtypes facilitates prognostication and management. Recent research on antineutrophil cytoplasmic antibody-associated vasculitis has added to our understanding of its neurologic complications. The treatment of vasculitis is also evolving, and new nonsystemic vasculitic neuropathy classification has impacted the treatment and management of this disorder. New classification criteria for Sjogren syndrome (which commonly causes neurologic complications) facilitate accurate and timely diagnosis. Summary Vasculitis and autoimmune connective tissue disease are underrecognized and treatable causes of peripheral neuropathy. Furthermore, peripheral neuropathy may reveal an underlying rheumatologic or vasculitic disorder. Rapid recognition and treatment are essential. Familiarity with the diagnosis and treatment of neuropathies in the setting of connective tissue disease and vasculitis reduces morbidity and, in some cases, mortality.

PurposeThe goal of this study is to determine a link between benign essential blepharospasm and Sjogren's syndrome by analyzing the presence of extractable nuclear antigens in this population.MethodsSeventy-two patients with benign essential blepharospasm (BEB) were included in this study. We eliminated patients with hemifacial spasm or blepharospasm secondary to corneal pathology. We collected the values of the Schirmer I test and the results of the anti-SSA and anti-SSB antibodies.ResultsOur study included 72 patients (144 eyes) whose 62 women (86.1%). Mean age was 74.3 years ± 10.73. Average Schirmer I test was 3.14 mm ± 4.00 mm. Five women (8% of this female population) had positive anti-SSA and SSB antibodies. Their mean age was 65.66 years ± 13.24 whereas the negative antibody patients had an average age of 75.42 ± 9.27. There was no significant difference between their Schimer I test and the Schirmer I of negative antibody population.ConclusionThis study illustrates the possible association between the presence of Sjögren's syndrome and the occurrence of a BEB justifying the search for anti-SSA and anti SSB in blepharospasm patients., ObjectifÉtudier s’il existe un lien entre le blépharospasme essentiel et le syndrome de Sjögren en analysant la présence d’anticorps nucléaires solubles dans cette population.MéthodesSoixante douze patients atteints de blépharospasme essentiel (BSE) ont été inclus dans cette étude. Nous avons éliminé les patients atteints de spasme hémifacial, ou de blépharospasme secondaire à une pathologie cornéenne avérée. Nous avons recueilli les valeurs des test de Schirmer I et les résultats des anticorps anti-SSA et anti-SSB.RésultatsNotre étude a regroupé 72 patients (144 yeux) dont 62 femmes (86,1 %). L’âge moyen était de 74,3 ans ± 10,73. Le test de Schirmer I était en moyenne de 3,14 mm ± 4,00 mm. Cinq femmes (8 % de cette population féminine) ont révélés des anticorps anti-SSA et SSB positifs. Leur moyenne d’âge était de 65,66 ans ± 13,24 alors que les patients anticorps négatifs avaient une moyenne d’âge de 75,42 ± 9,27. Il n’existait pas de différence statistiquement significative entre leur test de Schimer I et celui des patients anticorps négatifs.ConclusionCette étude illustre la possible association entre la présence d’une maladie de Sjögren et la présence d’un BSE justifiant la recherche d’anticorps anti-SSA et SSB chez ces patients.

ObjectiveTo investigate salivary gland ultrasound (SGUS) abnormalities in relation to clinical phenotype and patient characteristics, disease activity, and disease damage in patients with primary Sjögren syndrome (pSS).MethodsConsecutive outpatients included in our REgistry of Sjögren Syndrome LongiTudinal (RESULT) cohort were selected. Patients with pSS who were included were classified according to the American College of Rheumatology/European League Against Rheumatism (EULAR) criteria and underwent full ultrasonographic examination (Hocevar score 0–48) at baseline. Total SGUS scores of ≥ 15 were considered positive. Patient characteristics, disease activity, and disease damage were compared between the different SGUS groups.ResultsIn total, 172 of 186 patients with pSS were eligible, of whom 136 (79%) were SGUS positive. Compared with patients who were SGUS negative, SGUS-positive patients had significantly longer disease duration, higher EULAR Sjögren Syndrome Disease Activity Index, higher Sjögren Syndrome Disease Damage Index, and were more likely to have a positive parotid gland biopsy, anti-SSA/SSB antibodies, and abnormal unstimulated whole saliva (UWS) and ocular staining score (OSS), and higher levels of IgG and rheumatoid factor. Regarding patient-reported outcome measurements (PROM), patients who were SGUS positive scored significantly lower on the EULAR Sjögren Syndrome Patient-Reported Index for fatigue and pain, and more often found their disease state acceptable compared with patients who were SGUS negative. SGUS total score showed significant associations with various clinical and serological variables, and with PROM. Highest associations were found for UWS (ρ = –0.551) and OSS (ρ = 0.532).ConclusionPatients who were SGUS positive show a distinct clinical phenotype in all aspects of the disease compared with patients who were SGUS negative: clinical, functional, serological, and PROM. SGUS could be a helpful tool in selecting patients for clinical trials and estimating treatment need.

