Introdução: A Síndrome de Sjögren (SS) é uma doença crônica, sistêmica, autoimune que afeta diversas glândulas, principalmente lacrimais e salivares, causando sensação de olhos e boca secos. Objetivo: Investigar as características miofuncionais orofaciais e a presença de sinais e sintomas de Desordem Temporomandibular (DTM) em portadores de Síndrome de Sjögren, comparativamente a um grupo controle. Metodologia: Estudo transversal, prospectivo descritivo e comparativo. Participaram 19 mulheres (média de idade: 33,2±8,7 anos), seis portadoras da forma primária da SS e treze portadoras da forma secundária da SS.Vinte mulheres saudáveis, pareadas por idade (média de idade 31,9±9,3 anos) compuseram o grupo controle (grupo C). As avaliações e os exames realizados foram: Protocolo de Avaliação Miofuncional Orofacial com Escores (AMIOFE), medida dos limites de movimentos mandibulares, pressão de língua e de lábios (Iowa Oral PerformanceInstrument- IOPI), palpação dos músculos e das articulações temporomandibulares (ATMs), Instrumento de Autoavaliação da Alimentação (EAT-10), questionário para investigação dos sinais e sintomas de desordem temporomandibular (ProDTMmulti), escala de dificuldade para mastigar, escala de funcionalidade mandibular (JFLS) e eletromiografia de superfície (EMG) dos músculos da mastigação e deglutição. Os dados categóricos ou com distribuição não normal foram analisados pelo teste estatístico não paramétrico de Mann-Whitney e aqueles com distribuição normal pela Análise de Variância (ANOVA), seguida do pós-teste. O nível de significância adotado foi P< 0,05. Resultados: O grupo SS apresentou diferenças estatisticamente significantes na comparação com o grupo C, com piores condições na avaliação miofuncional orofacial, quanto ao aspecto/postura das estruturas orais, a mobilidade de lábios, língua, bochechas e mandíbula, assim como nas funções de mastigação, deglutição e respiração. Foram também estatisticamente menores a abertura bucal, a distância interincisal horizontal, a pressão da língua na protrusão e elevação (valor da média e máximo), bem como no valor da deglutição. Os portadores de SS mostraram, por meio de escores,maior dificuldade para mastigar e risco de disfagia (EAT-10). Também foram maiores no grupo SS, comparado ao controle, os escores de dor à palpação, a severidade dos sinais e sintomas de DTM (ProDTMmulti) e a limitação funcional da mandíbula (JFLS). A atividade dos músculos mastigatórios nas provas de máxima contração voluntária (MCV) foi menor no grupo SS com um maior impacto por ciclo na mastigação unilateral esquerda. As mudanças temporais na deglutição, com duração aumentada para a consistência líquida e reduzida na deglutição de sólido também foram significantes no grupo SS, comparado ao grupo C. Conclusão: os resultados mostraram que os portadores de Síndrome de Sjögren apresentam prejuízos acentuados na musculatura e funções orofaciais e sinais e sintomas de DTM moderados, uma percepção de grande limitação funcional. Portanto, as consequências da SS não se resumem às queixas subjetivas, mas sim envolvem comprometimentos reais do sistema e funções estomatognáticas. / Introduction: Sjögren\'s syndrome (SS) is a chronic, systemic, autoimmune disease that affects several glands, mainly lacrimal and salivary, causing dry eyes and mouth. Objective: To investigate myofunctional orofacial characteristics and the presence of signs and symptoms of Temporomandibular Disorderers (TMD) in Sjögren\'s Syndrome patients compared to a control group. Methodology: Prospective descriptive and comparative cross-sectional study. Participants were 19 women (mean age: 33,2±8,7), with primary (n = 6) or secondary (n = 13) Sjögren Syndrome (SS group) and 20 healthy women, matched by age (mean age: 31,9 ±9,3-C group). The evaluations and examinations were performed with the Protocol of Orofacial Myofunctional Evaluation with Scores (OMES), measurement of limits of mandibular movements, tongue and lip pressure measure with Iowa Oral Performance Instrument (IOPI), Eating Assessment Tool (EAT-10), Temporomandibular Muscles and