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ABSTRACT Purpose: To compare the 3-month results of treatment with 20% autologous serum or combination treatment with preservative-free artificial tears and 0.05% cyclosporine in patients with dry eye disease due to primary Sjögren's syndrome. Methods: A total of 130 eyes of 65 patients with newly diagnosed dry eye disease due to primary Sjögren's syndrome were included in the study. The patients were divided into two treatment groups: 66 eyes of 33 patients were assigned to the autologous serum treatment group, and 64 eyes of 32 patients were assigned to the combination treatment group. Schirmer test, tear break-up time and Ocular Surface Disease Index (OSDI) scores were recorded at pretreatment and at 3 months of treatment. Results: At 3 months of treatment, the mean Schirmer value and the mean tear break-up time were significantly higher in the combination treatment group (p<0.0001 and p=0.034, respectively). The OSDI score at 3 months was significantly lower in the autologous serum Group (p=0.004). When the two groups were evaluated separately, the improvements in Schirmer, tear break-up time test, and OSDI scores from before to after treatment were statistically significant: p<0.0001, p<0.001, and p<0.0001, respectively, for the authologus serum Group, and p<0.0001, p<0.001, and p<0.0001, respectively, for the combination treatment group. Conclusions: In short-term treatment of dry eye disease due to primary Sjögren's syndrome, treatment with autologous serum was significantly superior to -combination treatment with preservative-free artificial tears and 0.05% cyclosporine in terms of improvement in OSDI scores. Improvements in Schirmer test and tear break-up time scores were significantly superior in the group treated with preservative-free artificial tears and 0.05% cyclosporine.
RESUMO Objetivo: Comparar os resultados de 3 meses de soro autólogo a 20% com um tratamento combinado, ou seja, lubrificantes oculares sem conservantes e ciclosporina a 0,05% em pacientes com síndrome do olho seco devida à síndrome de Sjögren primária. Métodos: Foram incluídos no estudo 130 olhos de 65 pacientes recentemente diagnosticados com síndrome do olho seco devida à síndrome de Sjögren primária. Os pacientes foram divididos em dois grupos de tratamento, 66 olhos de 33 pacientes foram incluídos no grupo de tratamento com soro autólogo e 64 olhos de 32 pacientes foram incluídos no grupo de tratamento combinado com lubrificantes oculares sem conservantes e ciclosporina. Os resultados do teste de Schirmer e do tempo de ruptura do filme lacrimal e os índices de doença da superfície ocular (OSDI) foram registrados antes e depois de três meses de tratamento. Resultados: Três meses após o tratamento, o valor médio do teste de Schirmer foi mais alto com significância estatística no grupo do tratamento combinado com lubrificantes oculares sem conservantes e ciclosporina (p<0,0001) e o tempo de ruptura do filme lacrimal também foi significativamente maior nesse grupo (p=0,034). Também aos três meses, a doença da superfície ocular foi menor com significância estatística no grupo de tratamento com soro autólogo (p=0,004). Quando os dois grupos foram avaliados separadamente, a melhora no teste de Schirmer, o tempo de ruptura e a doença da superfície ocular antes e depois do tratamento tiveram diferenças estatisticamente significativas tanto no grupo de soro autólogo (p<0,0001, p<0,001 e p<0,0001, respectivamente) quanto no grupo de tratamento combinado (p<0,0001, p<0,001 e p<0,0001, respectivamente). Conclusões: No tratamento de curto prazo da síndrome do olho seco devida à síndrome de Sjögren primária, o tratamento com soro autólogo foi significativamente superior ao tratamento com lubrificantes oculares sem conservantes combinados com ciclosporina, em termos de melhora no doença da superfície ocular. As melhoras no teste de Schirmer e no tempo de ruptura do filme lacrimal foram significativamente maiores no grupo de tratamento combinado com lubrificantes oculares sem conservantes e ciclosporina.
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ABSTRACT Purpose: This clinical study compared autologous serum eye drops diluted with 0.5% methylcellulose and 0.9% saline solution. The subjective criteria for symptom improvement and the objective clinical criteria for response to therapy were evaluated. Methods: This longitudinal prospective study enrolled 23 patients (42 eyes) with persistent epithelial defects or severe dry eye disease refractory to conventional therapy who had been using autologous serum 20% prepared with methylcellulose for > 6 months and started on autologous serum diluted in 0.9% saline solution. The control and intervention groups consisted of the same patients under alternate treatments. The subjective criteria for symptom relief were evaluated using the Salisbury Eye Evaluation Questionnaire. The objective clinical criteria were evaluated through a slit-lamp examination of the ocular surface, tear breakup time, corneal fluorescein staining, Schirmer's test, rose Bengal test, and tear meniscus height. These criteria were evaluated before the diluent was changed and after 30, 90, and 180 days. Results: In total, 42 eyes were analyzed before and after 6 months using autologous serum diluted with 0.9% saline. No significant differences were found in the subjective criteria, tear breakup time, tear meniscus, corneal fluorescein staining, or rose Bengal test. Schirmer's test scores significantly worsened at 30 and 90 days (p=0.008). No complications or adverse effects were observed. Conclusions: This study reinforces the use of autologous serum 20% as a successful treatment for severe dry eye disease resistant to conventional therapy. Autologous serum in 0.9% saline was not inferior to the methylcellulose formulation and is much more cost-effective.
