Unstimulated whole salivary flow in Sjögren's Syndrome: systematic literature review and meta-analysis

Abstract Background: Sjögren's Syndrome compromises the exocrine function, producing xerostomia and xerophthalmia. It can appear as an isolated condition or associated with other autoimmune diseases (polyautoimmunity). The Unstimulated Salivary Flow rate (UWSF) is used to quantify saliva production. There is no objective evidence to differentiate the values in patients with Sjögren's versus healthy people or patients with non-Sjögren's sicca. The objective of the present review was to evaluate the UWSF in patients with Sjögren's syndrome in comparison to controls (healthy and non-Sjögren's sicca patients). Methods: A systematic literature review was carried out (PRISMA guidelines). Analytical observational studies of cases and controls, cross-sectional studies, cohort studies and randomized clinical trials (including healthy controls) were considered. The Medline/OVID, Lilacs, Embase, and Cochrane/OVID databases were consulted. MeSH, DeCS, keywords, and Boolean operators were used. The meta-analysis (RevMan 5.2) was done through the random-effects model [mean difference (MD)]. Level and quality of evidence were evaluated by the Oxford Center Levels of Evidence and Joanna Brigs list respectively. Results: Thirty-two articles were included (20 were case-control studies,6 were cross-sectional,2 prospective cohort,2 retrospective cohort, and2 studies were abstracts) and 28 were meta-analyzed. The unstimulated whole salivary flow rate in the Sjögren's group was lower than in controls (healthy and patients with non-Sjögren Sicca syndrome) (MD-0.18 ml/min; 95% CI, −0.24 to −0.13; chi2-P-value <0.00001). Heterogeneity was 97% and there was publication bias (funnel plot). The level of evidence was mostly3 or 4. The quality of evidence was met (97% of items valued). Conclusion: For the first time, the unstimulated whole salivary flow rate is found to be lower in patients with Sjögren's syndrome compared to controls (healthy and non-SS sicca) through a meta-analysis. (AU)

Evaluation of corneal layers and anterior sclera in patients with primary Sjögren's syndrome / Avaliação das camadas da córnea e da esclera anterior na síndrome de Sjögren primária

ABSTRACT Purpose: We aimed to compare the thickness of anterior sclera, corneal layers, and pre-ocular tear film between patients with primary Sjögren's syndrome and healthy individuals. Methods: Fifty-one patients with primary Sjögren's syndrome and 41 healthy control participants were recruited in this cross-sectional and comparative study. The thickness of the pre-ocular tear film, corneal epithelium, Bowman's layer, stroma, Descemet's membrane, and endothelium were measured on the corneal apex. Anterior scleral thickness was measured at distances of 1 mm and 3 mm from the limbus. The anterior segment module of spectral-domain optical coherence tomography was used to measure thicknesses of pre-ocular tear film, corneal layers, and anterior sclera. Results: Tear film thickness, Schirmer's test, and tear break up time values were significantly lower in the Sjögren's disease group than in the healthy controls (p<0.05). The thickness measurements of corneal layers and sclera were similar between the groups. Tear film thickness was moderately correlated with the Schirmer's test results (r=0.34, p=0.001), but there was no correlation between the Schirmer's test results and tear break up time (r=0.18, p=0.09). Conclusions: Pre-ocular tear film, as measured by anterior segment optical coherence tomography, was thinner in patients with primary Sjögren's syndrome than in the healthy controls. The thicknesses of corneal layers and anterior sclera were similar between the groups.

RESUMO Propósito: Nosso objetivo foi comparar a espessura da esclera anterior, camadas da córnea e do filme lacrimal pré-ocular entre pacientes com síndrome de Sjögren primária e indivíduos saudáveis. Métodos: Cinquenta e um pacientes com síndrome de Sjögren primária e 41 controles saudáveis foram recrutados neste estudo comparativo e transversal. A espessura do filme lacrimal pré-ocular, epitélio corneal, camada de Bowman, estroma, membrana de Descemet e endotélio foram medidos no ápice corneal. A espessura da esclera anterior foi medida às distâncias de 1 mm e 3 mm do limbo. O módulo do segmento anterior da tomografia de coerência óptica de domínio espectral foi utilizado para mensurar as espessuras do filme lacrimal pré-ocular, camadas da córnea e esclera anterior. Resultados: A espessura do filme lacrimal, o teste de Schirmer e os valores do tempo de ruptura do filme lacrimal foram significativamente menores no grupo com síndrome de Sjögren do que nos controles saudáveis (p<0,05). As medidas de espessura das camadas corneais e da esclera foram similares entre os grupos. A espessura do filme lacrimal foi moderadamente correlacionada com os resultados do teste de Schirmer (r=0,34, p=0,001), mas não houve correlação entre os resultados do teste de Schirmer e tempo de ruptura (r=0,18, p=0,09). Conclusões: O filme lacrimal pré-ocular, medido pela tomografia de coerência óptica de segmento anterior, foi mais fino em pacientes com síndrome de Sjögren primária do que nos controles saudáveis. As espessuras das camadas da córnea e da esclera anterior foram semelhantes entre os grupos.