BACKGROUND Celiac disease (CD) is a systemic, chronic immune-mediated disease triggered by gluten ingestion in genetically-susceptible individuals, with a prevalence of 1% worldwide. Sjogren's syndrome (SS) is also a systemic autoimmune disease, mainly characterized by ocular and oral sicca symptoms and signs. Sharing a common genetic background, CD and SS are known associated autoimmune diseases, but currently available guidelines are not reporting it. CASE SUMMARY We report the case of a 39-year-old woman, who was in the care of her rheumatologist for 2 years with SS. On routine follow-up she was found to have iron deficiency, without anemia. She had no gastrointestinal complaints and denied any obvious source of blood loss. IgA tissue transglutaminase antibodies were positive and endoscopy with duodenal biopsies revealed crypt hyperplasia and villous atrophy. A diagnosis of CD was set and gluten-free diet was recommended. CONCLUSION We present a review of existing data in the literature regarding the association of the two diseases, summarizing prevalence studies of CD in SS patients and the other way around. Screening recommendations and future research perspectives are also discussed, highlighting clinically relevant unanswered questions with respect to the association of CD with SS. publishedVersion

Objective Menopausal women appear to report a higher risk of Sjogren syndrome (SS). Although Chinese herbal medicines (CHMs) are proven to lower SS risk, the scientific evidence of whether it can lessen the occurrence of SS among menopausal women is limited. This longitudinal cohort study aimed to clarify the relationship between CHMs use and SS risk in menopausal women. Methods Using a nationwide claims data, we enrolled 31,917 women with first-time diagnosed menopause who simultaneously were free of SS between 2000 and 2007. Among them, we randomly selected 12,757 CHMs users and 12,757 non-CHMs users using propensity scores matching. All participants were followed until the end of 2012 to record SS incidence. The hazard ratio of SS with regard to CHMs use was estimated using the Cox proportional hazards regression model. Results In the follow-up period, 589 CHMs users and 644 non-CHMs users developed SS, representing incidence rates of 5.12 and 6.40, respectively, per 1,000 person-years. CHMs use was associated with a 21% lower subsequent risk of SS (adjusted hazard ratio, 0.79; 95% CI, 0.71-0.89). Six commonly prescribed CHMs were discovered to be associated with lower SS risk: Ge-Gen-Tang, Zhi-Gan-Cao-Tag, Da-Huang, Ye-Jiao-Teng, Tian-Hua-Fen, and Bo-Zi-Ren. Conclusions A statistically significant association was found between CHMs use and lower risk of SS onset in menopausal women, suggesting that CHMs could be considered to integrate it into conventional therapy to reduce subsequent SS risk for menopausal women.

Objective Sjogren syndrome is a systemic inflammatory disease causing gland dysfunction. Few (and contradictory) reports on the mucosal effects of Sjogren syndrome have appeared. Here, we objectively demonstrate nasal dryness in Sjogren syndrome patients and explore the effect of such dryness on olfaction. Methods Thirty-four consecutive patients with primary Sjogren syndrome were enrolled in this cross-sectional study. The control group consisted of 21 age- and sex-matched volunteers. Medical histories and nasal findings were recorded. The Connecticut Chemosensory Clinical Research Center test was used to evaluate olfactory function. All subjects underwent mucucociliary clearance analysis (the saccharin test and peak nasal inspiratory flowmetry). The intranasal Schirmer test was used to evaluate the nasal cavity. Results The nasal Schirmer test scores were 8.4 mm (right) and 8 mm (left) (P = .041, P = .016, respectively, compared to controls). The Chi-squared test revealed significant differences (compared to controls) in nasal dryness (P = .001), postnasal drip (P = .04), and smell (a decrease) (P = .005). Neither olfactory function nor mucociliary clearance differed between the groups. We noted a trend toward a positive correlation between olfactory function and the nasal Schirmer score but statistical significance was not attained. Conclusion The intranasal Schirmer test objectively showed that Sjogren syndrome patients exhibited nasal cavity dryness; this is useful in terms of follow-up. This did not affect olfactory function. Level of evidence 4 Laryngoscope, 131:370-373, 2021.