Temporomandibular Joint (TMJ) palpation, questionnaire for investigation of signs and symptoms of temporomandibular disorder (ProDTMmulti), difficulty of Chewing Scale, Jaw Functional Limitation Scale (JFLS), and Surface Electromyography (sEMG) of chewing and swallowing muscles. The categorical or non-normal distribution data were analyzed by nonparametric Mann-Whitney statistical test and data with normal distribution analyzed by Variance Analysis (ANOVA), followed by post-test. The level of significance was P<0.05. Results: The SS group presented statistically significant differences in comparison with group C, with worse conditions in the orofacial myofunctional evaluation, regarding the appearance / posture of oral structures, mobility of lips, tongue, cheeks and mandible, as well as in the functions of chewing, swallowing and breathing. The oral opening, horizontal interincisal distance, tongue pressure on protrusion and elevation (mean and maximum value), as well as the value of swallowing were also statistically lower. SS patients showed by scores greater difficulty in chewing and risk of dysphagia (EAT-10). There were also higher in the SS group, compared to control, pain scores at palpation, the severity of TMD signs and symptoms (ProDTMmulti) and functional limitation of the mandible (JFLS). The activity of the masticatory muscles in the tests of maximum voluntary contraction (MCV) was also lower in the SS group with a greater impact per cycle during the masticatory EMG. The temporal changes in swallowing, with increased duration for the liquid consistency and reduced for solid swallowing were also significant in the SS group, compared to the control. Conclusion: the results showed that patients with Sjögren\'s Syndrome present marked impairments in musculature and orofacial functions and moderate TMD signs and symptoms, a perception of great functional limitation. Therefore, the consequences of SS are not limited to subjective complaints, but rather involve real system compromises and stomatognatic functions.

Introdução: A Síndrome de Sjögren (SS) é uma doença crônica, sistêmica, autoimune que afeta diversas glândulas, principalmente lacrimais e salivares, causando sensação de olhos e boca secos. Objetivo: Investigar as características miofuncionais orofaciais e a presença de sinais e sintomas de Desordem Temporomandibular (DTM) em portadores de Síndrome de Sjögren, comparativamente a um grupo controle. Metodologia: Estudo transversal, prospectivo descritivo e comparativo. Participaram 19 mulheres (média de idade: 33,2±8,7 anos), seis portadoras da forma primária da SS e treze portadoras da forma secundária da SS.Vinte mulheres saudáveis, pareadas por idade (média de idade 31,9±9,3 anos) compuseram o grupo controle (grupo C). As avaliações e os exames realizados foram: Protocolo de Avaliação Miofuncional Orofacial com Escores (AMIOFE), medida dos limites de movimentos mandibulares, pressão de língua e de lábios (Iowa Oral PerformanceInstrument- IOPI), palpação dos músculos e das articulações temporomandibulares (ATMs), Instrumento de Autoavaliação da Alimentação (EAT-10), questionário para investigação dos sinais e sintomas de desordem temporomandibular (ProDTMmulti), escala de dificuldade para mastigar, escala de funcionalidade mandibular (JFLS) e eletromiografia de superfície (EMG) dos músculos da mastigação e deglutição. Os dados categóricos ou com distribuição não normal foram analisados pelo teste estatístico não paramétrico de Mann-Whitney e aqueles com distribuição normal pela Análise de Variância (ANOVA), seguida do pós-teste. O nível de significância adotado foi P< 0,05. Resultados: O grupo SS apresentou diferenças estatisticamente significantes na comparação com o grupo C, com piores condições na avaliação miofuncional orofacial, quanto ao aspecto/postura das estruturas orais, a mobilidade de lábios, língua, bochechas e mandíbula, assim como nas funções de mastigação, deglutição e respiração. Foram também estatisticamente menores a abertura bucal, a distância interincisal horizontal, a pressão da língua na protrusão