Association between systemic activity index and dry eye severity in patients with primary Sjögren syndrome / A associação entre o índice de atividade sistêmica e a severidade do olho seco em pacientes com síndrome de Sjögren primária

ABSTRACT Purpose: The aim of the present study was to compare the severity of ocular and systemic findings among patients with primary Sjögren syndrome. Methods: The study followed a prospective controlled design and comprised two groups; the test group included 58 eyes of 58 patients newly diagnosed with primary Sjögren syndrome with poor dry eye test findings and the control group included 45 right eyes of 45 healthy age- and sex-matched individuals. The ocular surface disease index score, tear osmolarity, Schirmer I test without anesthesia, fluorescein tear breakup time, and cornea-conjunctiva staining with lissamine green (van Bijsterveld scoring) were used to examine tear function in the patients via a complete ophthalmological examination. The results were graded and classified on the basis of a Dry Eye WorkShop report and results of the corneal and conjunctival staining test, Schirmer's test, and fluorescein tear breakup time test. Discomfort, severity and frequency of symptoms, visual symptoms, conjunctival injection, eyelid-meibomian gland findings, and corneal-tear signs were interpreted. Disease activity was scored per the EULAR Sjögren's syndrome disease activity index (ESSDAI) via systemic examination and laboratory evaluations, and the EULAR Sjögren's syndrome patient-reported index (ESSPRI) assessed via a survey of patient responses. Results: Mean patient age was 48.15 ± 16.34 years in the primary Sjögren syndrome group and 44.06 ± 9.15 years in the control group. Mean fluorescein tear breakup time was 4.51 ± 2.89s in the primary Sjögren syndrome group and 10.20 ± 2.39 s in the control group. Mean Schirmer I test result was 3.51 ± 3.18 mm/5 min in the primary Sjögren syndrome group and 9.77±2.30 mm/5 min in the control group. Mean ocular surface disease index score was 18.56 ± 16.09 in the primary Sjögren syndrome group, and 19.92 ± 7.16 in the control group. Mean osmolarity was 306.48 ± 19.35 in the primary Sjögren syndrome group, and 292.54 ± 10.67 in the control group. Mean lissamine green staining score was 2.17 ± 2.76 in the primary Sjögren syndrome group, and 0.00 in the control group. Statistically significant differences were found berween the primary Sjögren syndrome group and control group in terms of fluorescein tear breakup time, Schirmer's test, lissamine green staining, and osmolarity tests (p=0.036, p=0.041, p=0.001, and p=0.001 respectively). The Dry Eye WorkShop score was 2.15 ± 0.98, the EULAR Sjögren's syndrome disease activity index score was 11.18 ± 4.05, and the EULAR Sjögren's syndrome patient-reported index score was 5.20±2.63. When potential associations of the Dry Eye Workshop Study scores and osmolarity scores with the Eular Sjögren's syndrome disease activity index scores were evaluated, the results were found to be statistically significant (p=0.001, p=0.001 respectively). Conclusion: The results showed an association between dry eye severity and systemic activity index in primary Sjögren syndrome patients.