Soft tissue calcifications associated with various connective tissue diseases such as dermatomyositis and scleroderma have been well documented Plaque-like sheets of subcutaneous calcifications presenting as an indurated soft tissue mass in a patient with primary Sjogren syndrome have been rarely documented in the literature. We present the magnetic resonance and conventional radiographic findings of calcinosis cutis and calcinosis circumscripta of a 47-year-old woman with biopsy proven Sjogren syndrome. We also delineate various types of soft tissue calcification, histopathology of calcinosis cutis, and current treatment options. Recognizing the magnetic resonance characteristics of this phenomenon may prove useful to radiologists, especially in the absence of clinical history and conventional radiographs.

Sjogren's syndrome is a systemic connective tissue disease characterized by pronounced clinical polymorphism, which can be encountered in outpatient practice by doctors of various specialties. The article discusses the problems of the resistant course of gastroesophageal reflux disease, which developed against the background of the primary Sjogren syndrome, on the example of clinical observation. The role of in-depth examination of the patient for the correct clinical diagnosis and the involvement of a team of specialists to achieve remission of Sjogren's syndrome is emphasized.

Xerostomia is a self-conscious symptom. High-mobility group box 1 (HMGB1) promotes pro-inflammatory effects in many diseases. This study aimed to clarify the role of HMGB1 in Sjögren syndrome (SS)-triggered xerostomia. Nonobese diabetic (NOD)/Ltj mice were used to establish an SS-triggered xerostomia model. The results showed that saliva production was decreased and anti-Sjögren syndrome B (anti-SSB) level was increased in SS. PCR, Western blot, and immunohistochemistry experiments indicated that the HMGB1 and aquaporin 5 (AQP5) levels were enhanced and diminished in SS compared with those in the control, respectively. While the mice were treated with anti-HMGB1, xerostomia was reversed due to the elevated saliva production and reduced anti-SSB level. In addition, it was found that the inhibition of HMGB1 restrained the toll-like receptor 4 (TLR4)/nuclear factor kappa B (NF-κB) axis activation. The TLR4 and p-IκB levels were alleviated, while the IκBα and NF-κB p65 levels were augmented. The NF-κB p65 binding activity was attenuated via the electrophoretic mobility shift assay (EMSA) after anti-HMGB1 treatment. Moreover, the repression of HMGB1 facilitated the expression of AQP5. These findings demonstrate that suppression of HMGB1 ameliorates SS-triggered xerostomia via suppressing the HMGB1/TLR4/NF-κB signaling pathway and upregulating AQP5 expression.

Antinuclear antibodies (ANA) and extractable nuclear antigens (ENA) are instrumental biomarkers crucial in the detection of autoimmune disorders (AID) such as systemic lupus erythematosus (SLE), Sjogren syndrome, etc.. In the present study, an assessment of the most frequent ANA patterns associated with most detectable ENA that could be used as efficient prognostic markers in the diagnosis of autoimmune diseases was conducted. Data was retrospectively analyzed from AID patients, retrieved from the medical records of King Fahad Medical City, Riyadh, KSA, from January 2016 to October 2018 who underwent ANA immunofluorescence of HEp-2 cells and their ENA detection was studied. Of the 453 total patients, 39/55 AID males (71%) and 332/398 AID females (83.4%) exhibited ANA positivity. The most common pattern was speckled S-ANA (32.4%) in females and homogenous H-ANA pattern (25.4%) in males. The histones were found at higher frequency in different ANA patterns. anti-Sjogren syndrome related antigen A (SSA), anti-ribonucleoprotein antibody (RNP-Sm), and histones were observed to be associated with homogenous and speckled nuclear patterns. Frequencies of ENA in all ANA patterns were found significant at p < 0.05 in males and p < 0.001 in females. Spearman's rank correlation of ENA within and among the ANA patterns was non-significant. SSA was significantly correlated with RNP-Sm and Sm at p < 0.05 and p < 0.01, respectively. The extractable nuclear antigens SSA, RNP-Sm, and histones were found associated with the S-ANA and H-ANA patterns. These correlations are of relevance for the accurate diagnosis of autoimmune diseases.