e elevação (valor da média e máximo), bem como no valor da deglutição. Os portadores de SS mostraram, por meio de escores,maior dificuldade para mastigar e risco de disfagia (EAT-10). Também foram maiores no grupo SS, comparado ao controle, os escores de dor à palpação, a severidade dos sinais e sintomas de DTM (ProDTMmulti) e a limitação funcional da mandíbula (JFLS). A atividade dos músculos mastigatórios nas provas de máxima contração voluntária (MCV) foi menor no grupo SS com um maior impacto por ciclo na mastigação unilateral esquerda. As mudanças temporais na deglutição, com duração aumentada para a consistência líquida e reduzida na deglutição de sólido também foram significantes no grupo SS, comparado ao grupo C. Conclusão: os resultados mostraram que os portadores de Síndrome de Sjögren apresentam prejuízos acentuados na musculatura e funções orofaciais e sinais e sintomas de DTM moderados, uma percepção de grande limitação funcional. Portanto, as consequências da SS não se resumem às queixas subjetivas, mas sim envolvem comprometimentos reais do sistema e funções estomatognáticas. / Introduction: Sjögren\'s syndrome (SS) is a chronic, systemic, autoimmune disease that affects several glands, mainly lacrimal and salivary, causing dry eyes and mouth. Objective: To investigate myofunctional orofacial characteristics and the presence of signs and symptoms of Temporomandibular Disorderers (TMD) in Sjögren\'s Syndrome patients compared to a control group. Methodology: Prospective descriptive and comparative cross-sectional study. Participants were 19 women (mean age: 33,2±8,7), with primary (n = 6) or secondary (n = 13) Sjögren Syndrome (SS group) and 20 healthy women, matched by age (mean age: 31,9 ±9,3-C group). The evaluations and examinations were performed with the Protocol of Orofacial Myofunctional Evaluation with Scores (OMES), measurement of limits of mandibular movements, tongue and lip pressure measure with Iowa Oral Performance Instrument (IOPI), Eating Assessment Tool (EAT-10), Temporomandibular Muscles and Temporomandibular Joint (TMJ) palpation, questionnaire for investigation of signs and symptoms of temporomandibular disorder (ProDTMmulti), difficulty of Chewing Scale, Jaw Functional Limitation Scale (JFLS), and Surface Electromyography (sEMG) of chewing and swallowing muscles. The categorical or non-normal distribution data were analyzed by nonparametric Mann-Whitney statistical test and data with normal distribution analyzed by Variance Analysis (ANOVA), followed by post-test. The level of significance was P<0.05. Results: The SS group presented statistically significant differences in comparison with group C, with worse conditions in the orofacial myofunctional evaluation, regarding the appearance / posture of oral structures, mobility of lips, tongue, cheeks and mandible, as well as in the functions of chewing, swallowing and breathing. The oral opening, horizontal interincisal distance, tongue pressure on protrusion and elevation (mean and maximum value), as well as the value of swallowing were also statistically lower. SS patients showed by scores greater difficulty in chewing and risk of dysphagia (EAT-10). There were also higher in the SS group, compared to control, pain scores at palpation, the severity of TMD signs and symptoms (ProDTMmulti) and functional limitation of the mandible (JFLS). The activity of the masticatory muscles in the tests of maximum voluntary contraction (MCV) was also lower in the SS group with a greater impact per cycle during the masticatory EMG. The temporal changes in swallowing, with increased duration for the liquid consistency and reduced for solid swallowing were also significant in the SS group, compared to the control. Conclusion: the results showed that patients with Sjögren\'s Syndrome present marked impairments in musculature and orofacial functions and moderate TMD signs and symptoms, a perception of great functional limitation. Therefore, the consequences of SS are not limited to subjective complaints, but rather involve real system compromises and stomatognatic functions.