RESUMO Objetivo: O objetivo do presente estudo foi comparar a gravidade dos achados oculares e sistêmicos entre pacientes com síndrome de Sjögren primária. Métodos: O estudo seguiu um delineamento prospectivo controlado e compreendeu dois grupos; o grupo de teste incluiu 58 olhos de 58 pacientes recém-diagnosticados com síndrome de Sjögren primária com resultados deficientes no teste de olho seco e o grupo controle incluiu 45 olhos direitos de 45 indivíduos saudáveis pareados idade e sexo. A contagem do índice de doença da superfície ocular, osmolaridade lacrimal, teste de Schirmer I sem anestesia, tempo de ruptura da fluoresceína e coloração córnea-conjuntiva com verde de lissamina (índice de van Bijsterveld) foram utilizados para examinar a função lacrimal dos pacientes através de exame oftalmológico completo. Os resultados foram classificados com base em um relatório da "Dry Eye Workshop" e resultados do teste de coloração da córnea e conjuntiva, teste de Schirmer e teste do tempo de ruptura da fluoresceína. Desconforto, gravidade e frequência dos sintomas, sintomas visuais, injeção conjuntival, achados das glândulas palpebrais e sinais da córnea foram interpretados. A atividade da doença foi avaliada pelo índice de atividade da doença da síndrome de Sjögren EULAR por meio de exame sistêmico e avaliações laboratoriais, e o índice relatado pelo paciente da síndrome de Sjörgen EULAR avaliado através de uma pesquisa das respostas dos pacientes. Resultados: A média de idade dos pacientes foi de 48,15 ± 16,34 anos no grupo da Síndrome de Sjörgen primária e 44,06 ± 9,15 anos no grupo controle. O tempo médio de ruptura da fluoresceína foi de 4,51 ± 2,89 s no grupo síndrome de Sjögren primária e 10,20 ± 2,39 s no grupo controle. O resultado do teste de Schirmer I médio foi de 3,51 ± 3,18 mm/5 min no grupo síndrome de Sjögren primária e de 9,77 ± 2,30 mm/5 min no grupo controle. O índice médio de doença da superfície ocular foi de 18,56 ± 16,09 no grupo síndrome de Sjögren primária e 19,92 ± 7,16 no grupo controle. A osmolaridade média foi 306,48 ± 19,35 no grupo síndrome de Sjögren primária e 292,54 ± 10,67 no grupo controle. O resultado médio de coloração com lissamina verde foi de 2,17 ± 2,76 no grupo síndrome de Sjögren primária e 0,00 no grupo controle. Diferenças es­tatisticamente significativas foram encontradas entre o com sín­­drome de Sjögren primária e o grupo controle em termos de tempo de ruptura da fluoresceína lacrimal, teste de Schirmer I, coloração com lissamina verde e osmolaridade (p=0,036, p=0,041, p=0,001, p=0,001 respectivamente). O índice Estudo do Olho Seco foi de 2,15 ± 0,98, o índice de atividade da doença da síndrome de Sjögren EULAR foi de 11,18 ± 4,05 e a pontuação do índice relatado pelo paciente EULAR Sjögren foi de 5,20 ± 2,63. Quando associações potenciais do Estudo do Olho Seco e o índice da osmolaridade foram comparados a pontuação de índice de atividade da doença da síndrome de Sjögren EULAR, os resultados foram estatisticamente significantes (p=0,001, p=0,001 respectivamente). Conclusão: Os resultados mostraram uma associação entre a gravidade do olho seco e o índice de atividade sistêmica em pacientes com síndrome de Sjögren primária.

Características salivales y estado sistémico de sujetos con xerostomía / Salivary characteristics and systemic status of subjects with xerostomia

RESUMEN: Xerostomía o sensación de boca es una afección que afecta severamente la calidad de vida de quienes la padecen. Si bien se relaciona con la reducción del flujo salival (hiposalivación), existe evidencia contradictoria y se sugiere evaluar características cualitativas salivales y estado sistémico de los afectados para comprender su etiología y mejorar terapias asociadas. El objetivo de este estudio fue comparar pH y concentración de proteínas en saliva y estado sistémico entre sujetos xerostómicos con y sin hiposialia. Se midió pH, concentración de proteínas salivales y se consignaron antecedentes sistémicos y uso de fármacos en 27 individuos xerostómicos, que fueron divididos en hiposiálicos y no hiposiálicos. Se compararon las variables mencionadas usando test no paramétrico de Mann-Whitney y test Chi-Cuadrado. Se aceptaron diferencias estadísticamente significativas con error alfa igual o menor a 5 %. Sujetos xerostómicos con hiposialia presentaron mayor prevalencia de Artritis Reumatoide y Síndrome de Sjögren en comparación con xerostómicos sin hiposialia. No se encontraron diferencias estadísticamente significativas en relación a pH, concentración de proteínas y uso de medicamentos, variables que no influirían en el padecimiento de xerostomía, independiente de la existencia de hiposialia. Sujetos con xerostomía e hiposialia presentan mayor frecuencia de Artritis Reumatoide y Síndrome de Sjögren.

ABSTRACT: Xerostomia or mouth feeling is a condition that severely affects the quality of life of thosewho suffer from it. Although it is related to the reduction of salivary flow (hyposalivation),there is contradictory evidence and it is suggested to evaluate qualitative salivarycharacteristics and systemic state of those affected in order to understand its etiology and improve associated terapies. The objective of this study was to compare saliva, pH and protein concentration and systemic status among xerostomic subjects with and without hyposialia. Were measured PH and protein concentration and were recorded systemic antecedents and drug use in 27 xerostomic individuals, who were divided into hyposalic and nonhyposalic individuals. The mentioned variables were compared using non-parametric Mann-Whitney test and Chi-Square test. Statistically significant differences were accepted with alpha error equal to or less than 0.05%. Xerostomic subjects with hyposialia presented higher prevalence of Rheumatoid Arthritis and Sjögren's Syndrome compared to xerostomics without hyposialia. No statistically significant differences were found in relation to pH, protein concentration and drug use, variables that would not influence xerostomia, independent of hyposialia. Subjects with xerostomia with hyposialia present a higher frequency of Rheumatoid Arthritis and Sjögren's Syndrome.