Objective.Major salivary gland ultrasonography (SGUS) is a widely used imaging technique to evaluate salivary gland involvement in primary Sjögren syndrome (pSS). The aim of this study was to evaluate the relationship between SGUS, salivary flow rate (SFR) as an objective measure of the gland function, and oral health-related quality of life (OHRQOL) as a patient-reported outcome measure (PROM) in a pSS cohort.Methods.Sixty-six patients with pSS were examined by SGUS according to Hocevar and Milic scoring systems. Patients with inhomogeneity/hypoechoic areas with scores ≥ 2 in parotid and submandibular glands were classified separately as “severe glandular involvement.” Further, oral health, SFR, and Oral Health Impact Profile-14 (OHIP-14) for OHRQOL were assessed.ResultsBoth total Hocevar and Milic scores were higher in 21 pSS patients with low unstimulated whole salivary flow rate (U-WSFR) than 45 pSS patients without low U-WSFR (P = 0.001 and P < 0.0001, respectively). Increased scores of homogeneity, hypoechoic areas and glandular border visibility were observed in patients with low U-WSFR (P < 0.05). Among these variables, homogeneity score was found to be an independent risk factor for low U-WSFR in pSS according to logistic regression analysis (OR 1.586, P = 0.001). Moreover, a higher OHIP-14 score was observed in severe parotid involvement compared to nonsevere cases (23.26 ± 21.19 vs 8.32 ± 13.82, P = 0.004).Conclusion.High Milic and Hocevar SGUS scores are associated with reduced SFR and poor OHRQOL as a PROM. The inhomogeneity component of the SGUS score is associated with low U-WSFR and is an indicator of severely affected gland function.

A 52-year-old woman was diagnosed as having anti-centromere antibody (ACA)-positive primary Sjögren syndrome (pSS). Eight years later, she visited our hospital because she had developed dyspnoea. She was diagnosed as having pulmonary arterial hypertension (PAH) with pulmonary veno-occlusive disease on the basis of the results of right heart catheterisation, a severe decrease in diffusing capacity of the lung for carbon monoxide (D

Only proven pathogenic mutations associated with myeloid neoplasms are key to establish the clonal nature of the bone marrow fibrosis. In cases with genetic variants of uncertain meaning, the clinical picture may be required to rule out secondary causes.

Background Gourgerot-Sjögren syndrome (GSS) is a systemic autoimmune disease characterized by lymphocytic infiltration of the exocrine glands leading to dryness of the mucous membranes. It may also involve other organs and organ systems. GSS can be primary (pGSS) or secondary due to other autoimmune diseases. pSGS mainly affects women over 40 years old, but can occur at any age. GSS is rare in children and is often secondary to other diseases. We report the case of a 12-year-old girl with primary Gougerot-Sjögren Syndrome Methods and results A 12-year-old girl from Batna (Algeria) presented to her pediatrician in 2016 for a rash with arthralgia. She had no relevant past medical history and is the child of a non-consanguineous marriage. Clinical examination found a well-nourished child with a purpuric rash on her lower limbs. She complained of arthralgia without clinical signs of arthritis. The child also complained of a sensation of a foreign body and burning in both eyes. The eye exam was normal except for a positive Schirmer's test. There were no signs of parotitis. Laboratory tests showed an erythrocyte sedimentation rate (ESR) of 84 mm the first h and a negative C-reactive protein (CRP). She was also Leukopenic. Serological testing resulted in a positive Anti SS-A and Anti SS-B. The abdominal pelvic ultrasound was normal. The patient was diagnosed with primary Gougerot-Sjögren Syndrome. Artificial tears and hydroxychloroquine were started as initial therapy. One year later we added an immunosuppressant (Methotrexate) due to the persistence of clinical signs. Conclusion Our case is particular in that primary Gougerot-Sjögren Syndrome is rare in children. Routine follow- up with this patient is important to determine whether it is indeed pGSS with pediatric onset or GSS secondary to another autoimmune disease.