O HTLV-1 (human T-cell lymphotropic virus type 1) foi o primeiro retrovírus humano a ser identificado. É comprovadamente o agente etiológico da leucemia/linfoma de células T no adulto (ATLL) e da paraparesia espástica tropical ou mielopatia associada ao HTLV (HAM/TSP). Porém se evidencia que o vírus possa estar relacionado a várias outras manifestações sistêmicas. A Síndrome de Sjögren (SS) é uma das desordens que têm sido associada ao HTLV-1. Embora a infecção pelo HTLV seja reconhecidamente endêmica no Brasil, não há informações sobre essa associação na população brasileira. Este trabalho propõe-se a investigar a prevalência de SS em pacientes infectados pelo HTLV e a prevalência de HTLV em pacientes diagnosticados com SS. Exames sorológicos para investigação do HTLV foram realizados em 50 pacientes da Irmandade da Santa Casa de Misericórdia de São Paulo (ISCMSP) que apresentavam queixas compatíveis com a SS (grupo 1). No Instituto de Infectologia Emílio Ribas foram avaliados 129 pacientes HTLV+ que passaram pelo processo diagnóstico para a SS (grupo 2). Nenhum dos pacientes do grupo 1 apresentou soropositividade para o HTLV. No grupo 2, 46 (35,7%) apresentaram algum grau de xerostomia, 18 (13,95%) apresentaram xeroftalmia, 8 (6,2%) apresentaram hipossalivação, 2 (1,55%) apresentaram fluxo lacrimal alterado e 1 paciente (0,77%) apresentou autoanticorpos reagentes (anti-SSB). Foram executadas biópsias incisionais de glândulas salivares menores em 5 pacientes do grupo 2. Apenas 2 pacientes (1,55%) HTLV+ completaram os critérios para o diagnóstico de SS. A SS mostrou ser três vezes mais prevalente em pacientes HTLV+ do IIER do que nos pacientes que buscaram atendimento no serviço de Otorrinolaringologia da ISCMSP. / HTLV-1 (human T-cell lymphotropic virus type 1) was the first human retrovirus identified. It is proven to be the etiological agent of adult T-cell leukemia/lymphoma (ATLL) and of a neurological disease known as HTLV-1 associated myelopathy or tropical spastic paraparesis (HAM/TSP). However, there is the evidence that the virus could be related to several other systemic manifestations. Sjögren\'s Syndrome (SS) is one of the disorders that have been associated with HTLV-1. Although HTLV infection is known to be endemic in Brazil, there is no information about this association in Brazilian population. This study proposes to investigate the prevalence of SS among patients infected with HTLV and the prevalence of HTLV among patients diagnosed with SS. Serological tests for HTLV were performed in 50 patients from Irmandade da Santa Casa de Misericórdia de São Paulo (ISCMSP) with complaints compatible with the SS (group 1). At Institute of Infectious Diseases Emilio Ribas (IIER), 129 HTLV+ patients were evaluated and the diagnostic process for SS was performed (group 2). None of the patients in group 1 was positive for HTLV. In group 2, 46 (35.7%) reported any degree of xerostomia, 18 (13.95%) had xerophtalmia, hyposalivation was present in 8 (6.2%) patients and decrease in tear secretion, in only one patient (0.77%) the auto-antibodies was positive ( Anti-SSB). Incisional biopsies of labial minor salivary glands were executed in 5 patients in group 2. Only 2 HTLV+ patients (1.55%) have fulfilled the classification criteria for SS. SS proved to be three times more prevalent in HTLV patients from IIER than in patients who sought care in the service of Otorhinolaryngology at ISCMSP.

O HTLV-1 (human T-cell lymphotropic virus type 1) foi o primeiro retrovírus humano a ser identificado. É comprovadamente o agente etiológico da leucemia/linfoma de células T no adulto (ATLL) e da paraparesia espástica tropical ou mielopatia associada ao HTLV (HAM/TSP). Porém se evidencia que o vírus possa estar relacionado a várias outras manifestações sistêmicas. A Síndrome de Sjögren (SS) é uma das desordens que têm sido associada ao HTLV-1. Embora a infecção pelo HTLV seja reconhecidamente endêmica no Brasil, não há informações sobre essa associação na população brasileira. Este trabalho propõe-se a investigar a prevalência de SS em pacientes infectados pelo HTLV e a prevalência de HTLV em pacientes diagnosticados com SS. Exames sorológicos para investigação do HTLV foram realizados em 50 pacientes da Irmandade da Santa Casa de Misericórdia de São Paulo (ISCMSP) que