Effects of periodontal treatment on primary sjogren's syndrome symptoms

Abstract The aim of this longitudinal prospective study was to evaluate the effects of periodontal treatment on the clinical, microbiological and immunological periodontal parameters, and on the systemic activity (ESSDAI) and subjective (ESSPRI) indexes in patients with primary Sjögren’s Syndrome (pSS). Twenty-eight female patients were divided into four groups: pSS patients with or without chronic periodontitis (SCP, SC, respectively), and systemically healthy patients with or without chronic periodontitis (CP, C, respectively). Periodontal clinical examination and immunological and microbiological sample collection were performed at baseline, 30 and 90 days after nonsurgical periodontal treatment (NSPT). Levels of interleukin IL-1β, IL-8 and IL-10 in saliva and gingival crevicular fluid (GCF) were evaluated by ELISA, as well as the expression of Porphyromonas gingivalis (Pg), Aggregatibacter actinomycetemcomitans, (Aa) Tannerella forsythia (Tf), and Treponema denticola (Td), by qPCR. Systemic activity and pSS symptoms were evaluated by ESSDAI and ESSPRI. NSPT resulted in improved periodontal clinical parameters in both SCP and CP groups (p>0.05). Pg, Aa, and Tf levels decreased after NSPT only in CP patients (p<0.05). Significantly greater levels of IL-10 in GCF were verified in both SCP and CP groups (p<0.05). SCP patients showed increased salivary flow rates and decreased ESSPRI scores after NSPT. In conclusion, NSPT in pSS patients resulted in improved clinical and immunological parameters, with no significant effects on microbiological status. pSS patients also showed increased salivary flow and lower ESSPRI scores after therapy. Therefore, it can be suggested that NSPT may improve the quality of life of pSS patients.

Alteraciones motoras esofágicas en pacientes con síndrome de Sjögren: estudio de casos y controles / Esophageal motility disorders in patients with primary Sjogren's syndrome: study of cases and controls

El síndrome de Sjögren primario (SSp), afecta diversos sistemas, como el digestivo; destacando la dificultad deglutoria del bolo alimentario. El objetivo de este estudio es describir la función y motilidad esofágica de pacientes con SSp y compararla con sujetos sanos. Estudio de casos y controles, realizado en Clínica Mayor de Temuco (2004-2009). Se estudiaron pacientes con SSp, con disfagia permanente, sin límite de edad ni sexo (casos); y sujetos asintomáticos digestivos (controles); emparejados por edad y sexo. Se estudiaron con manometría esofágica estacionaria. Se determinó presión de reposo (PR) y longitud (L) del esfínter esofágico inferior (EEI) y superior (EES); amplitud (A) y duración (D) de las ondas peristálticas desencadenadas con degluciones secas y húmedas en los tres tercios del cuerpo esofágico (S, M e I), y aparición de ondas terciarias (OT). Se estimó una muestra 20 casos y 40 controles. Se aplicó estadística descriptiva, Chi2 de Pearson o exacto de Fischer para variables categóricas; y Prueba t para variables continuas. La PREEI fue menor en los casos (11,8±0,7 mmHg vs. 14,8±0,5 mmHg respectivamente). Se verificaron diferencias estadísticamente significativas en la AS, DM, DI y OT tras las degluciones secas; y, en AS, AM, AI, DM, DI y OT, tras las degluciones húmedas. No hubo variaciones en la PREES ni LEES entre casos y controles. Se verificaron alteraciones motoras esofágicas y baja PREEI en pacientes con SSp, respecto de un grupo de controles.

Primary Sjögren's syndrome (PSS), affects various systems such as the digestive; highlighting the difficulty swallowing food bolus. The objective of this study is to describe the function and in patients with esophageal motility PSS compare them to healthy subjects. Case studies and tests conducted at the clinica Mayor Temuco (2004-2009). PSS patients with permanent dysphagia, regardless of age or sex (cases) were studied and digestive asymptomatic subjects (controls); matched for age and sex. We studied these with stationary esophageal manometry. Resting pressure (RP) and length (L) of the lower esophageal sphincter (LES) and upper (UES) was determined; amplitude (A) and duration (D) of the peristaltic waves triggered with dry and wet swallows in the three thirds of the esophageal body (S, M and I), and appearance of tertiary waves (TW). A sample of 20 cases and 40 controls were estimated. Descriptive statistics, Pearson Chi2 or Fisher's exact was applied for categorical variables; and t-test for continuous variables. The PREEI was lower in cases (11.8±0.7 mmHg vs. 14.8±0.5 mmHg respectively). Statistically significant differences in the AS, DM, DI and TW were verified after dry swallows; and in AS, AM, AI, DM, DI and TW after wet swallows. There were no changes in Prees or LEES between cases and controls. Lower esophageal motor disorders was checked in patients with PSS RPEEI, for a control group.