We describe a case of Sjögren's syndrome-associated trigeminal neuropathy with mandibular nerve enhancement at the foramen ovale and Gasserian ganglion (Meckel's cave) in a patient with a prior history of breast cancer. We also explore the differential diagnosis of trigeminal neuropathy associated with mandibular nerve involvement at the foramen ovale and exclude other diseases such as Sjögren's syndrome or perineurial invasion as a result of various carcinomas. We emphasize the importance of an investigative triad of searching for a local head-and-neck malignancy that may metastasize by perineural spread or invasion, excluding a distant malignancy or paraneoplastic phenomenon and ruling out an autoimmune etiology such as Sjögren's syndrome. In the process, we briefly outline the basic immunopathologic processes.

Objectives Decreased blink rate during video display viewing (VDV) has been reported; however, patients with tear dysfunction often complain of more frequent blinking. The objectives were to compare blink rates during conversation and VDV in normal and dry eyes and correlate blink rates with signs and symptoms. Methods Blink rate was measured with an infrared blink sensor for 2 minutes during conversation and for 2 minutes while watching a video clip (VDV) in patients without dry eye disease (control, n=18) and in patients with tear dysfunction from meibomian gland dysfunction (MGD, n=23), conjunctivochalasis (CCh, n=19), and aqueous tear deficiency (n=34; non-Sjogren syndrome n=18 and Sjogren syndrome n=16). Patients completed visual analogue questionnaires assessing irritation frequency and severity and blink frequency and underwent an ocular surface evaluation. Group comparisons and correlations were calculated. Results Compared with control, conversational and VDV blink rates were significantly higher in CCh and aqueous tear deficiency (P≤0.005). Self-reported blink frequency was higher in all tear dysfunction groups compared with control. The VDV blink rate was significantly lower than the conversation blink rate in the control group (P=0.006) but not in any of the tear dysfunction groups. Conversational and VDV blink rates were significantly correlated with irritation frequency and severity. Conclusions Blink rate decreases during VDV in normal eyes; however, this blink rate suppression was not observed in patients with tear dysfunction, perhaps because of their irritation symptoms or tear instability.

Jonathan Lilley,1 Erin C O’Neil,1 Vatinee Y Bunya,1 Kennedy Johnson,1 Gui-Shuang Ying,2 Peiying Hua,2 Mina Massaro-Giordano1 1Department of Ophthalmology, Scheie Eye Institute, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA; 2Center for Preventive Ophthalmology and Biostatistics, University of Pennsylvania, Philadelphia, PA, USACorrespondence: Mina Massaro-GiordanoDepartment of Ophthalmology, Scheie Eye Institute, Perelman School of Medicine at the University of Pennsylvania, 51 N. 39th Street, Philadelphia, PA, 19104, USATel +1 215-662-9393Email mina@pennmedicine.upenn.eduPurpose: To assess the efficacy and safety of an intranasal tear neurostimulator (ITN) device in Sjögren syndrome (SS) patients.Methods: This was a two-visit prospective, randomized, controlled, same-day crossover study in participants with SS. Inclusion criteria were assessed at a baseline screening visit and included an Ocular Surface Disease Index (OSDI) score ≥ 13, and a Schirmer with anesthesia ≤ 10 mm/5 min (in at least one eye), with a cotton swab stimulation induced increase of ≥ 4 mm in the same eye. Participants returned for the application visit, where they received intranasal and extranasal applications of the ITN in random sequence, separated by at least 60 min. Schirmer scores were measured in both eyes after each application and compared to baseline values. Generalized linear models were performed to compare the change in Schirmer scores from baseline, and generalized estimating equations were used to account for correlations from repeated measurements in the same eye and measurements from two eyes of the same patient.Results: Fifty-five participants were screened and 35 were enrolled (all female), ranging in age from 31 to 72 years (mean, 57 years). The baseline OSDI score ranged from 14 to 91 (mean, 50.5), and the baseline Schirmer score had a mean (SD) of 6.4 (3.5) ranging from 0 to 20 (mm/5min). Improvement in Schirmer scores was significantly greater for intranasal device application (13.5 mm/5min, 95% CI: 10.4, 16.5) compared to extranasal device application (0.8mm/5min, 95% CI: − 0.9, 2.4) (p< 0.0001). The effects of the intranasal device application were significant regardless of the participant’s baseline Schirmer score and systemic SS medication usage (p< 0.05).Conclusion: Intranasal application of the ITN device significantly increased tear production in a subset of SS patients compared to baseline and was more effective than extranasal application. While production of the ITN device was recently discontinued, our findings suggest that other therapies that neurostimulate the lacrimal function unit may be effective in a subset of SS patients.Keywords: Sjögren syndrome, intranasal tear neurostimulation, tear production