apresentavam queixas compatíveis com a SS (grupo 1). No Instituto de Infectologia Emílio Ribas foram avaliados 129 pacientes HTLV+ que passaram pelo processo diagnóstico para a SS (grupo 2). Nenhum dos pacientes do grupo 1 apresentou soropositividade para o HTLV. No grupo 2, 46 (35,7%) apresentaram algum grau de xerostomia, 18 (13,95%) apresentaram xeroftalmia, 8 (6,2%) apresentaram hipossalivação, 2 (1,55%) apresentaram fluxo lacrimal alterado e 1 paciente (0,77%) apresentou autoanticorpos reagentes (anti-SSB). Foram executadas biópsias incisionais de glândulas salivares menores em 5 pacientes do grupo 2. Apenas 2 pacientes (1,55%) HTLV+ completaram os critérios para o diagnóstico de SS. A SS mostrou ser três vezes mais prevalente em pacientes HTLV+ do IIER do que nos pacientes que buscaram atendimento no serviço de Otorrinolaringologia da ISCMSP. / HTLV-1 (human T-cell lymphotropic virus type 1) was the first human retrovirus identified. It is proven to be the etiological agent of adult T-cell leukemia/lymphoma (ATLL) and of a neurological disease known as HTLV-1 associated myelopathy or tropical spastic paraparesis (HAM/TSP). However, there is the evidence that the virus could be related to several other systemic manifestations. Sjögren\'s Syndrome (SS) is one of the disorders that have been associated with HTLV-1. Although HTLV infection is known to be endemic in Brazil, there is no information about this association in Brazilian population. This study proposes to investigate the prevalence of SS among patients infected with HTLV and the prevalence of HTLV among patients diagnosed with SS. Serological tests for HTLV were performed in 50 patients from Irmandade da Santa Casa de Misericórdia de São Paulo (ISCMSP) with complaints compatible with the SS (group 1). At Institute of Infectious Diseases Emilio Ribas (IIER), 129 HTLV+ patients were evaluated and the diagnostic process for SS was performed (group 2). None of the patients in group 1 was positive for HTLV. In group 2, 46 (35.7%) reported any degree of xerostomia, 18 (13.95%) had xerophtalmia, hyposalivation was present in 8 (6.2%) patients and decrease in tear secretion, in only one patient (0.77%) the auto-antibodies was positive ( Anti-SSB). Incisional biopsies of labial minor salivary glands were executed in 5 patients in group 2. Only 2 HTLV+ patients (1.55%) have fulfilled the classification criteria for SS. SS proved to be three times more prevalent in HTLV patients from IIER than in patients who sought care in the service of Otorhinolaryngology at ISCMSP.

A Síndrome de Sjögren primária (SSp) é uma doença autoimune sistêmica de etiologia desconhecida, caracterizada principalmente, pela presença de infiltrado linfocítico nas glândulas exócrinas (salivares e lacrimais) que leva ao quadro de boca seca e olhos secos (síndrome sicca). Apesar das evidências de que o nível de atividade física e das capacidades física e funcional estarem diminuídas na maioria das doenças reumáticas, essas variáveis tem sido pouco estudadas em pacientes com SSp. Assim, o objetivo desse estudo foi avaliar objetivamente o nível de atividade física e as capacidades físicas de pacientes com SSp com baixo nível de atividade da doença, bem como, a possível associação desses fatores com a fadiga, a qualidade de vida, a artralgia e a depressão. Trinta pacientes diagnosticadas com SSp (Vitali et al., 2002) utilizaram acelerômetros por 7 dias consecutivos para avaliação do nível de atividade física, realizaram teste ergoespirométrico, testes de força, testes funcionais, exames laboratoriais, densitometria óssea e responderam a questionários de fadiga, qualidade de vida, depressão e artralgia. Os pacientes com SSp apresentaram menor tempo em atividades físicas moderadas e menor quantidade de passos por dia do que o grupo controle. Os pacientes apresentaram ainda redução da aptidão aeróbia, da força muscular de membros inferiores e superiores, da capacidade funcional e da qualidade de vida, além de fadiga aumentada em comparação com o GC. Não foram encontradas diferenças significativas para a composição corporal, artralgia e depressão entre os grupos. Pacientes com SSp mesmo em baixa atividade da doença mantêm um estilo de vida sedentário e redução das capacidades físicas e qualidade de vida. Faz-se necessária a implementação de