An update of neurological manifestations of vasculitides and connective tissue diseases: a literature review / Atualização das manifestações neurológicas das vasculitides e das doenças do tecido conjuntivo: revisão de literatura

Vasculitides comprise a heterogeneous group of autoimmune disorders, occurring as primary or secondary to a broad variety of systemic infectious, malignant or connective tissue diseases. The latter occur more often but their pathogenic mechanisms have not been fully established. Frequent and varied central and peripheral nervous system complications occur in vasculitides and connective tissue diseases. In many cases, the neurological disorders have an atypical clinical course or even an early onset, and the healthcare professionals should be aware of them. The purpose of this brief review was to give an update of the main neurological disorders of common vasculitis and connective tissue diseases, aiming at accurate diagnosis and management, with an emphasis on pathophysiologic mechanisms.

As vasculites são um grupo heterogêneo de doenças autoimunes primárias ou secundárias a uma grande variedade de doenças infecciosas sistêmicas, malignas ou do tecido conjuntivo. Estas últimas são as que ocorrem com mais frequência, porém os mecanismos patogênicos ainda não foram plenamente determinados. Diversas e frequentes complicações do sistema nervoso central e periférico ocorrem nas vasculites e doenças do tecido conjuntivo. Em muitos casos, os distúrbios neurológicos têm evolução clínica atípica ou mesmo início precoce, ao que todos os profissionais de saúde devem estar cientes. O objetivo desta breve revisão foi atualizar os principais distúrbios neurológicos da vasculite comum e das doenças do tecido conjuntivo, visando ao diagnóstico e ao tratamento corretos, com ênfase nos mecanismos fisiopatológicos.

Evaluation of possible factors affecting contrast sensitivity function in patients with primary Sjögren’s syndrome / A avaliação dos possíveis fatores que afetam a função de sensibilidade ao contraste em pacientes com síndrome de Sjögren primária

ABSTRACT Purpose: The contrast sensitivity (CS) function in patients with primary Sjögren’s syndrome (pSS) may be impaired either frequently as a result of dry eye diseases or rarely as a result of optic neuropathy. In this study, we aimed to evaluate the CS function in pSS patients as well as to assess corneal aberrations and thickness of the peripapillary retinal nerve fiber layer (pRNFL). Methods: Fourteen eyes of 14 pSS patients (pSS group) and 14 eyes of 14 healthy participants (control group) were subjected to assessment of CS at the spatial frequencies of 1.5, 3.0, 6.0, 12, and 18 cycles/degree (cpd) using a functional visual acuity contrast test (FACT); measurement of corneal high-order aberrations (HOAs) in terms of coma-like, spherical-like, and total HOAs using Scheimpflug corneal topography; and measurement of the thickness of both the macular ganglion cell-inner plexiform layer (mGCIPL) and pRNFL in all quadrants using optical coherence tomography. None of the participants were under treatment with artificial tears. Results: The results of the CS test did not differ between the 2 groups at all spatial frequencies (p>0.05). In addition, there were no statistically significant differences between the 2 groups in terms of corneal HOAs (p>0.05) and thickness of mGCIPL (p>0.05). However, among all quadrants, only the inferior quadrant of pRNFL in pSS patients was statistically significantly thinner than that in the healthy participants (p=0.04). Conclusions: The CS function in pSS patients can be maintained with normal thickness of both pRNFL and mGCIPL and with lack of increased corneal HOAs, which may be present even in the absence of artificial tear usage. .

RESUMO Objetivo: A função de sensibilidade ao contraste em pacientes com síndrome de Sjögren primário (pSS) pode ser prejudicada, quer frequentemente como resultado de doenças do olho seco, ou mais raramente como um resultado de neuropatia óptica. Neste estudo, objetivamos avaliar a função de sensibilidade ao contraste de pacientes com pSS, além da avaliação das aberrações da córnea e a espessura da camada de fibras nervosas da retina (pRNFL). Métodos: Catorze olhos de 14 pacientes com pSS e 14 olhos de 14 participantes saudáveis foram submetidos, respectivamente, à avaliação do teste de sensibilidade aos contrastes (CS) nas frequências espaciais de 1,5, 3,0, 6,0, 12 e 18 ciclos/grau (cpd), utilizando teste de contraste acuidade visual funcional (FACT); a medida das aberrações de alta ordem da córnea (HOAs) em termos de coma, aberrações esféricas e aberrações totais, utilizando topografia corneana por Scheimpflug; e medida de espessura da camada de macular de células ganglionares plexiforme interna (mGCIPL) e a espessura de pRNFL em todos os quadrantes usando tomografia de coerência óptica. Nenhum dos participantes estava sob tratamento com lágrimas artificiais. Resultados: O teste CS em pacientes pSS não diferiu do que o teste CS em participantes saudáveis em todas as frequências espaciais (p>0,05). Não houve também nenhuma diferença estatisticamente significativa entre os dois grupos em termos de HOAs da córnea (p>0,05), e espessura de mGCIPL (p>0,05). No entanto, entre todos os quadrantes, apenas o quadrante inferior da pRNFL em pacientes pSS foi significativamente mais fino que o quadrante inferior da pRNFL em participantes saudáveis (p=0,04). Conclusões: A função de CS em doentes com pSS pode ser mantida em condições de ambas as espessuras normais de pRNFL e mGCIPL, assim como nas condições de falta de aumento HOAs da córnea, que pode ser mantida, mesmo na ausência do uso de lágrimas artificiais. .