Background Cerebellar degeneration is a rare and severe presentation of primary Sjögren syndrome. There are few case reports of cerebellar degeneration associated with different autoimmune diseases, especially with systemic lupus erythematosus and neuro-Behcet’s disease. There are only six patients reported worldwide to be affected by cerebellar atrophy associated with primary Sjögren syndrome. In this report, we describe a patient with primary Sjögren syndrome who presented with ataxia due to cerebellar degeneration. Case presentation We report the case of a 37-year-old Chinese woman with primary Sjögren syndrome who presented with ataxia over 3 months associated with tremor of the limbs. Magnetic resonance imaging of the brain revealed bilateral cerebellar atrophy. Based on the presence of cerebellar signs with magnetic resonance imaging brain findings, she was diagnosed as cerebellar degeneration secondary to primary Sjögren syndrome. She was treated with methylprednisolone, hydroxychloroquine, and two cycles of monthly intravenous cyclophosphamide. Subsequently, she refused further treatment, and her neurological symptoms remained the same upon the last clinic review. Primary cerebellar degeneration is rarely associated with primary Sjögren syndrome. The pathogenesis of the neurological manifestations in primary Sjögren syndrome is unclear. Treatment involves corticosteroids and immunosuppressive agents with no consensus of a specific therapy for the management of primary Sjögren syndrome with central nervous system involvement. Conclusions Cerebellar degeneration is a rare presentation of primary Sjögren syndrome. Early diagnosis and treatment of this condition is needed to ensure a good outcome.

Autoimmune connective tissue diseases (ACTD) are a broad spectrum of diseases including dermatomyositis, systemic lupus erythematosus, Sjogren syndrome, systemic sclerosis, and mixed connective tissue diseases. Pruritus is an unpleasant sensation leading to scratching. It is most commonly seen in dermatomyositis patients among ACTD. Itch can be mild to severe, hampering daily activities. It is often associated with disease severity. Apart from ACTD, pruritus may be due to antimalarial agents or due to adverse effects of medications used in ACTD. Therefore, it is prudent to find the pathogenesis of pruritus for adequate treatment. Pruritus in ACTD is underreported and undertreated. The article gives a comprehensive view of pruritus in ACTD, its pathogenesis, and management.

RESUMEN Introducción: El síndrome de Sjögren (SS) es una enfermedad autoinmune, con compromiso multisistémico, especialmente de la función de las glándulas exocrinas. Los pacientes cursan con una alta carga de la enfermedad que compromete su calidad de vida, asociado sobre todo a fatiga crónica, ansiedad, depresión y disfunción sexual, entre otras dificultades. Objetivo: Describir las generalidades de las principales escalas (específicas y no específicas) para la evaluación de la calidad de vida en pacientes con SS. Métodos: Se realizó una revisión narrativa y ampliada de la literatura. Se hizo una búsqueda en las diferentes bases de datos (PubMed, Embase, Cochrane, BVS) y de literatura gris, seleccionando los artículos relacionados con el tema y realizando una extracción de datos de estos. Resultados: Se encontraron 3 escalas realizadas específicamente para pacientes con SS: Sjögren’s Syndrome Patient Reported Index, Psychometric Testing of the Primary Sjögren’s Syndrome Quality of Life Questionnaire y Profile of Fatigue and Discomfort, y 14 escalas realizadas en el contexto de otras enfermedades (no específicas), utilizadas en pacientes con SS. Conclusiones: Como en otras afecciones, en pacientes con SS es importante la evaluación de la calidad de vida. A tal efecto se cuenta con múltiples escalas, a parte de las 3 citadas, elaboradas específicamente para este tipo de pacientes, sin dejar de lado las escalas no específicas, como el Beck Depression Inventory y el Ocular Surface Disease Index, que tienen su mayor utilidad en la evaluación específica de síntomas asociados a esta enfermedad. Resultados: Se encontraron 3 escalas realizadas específicamente para pacientes con SS: Sjögren’s Syndrome Patient Reported Index, Psychometric Testing of the Primary Sjögren’s Syndrome Quality of Life Questionnaire y Profile of Fatigue and Discomfort, y 14 escalas realizadas en el contexto de otras enfermedades (no específicas), utilizadas en pacientes con SS. ABSTRACT Introduction: Sjögren’s syndrome (SS) is an autoimmune disease, with multisystemic invol- vement, particularly compromising the function of exocrine glands. The patients have a high burden of the disease associated with chronic fatigue, anxiety, depression, sexual dysfunction, among others. Objective: To describe the generalities of the main scales (specific and non-specific) for the evaluation of quality of life in patients with SS. Methods: A narrative review and expanded review was performed, including a search conducted in the different databases (PubMed, Embase, Cochrane, VHL) and grey literature. Articles related to the topic were selected and data extracted from them. Results: Three scales were developed specifically for patients with SS, and included Sjögren’s Syndrome Patient Reported Index, Psychometric Testing of the Primary Sjögren’s Syndrome Quality of Life Questionnaire, and Profile of Fatigue and Discomfort, and 14 scales performed in context of other diseases (non-specific) used in patients with SS. Conclusions: As is the case in other diseases, it is important to evaluate the quality of life in patients with SS. There are multiple scales for this evaluation, including the 3 scales mentioned before, specifically developed for these types of patients. The non-specific scales worth noting are, the Beck Depression Inventory and the Ocular Surface Disease Index, having their greatest use in the specific evaluation of symptoms associated with this disease.