estratégias que reduzam o tempo gasto em atividades sedentárias, bem como, a elaboração de programas de exercícios físicos sistematizados de acordo com as características dessa população / Primary Sjögren´s syndrome (PSS) is a systemic autoimune disease of unknown aetiology characterized by lymphocytic infiltration in the exocrine glands (salivary and lacrimal) hwhich leads to dry mouth and dry eyes (sicca syndrome). Despite the evidence that physical activity level and physical and functional capabilities are reduced in most rheumatic diseases, these variables have been poorly studied in patients with pSS. The objective of this study was to objectively assess the physical activity level and the physical capacities of patients with pSS with mild disease activity, as well as the possible association of these factors with fatigue, quality of life, arthralgia and depression. Thirty patients diagnosed with pSS (Vitali et al., 2002) used accelerometers for 7 consecutive days to assess the physical activity level. They performed cardiopulmonary exercise test, strength tests, functional tests, laboratory tests, bone densitometry and answered questionnaires of fatigue, quality of life, depression and arthralgia. Patients with pSS showed less time in moderate physical activities and fewer steps per day than the control group. The patients also showed reduced aerobic conditioning, muscle strength of upper and lower limbs, functional capacity and quality of life, and also increased fatigue compared to the control group. No significant differences were found for body composition, arthralgia and depression between the groups. Patients with pSS, even with mild disease activity, maintain a sedentary lifestyle and reduced physical capacity and quality of life. It is necessary to implement strategies that reduce time spent on sedentary activities, as well as to elaborate physical exercise programs in accordance to the characteristics of this population

A Síndrome de Sjögren primária (SSp) é uma doença autoimune sistêmica de etiologia desconhecida, caracterizada principalmente, pela presença de infiltrado linfocítico nas glândulas exócrinas (salivares e lacrimais) que leva ao quadro de boca seca e olhos secos (síndrome sicca). Apesar das evidências de que o nível de atividade física e das capacidades física e funcional estarem diminuídas na maioria das doenças reumáticas, essas variáveis tem sido pouco estudadas em pacientes com SSp. Assim, o objetivo desse estudo foi avaliar objetivamente o nível de atividade física e as capacidades físicas de pacientes com SSp com baixo nível de atividade da doença, bem como, a possível associação desses fatores com a fadiga, a qualidade de vida, a artralgia e a depressão. Trinta pacientes diagnosticadas com SSp (Vitali et al., 2002) utilizaram acelerômetros por 7 dias consecutivos para avaliação do nível de atividade física, realizaram teste ergoespirométrico, testes de força, testes funcionais, exames laboratoriais, densitometria óssea e responderam a questionários de fadiga, qualidade de vida, depressão e artralgia. Os pacientes com SSp apresentaram menor tempo em atividades físicas moderadas e menor quantidade de passos por dia do que o grupo controle. Os pacientes apresentaram ainda redução da aptidão aeróbia, da força muscular de membros inferiores e superiores, da capacidade funcional e da qualidade de vida, além de fadiga aumentada em comparação com o GC. Não foram encontradas diferenças significativas para a composição corporal, artralgia e depressão entre os grupos. Pacientes com SSp mesmo em baixa atividade da doença mantêm um estilo de vida sedentário e redução das capacidades físicas e qualidade de vida. Faz-se necessária a implementação de estratégias que reduzam o tempo gasto em atividades sedentárias, bem como, a elaboração de programas de exercícios físicos sistematizados de acordo com as características dessa população / Primary Sjögren´s syndrome (PSS) is a systemic autoimune disease of unknown aetiology characterized by lymphocytic infiltration in the exocrine glands (salivary and lacrimal) hwhich leads to dry mouth and dry eyes (sicca syndrome). Despite the evidence that physical activity level and physical and functional capabilities are reduced in most rheumatic diseases, these variables have been poorly studied