A short neuropsychological evaluation of patients with primary Sjogren's syndrome / Avaliacao neuropsicologica breve em pacientes com sindrome de Sjogren primaria

Objective: To investigate cognitive deficits in patients with primary Sjögren’s syndrome (PSS). Method: Eighteen patients with PSS, aged between 25 and 61 years, were subjected to a short neuropsychological battery and compared with 18 patients with multiple sclerosis and 18 healthy controls. Results: The analysis of variance (ANOVA) revealed that the clinical groups had significantly worse performance than the control group on the Rey Auditory Verbal Learning Test 3; (F 2,53 =3.500, p=0.038) and 7 (F 2,53 =5.068, p=0.010). The clinical groups had elevated levels of depression on the Beck Depression Inventory (BDI); (p=0.003). The analysis of the data from the Trail Making Test B-A revealed a significant difference between the clinical and control groups (p=0.023). The analysis of covariance with BDI score as a covariate, did not change the outcome. Conclusion: Our study revealed cognitive deficits in patients with PSS detectable by a short neuropsychological battery. .

Objetivo: Investigar déficits cognitivos em pacientes com síndrome de Sjögren primária (SSP) utilizando bateria neuropsicológica breve. Método: Dezoito pacientes com SSP e idade entre 25 e 61 anos, foram submetidos a uma bateria neuropsicológica breve e os resultados comparados com 18 pacientes com esclerose múltipla e com 18 controles saudáveis. Resultados: A análise da variância revelou que os grupos clínicos apresentaram desempenho significativamente pior que os controles ao Rey Auditory Verbal Learning Test 3 (RAVLT 3; F 2,53 =3,500, p=0,038) e RAVLT 7 (F 2,53 =5,068, p=0,010). Os grupos clínicos apresentaram índices elevados de depressão pela Beck Depression Inventor y (BDI); (F 2,53 =0.003). O desempenho no Trail Making Test B-A revelou diferença significativa entre os grupos clínicos e o grupo controle (p=0,023). A análise de covariância tomando a BDI como covariante, não revelou mudanças nos resultados. Conclusão: Nosso estudo revelou comprometimento cognitivo em pacientes com SSP detectável por bateria neuropsicológica breve. .

Endothelial dysfunction in primary Sjögren syndrome / Disfunción endotelial en el síndrome de Sjögren primario

OBJECTIVE: In this study, we aimed to investigate endothelial functions in primary Sjögren syndrome. METHODS: Thirty-five patients with primary Sjögren syndrome and 20 age and sexmatched healthy volunteers were recruited to the present study. Flow mediated dilatation of brachial artery and carotid intimamedia thickness were measured in the study population. RESULTS: Carotid intimamedia thickness values were similar between groups (0.50 ± 0.10, 0.53 ± 0.08, p > 0.05). Flow mediated dilatation of the brachial artery was disrupted in the primary Sjögren syndrome group (7% vs 12%, p = 0.002). CONCLUSION: There is endothelial dysfunction in patients with primary Sjögren syndrome, although they had comparable carotid intimamedia thickness with the healthy control group.

OBJETIVO: Este estudio se encaminó a investigar las funciones endoteliales en el síndrome de Sjögren primario. MÉTODOS: Para el presente estudio, se reclutaron treinta y cinco pacientes con síndrome de Sjögren primario y 20 voluntarios sanos apareados por edad y sexo. La dilatación mediada por flujo observada en la arteria braquial, y el espesor íntimamedia carotídeo fueron medidos en la población bajo estudio. RESULTADOS: Los valores del espesor íntimamedia carotídeo fueron similares entre los grupos (0.50 ± 0.10, 0.53 ± 0.08, p > 0.05). La dilatación mediada por flujo de la arteria braquial, estaba alterada en el grupo de síndrome del Sjögren primario (7% frente a 12%, p = 0.002). CONCLUSIÓN: Hay una disfunción endotelial en los pacientes con el síndrome de Sjögren primario, aunque estos tenían un espesor íntimamedia carotídeo comparable con el grupo de control saludable.