Introduction. The aim of this randomized, observer-masked, parallel group study was to evaluate the short-term and long-term effects of topical hydrocortisone administered in addition to topical ciclosporin A for the first 2 weeks of the treatment in patients with dry eye disease associated with Sjögren syndrome. Materials and Methods. 24 eyes of 12 patients with severe dry eye disease associated with Sjögren syndrome were included in this study. Both eyes of all patients were treated with preservative-free Ciclosporin A eye drops once daily for 6 months. Additionally, one eye of each patient received hydrocortisone eye drops three times daily for the first two weeks of treatment. The study parameters were assessed before treatment, after 2 weeks, and after 6 months of treatment. Results. Tear BUT and corneal fluorescein Oxford staining grade showed significant differences with respect to the baseline when treated with ciclosporin A and hydrocortisone (CsA + Hc) and a nonsignificant increase when treated with ciclosporin A (CsA) alone. After 6 months of treatment, significant increases of tear BUT and corneal Fluorescein Oxford staining grade compared to baseline could be observed in both treatment groups. Aberrometry measurements showed significantly increased optical image quality after 6 months in the CsA + Hc group, while no significant changes could be detected in the eyes treated with CsA alone. However, no significant differences between the two treatment groups could be detected. Discussion. This study indicates that hydrocortisone combined with ciclosporin A therapy may provide fast improvement of clinical symptoms and could have long-term positive effects on the optical image quality in severe DED patients with Sjögren syndrome.

AimTo describe salivary gland involvement in patients suspected of Sjögren’s syndrome (SS) using the OMERACT Ultrasound Scoring System for SS. Next, using different ultrasound cut-offs, to assess the performance of the scoring system for diagnosis and fulfilment of 2016 ACR/EULAR SS classification criteria.MethodsAll patients referred to our department with a suspicion of SS in a 12-month period were included. All underwent grey-scale ultrasound of the parotid and submandibular glands prior to clinical examination, Schirmer’s test, unstimulated salivary flow, blood samples including autoantibody analysis. Labial biopsy was performed according to clinicians’ judgement. Images of the four glands were scored 0–3 according to the scoring system and a consensus score was obtained using a developed ultrasound atlas.ResultsOf the 134 patients included in the analysis, 43 were diagnosed with primary SS (pSS) and all fulfilled the 2016 American College of Rheumatology (ACR)/EULAR classification criteria. More patients with pSS compared with non-pSS had score ≥2 in at least one gland (72% vs 13%; pConclusionThe OMERACT Ultrasound Scoring System showed good sensitivity (0.72) and excellent specificity (0.91) for fulfilling 2016 ACR/EULAR criteria using cut-off score >2 in at least one gland. Our data supports the use of ultrasound for diagnosing pSS and supports incorporation of ultrasound in the classification criteria.