in patients with pSS. The objective of this study was to objectively assess the physical activity level and the physical capacities of patients with pSS with mild disease activity, as well as the possible association of these factors with fatigue, quality of life, arthralgia and depression. Thirty patients diagnosed with pSS (Vitali et al., 2002) used accelerometers for 7 consecutive days to assess the physical activity level. They performed cardiopulmonary exercise test, strength tests, functional tests, laboratory tests, bone densitometry and answered questionnaires of fatigue, quality of life, depression and arthralgia. Patients with pSS showed less time in moderate physical activities and fewer steps per day than the control group. The patients also showed reduced aerobic conditioning, muscle strength of upper and lower limbs, functional capacity and quality of life, and also increased fatigue compared to the control group. No significant differences were found for body composition, arthralgia and depression between the groups. Patients with pSS, even with mild disease activity, maintain a sedentary lifestyle and reduced physical capacity and quality of life. It is necessary to implement strategies that reduce time spent on sedentary activities, as well as to elaborate physical exercise programs in accordance to the characteristics of this population

<p>Primary Sjögren's syndrome (pSS) is a chronic inflammatory connective tissue disease of unknown etiology. The disease primarily involves salivary and lacrimal glands which results in oral and ocular dryness (sicca symptoms). A wide spectrum of extraglandular features from various organs may be seen. </p><p>In this thesis, the frequency of psychiatric symptoms in women with primary Sjögren's syndrome was studied and an attempt was made to assess how these symptoms might influence their well being and quality of life. The main finding was that the women with pSS suffered significantly more often from symptoms of anxiety and depression when compared with age matched, healthy females and female patients with rheumatoid arthritis. The physical and mental well-being of the patients with pSS was significantly reduced compared to patient controls. </p><p>The possible link of psychiatric symptoms to the altered function of the hypothalamic-pituitary-gonadal axis and adrenal androgen secretion was elucidated. Women with pSS have intact cortisol synthesis but reduced serum concentrations of dehydroepiandrosterone sulphate (DHEA-S) (p<0.05) and an increased cortisol/DHEA-S ratio (p<0.05), compared to healthy controls. These findings may reflect a constitutional or disease-meditated influence on adrenal steroid synthesis. Positive correlation was found between DHEA-S serum levels and quality of sexual life (p<0.01) and mental well-being (p<0.01) in women with pSS. </p>

Primary Sjögren's syndrome (pSS) is a chronic inflammatory connective tissue disease of unknown etiology. The disease primarily involves salivary and lacrimal glands which results in oral and ocular dryness (sicca symptoms). A wide spectrum of extraglandular features from various organs may be seen. In this thesis, the frequency of psychiatric symptoms in women with primary Sjögren's syndrome was studied and an attempt was made to assess how these symptoms might influence their well being and quality of life. The main finding was that the women with pSS suffered significantly more often from symptoms of anxiety and depression when compared with age matched, healthy females and female patients with rheumatoid arthritis. The physical and mental well-being of the patients with pSS was significantly reduced compared to patient controls. The possible link of psychiatric symptoms to the altered function of the hypothalamic-pituitary-gonadal axis and adrenal androgen secretion was elucidated. Women with pSS have intact cortisol synthesis but reduced serum concentrations of dehydroepiandrosterone sulphate (DHEA-S) (p&lt;0.05) and an increased cortisol/DHEA-S ratio (p&lt;0.05), compared to healthy controls. These findings may reflect a constitutional or disease-meditated influence on adrenal steroid synthesis. Positive correlation was found between DHEA-S serum levels and quality of sexual life (p&lt;0.01) and mental well-being (p&lt;0.01) in women with pSS.