Curso de reumatologia: aula 7: manifestações neurológicas nas colagenoses / Course of rheumatology: class 7: neurologic manifestations in collagenosis

O propósito deste artigo é focar as principais manifestações neurológicas das doenças difusas do tecido conjuntivo, com ênfase nas suas manifestações clínicas. Os autores discutem as várias complicações do sistema nervoso central e periférico na doença muscular inflamatória (polimiosite e dermatomiosite), policondrite recidivante, esclerose sistêmica, artrite reumatoide, síndrome de Sjõgren, doença mista do tecido conjuntivo (doença de Sharp), lúpus eritematoso sistêmico e síndrome do anticorpo antifosfolipide.

The purpose of this article is to focus on the major nervous system manifestations in connective tissue diseases, with emphasis on their clinica findings. Authors discuss several complications in inflammatory muscle disease (polymyositis an dermatomyositis), relapsing polychondritis, systemic sclarosis, rheumatoid arthritis, Sjõgren syndorme, mixed connective tissue (Sharp disease), systemic lupus erythematosus and antiphospholipid syndrome.

Curso de Reumatologia: Aula 3: Síndrome de Sjõgren: Aspectos clínicos e condutas terapêuticas / Course of Reumatologia: Class 3: Syndrome of Sjõgren: Clinical aspects and therapeutic conducts

A síndrome de Sjõgren caracteriza-se por apresentar boca seca (xerostomia) e olhos secos (ceratoconjuntivite secal), associados a outras doenças difusas do tecido conjuntivo (forma secundária) ou manifestações isoladas (forma primária). Essa síndrome tem preferência pelo sexo feminino e apresenta distribuição mundial. Apesar de descrita em 1933, ainda hoje é subdiagnosticada, trazendo aos pacientes sofrimentos e repercussões socioeconômicas. O objetivo deste artigo é descrever as principais manifestações clínicas, os critérios utilizados para facilitar o seu diagnóstico e as modalidades terapêuticas disponíveis.

Sjõrgren's syndrome is a systemic inflammatory autoimmune disease with worldwide distribution. It affects primaly females during the forth and fifth decades of life. The disease's clinical features are: mucosal dryness manifested in keratoconjunctivitis sicca, xerostomia, xerotrachea and vaginal dryness. There are two forms of manifestation. The primary has isolated symptoms and the secondary associated with some connective tissue disease like rheumatoid arthritis, systemic lupus erythamatosus, systemic sclerosis and polymyositis. The aim of this article is to describe its clinical disclosures, the currently used diagnostic criteria and the available treatment for the syndrome.

Curso de Reumatologia: aula 4: manifestações gastrointestinais nas colagenoses / Course of Rheumatology: class 4: gastrointestinal manifestations in collagenosis

O termo colagenose, surgido em 1942, é hoje substituído por doenças difusas do tecido conjuntivo, que se constituem em um grupo de moléstias que afetam o tecido conjuntivo, rico em colágeno. São enfermidades que podem comprometer vários sistemas e múltiplos órgãos, sendo, portanto, de caráter sistêmico. O objetivo desta aula é discutir as manifestações gastrointestinais e sua abordagem terapêutica. Serão focadas algumas doenças difusas do tecido conjuntivo, a saber: esclerose sistêmica, lúpis eritematoso sistêmico, dermatopolimiosite e Sjõgren.

The term "collagen diseases" was first introduced by Klemperer in 1942, and is currently been called connective tissue diseases. All the organ and systems are both affected by them, so that's why it can be called systemic diseases. The aim of this lesson is to discuss gastrointestinal manifestations and its therapeutic approach, while it will be detailed the connective tissue diseases like systemic sclerosis, systemic lupus erythematosus, polymyositis/dermatomyositis and Sjõgren's syndrome.

Minor salivary glands morphology in xerostomia patients / Morfología de las Glándulas Salivales Menores en Pacientes Xerostomizados