Introduktion: Torra ögon (DES) och Sjögrens syndrom (SjS) är inflammatoriska sjukdomar som drabbar ögonen. DES drabbar tårkörtelns funktionella del vilket ger ögonsmärta och suddig syn. SjS är en kronisk autoimmun sjukdom som ger skada på tår- och salivproducerande körtlar. Det leder till kliniska symtom i form av mun- och ögontorrhet. Essentiella fettsyror bildar proinflammatoriska och antiinflammatoriska cytokiner som kan hjälpa vid behandling av dessa sjukdomar. Metod och syfte: En litteraturstudie genomfördes för att ta reda på om fleromättade fetter kan lindra symtomen vid SjS och DES. Resultat: Resultaten för studierna visade resultat på OSDI, Schirmers test, BUT, IL-17, tårosmolaritet, tårvolym, ostimulerat salivflöde, stimulerat salivflöde, sonderingsdjup, PGE1, van Bijstervelds poäng och flourscein infärgning. Diskussion: Resultatet visar signifikanta skillnader för vissa doser av n-3 och n-6. Det som skiljer resultaten åt är de olika doserna av fettsyror samt vad dess placebokontroller intog. OSDI visade främst skillnader på de patienter med DES men endast på en grupp med SjS som intog n-3 för behandling av torr mun. Schirmers test och BUT visade en ökning hos patienterna med mild och måttlig DES men inte på patienterna med SjS. Patienterna med SjS visade förbättringar på stimulerat salivflöde och OSDI för torr mun samt PGE1 nivåer och flourscein infärgning samtidigt som en del studier inte visade några signifikanta förbättringar på några mätvariabler. Det kan grunda sig i intaget av fettsyror, sjukdomstillstånd eller längden på behandling. Slutsats: En del personer kan få förbättrade symtom av att inta n-3 eller n-6 tillskott men skillnaderna är inte alltid statistiskt signifikanta för studierna. / Introduction: Dry eye syndrome (DES) and Sjögren ́s syndrome (SjS) are inflammatory diseases that affect the eyes. DES affects the lacrimal glands functional unit, causing eye pain and blurred vision. SjS is a chronical autoimmune disease that causes damage to tear and salivary glands. It leads to clinical symptoms in the form of mouth and eye irritation. Essential fatty acids form pro-inflammatory and anti-inflammatory cytokines that can help in the treatment of these diseases. Method and purpose: A literature study was performed to study if essential fatty acids can relieve the symptoms of DES and SjS. Results: The results for the studies showed results on OSDI, Schirmer ́s test, BUT, IL- 17, tear osmolarity, tear volume, unstimulated salivary flow, stimulated salivary flow, depth of probing, van Bijsterveld ́s score and flourscein staining. Discussion: The result shows significant differences for certain doses of n-3 and n-6. What separates the results are the different doses of fatty acids and what their placebo controls took. OSDI showed major differences in patients with DES but only in one group of patients with SjS who took n-3 for treating dry mouth. Schirmer ́s test and BUT showed an increase in patients with mild and moderate DES but not in patients with SjS. Patients with SjS showed improvements in stimulated salivary flow, dry mouth OSDI, PGE1 levels and flourscein staining, while some studies did not show any significant improvements in any measurement variables. It may be due to the intake of fatty acids, disease states or the length of treatment. Conclusion: Some people may get improved symptoms of taking n- 3 or n-6 supplements, but the differences are not always statistically significant for the studies.

<p>Patients with systemic lupus erythematosus (SLE) have an activated type I interferon (IFN) system with an ongoing IFN-α synthesis. This may be caused by circulating immune complexes, consisting of anti-DNA antibodies (Abs) and DNA, with IFN-α inducing capacity. Produced IFN-α may be crucial in the pathogenesis, because this cytokine can break tolerance and promote autoimmunity.</p><p>In the present thesis, possible mechanisms of the IFN-α production in SLE were studied. To investigate whether IFN-α inducing material could be derived from apoptotic cells, IgG from SLE patients (SLE-IgG) were combined with apoptotic cells. This combination induced high IFN-α production in normal peripheral blood mononuclear cells (PBMC). The IFN-α induction was associated to presence of anti-RNP Abs, but not to anti-dsDNA Abs, indicating that two inducers could be active in SLE, one containing DNA and the other RNA.</p><p>Apoptotic cells and SLE-IgG exclusively activated the natural interferon producing cells (NIPC) and the IFN-α response was enhanced by type I IFN and inhibited by IL-10 and TNF-α. The IFN-α induction was dependent on FcγRII, because blocking this receptor reduced IFN-α production and NIPC were found to express FcγRIIa.</p><p>To further elucidate the role of different autoantibodies in the IFN-α induction, sera from patients with Sjögren´s syndrome (SS), containing autoantibodies to RNA binding proteins (SSA, SSB, RNP and/or Sm) were investigated. The combination of SS or SLE sera and apoptotic or necrotic cell material induced high IFN-α production in PBMC. RNA, but not DNA, was required for IFN-α induction, indicating that RNA and Abs to RNA-binding proteins form potent IFN-α inducing complexes.</p><p>The findings in this thesis can explain central mechanisms for the activation of NIPC in SLE, and perhaps also other autoimmune diseases. This activation is mediated by interferogenic immune complexes, and modulating the NIPC activation may be a novel therapeutic approach in SLE.</p>