Las glándulas salivales menores son encontradas distribuidas a través de la mucosa oral, especialmente en los labios y en la mucosa del paladar blando. Varios factores pueden causar xerostomía, donde las características histológicas de las glándulas salivales son también consideradas como factores para definir la etiología. Así, las biopsias de las glándulas salivales menores representan una herramienta fundamental para alcanzar los criterios diagnósticos requeridos en la clasificación de pacientes con síndrome de Sjõgren, ya que no representa riesgo para los pacientes. El objetivo de este estudio es determinar las características histológicas de las glándulas mencionadas, obtenidas de biopsias de pacientes con xerostomía y clasificar los aspectos histológicos de las glándulas en el síndrome de Sjõgren. Estudiamos 40 láminas de pacientes con xerostomía, cuyas glándulas salivales menores fueron sometidas a biopsia en el Servicio de Estomatología de la Santa Casa de São Paulo, Brasil. Se observaron las variaciones de su aspecto histológico, desde la normalidad hasta la presencia de focos inflamatorios, los cambios del tejido conjuntivo entre los acinos y conductos, como también el parénquima. En 15 casos, el infltrado de células inflamatorias invadió el foco, es decir, grupos de al menos 50 células inflamatorias alrededor de acinos o conductos, lo cual es un aspecto característico del síndrome de Sjõgren. Por lo tanto, el hallazgo de al menos un foco inflamatorio de 4 mm2 de tejido glandular, representa un buen criterio, aunque no es uno de los criterios a considerar cuando se trata de clasificar a los pacientes con el Síndrome de Sjõgren.

The minor salivary glands are found scattered throughout the oral mucosa, especially in the lips and soft palate mucosa. Several factors can cause xerostomia, whereas the salivary glands histological characteristics are also considered as factors for defining the etiology. Thus, the minor salivary glands biopsy represents an essential tool for attending the required diagnosis criterion in the classification of Sjögren's Syndrome patients, since it does not present risk for the patient. The objective of this study is to determine the histological description of the minor salivary glands obtained from the biopsies of xerostomia patients and to classify the minor salivary gland histological aspect as the Sjögren's Syndrome. Forty laminas of xerostomia patients that were submitted to minor salivary glands biopsy at the Santa Casa de Sao Paulo Stomatology ambulatory were retrospectively studied. The variation in the glands histological aspect was observed, from the normality up to the presence of inflammatory focus, replacing the conjunctive between acini and ducts, as well as the parenchyma. In 15 cases, the infiltrated inflammatory cells amounted to focus, that is to say, groups of at least 50 inflammatory cells around the acini or ducts, which is a characteristic aspect of the Sjögren's Syndrome. Therefore, the finding of at least one inflammatory focus of 4 mm2 of glandular tissue represents a set criterion, although, not the only one in order to classify this patient as having the Sjögren's Syndrome.

Neuropsychological, neuroimage and psychiatric aspects of primary Sjögren's syndrome

We report a case of a 49-year-old woman diagnosed with primary Sjögrens Syndrome (pSS) who was submitted to extensive neuropsychobiological assessment. Examination revealed a Wechsler Adult Intelligence Scale-Revised (WAIS-R) Full Scale IQ of 97 with no Verbal/Performance IQ discrepancy and performance below estimated premorbid levels on arithmetic skills, visual tracking, naming and delayed paired associate learning/memory. CT scans of the brain were normal. However, there were subcortical hyperintensities on MRI and left parieto-temporal hypoperfusion on SPECT. Neuropsychological impairment is consistent with the pattern of neuroimage findings. We hypothesize that the pathophysiological mechanisms of pSS involve direct immune attack on neurons in addition to indirect effects through small-vessel angiopathy and thereby induce natural fracture lines in behavior according to location in the central nervous system

Síndrome de Sjögren: estudo clínico geral / Sjögren's syndrome: general clinycal study

A síndrome de Sjögren (SS) é uma doença inflamatória crônica auto-imune, conhecida principalmente por ocasionar diminuiçäo das secreções das glândulas salivares e lacrimais. Com etiologia multifatorial, pode ocorrer isoladamente, caracterizando a forma primária, ou ser secundária a outras doenças relacionadas à auto-imunidade, como a artrite reumatóide. Acomete com maior freqüência mulheres de meia-idade, observando-se uma prevalência de 1 por cento na populaçäo geral. Os autores abordam os principais aspectos clínicos da síndrome, dando ênfase à clinica, ao prognóstico e às perspectivas terapêuticas através da terapia gênica

Síndrome de Sjögren da forma primária / Sjögren's syndrome in the primary form

Os autores descrevem o caso de uma paciente com ceratoconjuntivite seca, apresentando características da síndrome de Sjörgren. Comentam o manejo de pacientes com esta síndrome e a importância de sua avaliação e acompanhamento oftalmológico

Síndrome Sjögren asociado a colagenopatías: presentación de un caso / Sjögren's syndrome associated to collagenophaties

Se describe un Síndrome Sicca en una mujer de 53 años de edad, internada en el Hospital Zenón Santillán, asociado a enfermedades del colágeno tales como: Artritis Reumatoidea, Vasculitis Leucocitoclástica y probable Tiroiditis. Los resultados de sus análisis bioquímicos, Rx de glándulas salivales, gammagrafía y la biopsia del labio inferior, confirman el diagnóstico. Se instituyó tratamiento con Bromhexina y gotas de Pilocarpina (medicación no habitual), obteniendo resultados satisfactorios.