RESUMO Objetivo: Este estudo comparou o colírio de soro au tólogo manipulado com metilcelulose a 0,5% com solução salina 0,9%. Critérios subjetivos de melhora dos sintomas e critérios clínicos objetivos para resposta à terapia foram avaliados. Métodos: Este estudo prospectivo longitudinal envolveu 23 pacientes (42 olhos) com defeitos epiteliais persistentes ou doença de olho seco grave refratária à terapia convencional que usavam colírio de soro autólogo 20% preparado com metilcelulose por mais de 6 meses e iniciaram soro autólogo diluído em solução salina 0,9%. Os grupos controle e intervenção consistiam dos mesmos pacientes sob tratamentos alternados. Os critérios subjetivos para o alívio dos sintomas foram avaliados usando o Salisbury Eye Evaluation Questionnaire. Os critérios objetivos foram avaliados por meio de exame em lâmpada de fenda incluindo: tempo de ruptura da lágrima, coloração da córnea com fluoresceína, teste de Schirmer, coloração com rosa bengala e altura do menisco lacrimal. Esses critérios foram avaliados antes da troca do diluente e após 30, 90 e 180 dias. Resultados: Um total de 42 olhos foram analisados antes e após 6 meses usando soro autólogo diluído com solução salina 0,9%. Nenhuma diferença significativa foi encontrada nos critérios subjetivos, tempo de ruptura da lágrima, menisco lacrimal, coloração com fluoresceína ou rosa bengala. Os resultados dos testes de Schirmer pioraram significativamente em 30 e 90 dias (p=0,008). Não foram observadas complicações ou efeitos adversos. Conclusões: Este estudo reforça o uso do colírio de soro autólogo 20% como um tratamento de sucesso para a doença do olho seco grave resistente à terapia convencional. O soro autólogo diluído em solução salina a 0,9% não foi inferior à formulação de metilcelulose.
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ObjectiveTo investigate the clinical efficacy and safety of iguratimod combined with the Chinese medicine Runzaoling in the treatment of primary Sjögren's syndrome （pSS）. MethodSeventy-two patients treated in the Department of Rheumatology and Immunology of the Second Affiliated Hospital of Guizhou University of Traditional Chinese Medicine（TCM） from January 2021 to June 2022 who met the Western medical diagnosis of pSS and had the TCM syndrome of Yin deficiency and heat toxin syndrome were randomly assigned into an observation group and a control group， with 36 patients in each group. The observation group was treated with iguratimod combined with Runzaoling， and the control group was treated with iguratimod. The treatment in both groups lasted for 12 weeks. The clinical symptoms， EULAR Sjogren's syndrome patient reported index （ESSPRI）， EULAR Sjögren's syndrome disease activity index （ESSDAI）， erythrocyte sedimentation Rate （ESR）， C-reactive protein （CRP）， immunoglobulin （IgG）， Schirmer score， and saliva flow of the two groups were determined before and after treatment. Furthermore， the incidence of adverse reactions was compared between the two groups. ResultThe total response rate in the observation group was 75.0% （27 patients with response and 9 patients with no response）， which was higher than that （61.11%， 22 patients with response and 14 patients without response） in the control group （P<0.05）. After treatment， the ESSPRI， ESSDAI， and TCM syndrome scores in both groups decreased and the decreases were more obvious in the observation group than in the control group （P<0.05）. The treatment in both groups recovered the ESR， CRP， IgG， Schirmer score， and saliva flow （P<0.05）. Moreover， the observation outperformed the control group in terms of the ESR， CRP， IgG， and saliva flow （P<0.05） and had no significant difference in the Schirmer score compared with the control group. During the treatment period， 2 patients in the observation group had nausea， and 1 patient had an abnormal liver function， which were relieved after symptomatic treatment and did not affect the treatment. In the control group， 1 patient withdrew from the study due to rashes and showed no special discomfort in the follow-up 4 weeks， and 1 patient had nausea， which was relieved after symptomatic treatment. ConclusionIguratimod combined with Runzaoling has good clinical efficacy and safety in the treatment of pSS.
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Screening and evaluating the diseases responding specifically to traditional Chinese medicine （TCM） will help to highlight the advantages of TCM treatment， and the evaluation method should be standardized with consideration to the unique characteristics of the diseases. The incidence of Sjögren's Syndrome （SS） is increasing year by year， while the pathogenesis of this disease remains unclear. Modern therapies for this disease include biological agents and immunosuppressants， which generally have unsatisfactory efficacy. The TCM treatment of SS focuses on the harmony of the physical and mental health. The Rheumatology Branch of the China Association of Chinese Medicine organizes experts in TCM， Western medicine， and evidence-based medicine to form working groups. Delphi method and bibliometric method were used for analysis， and SS was selected as a disease responding specifically to TCM. Furthermore， the evaluation system was established for this disease， and the consensus regarding this disease was reached after seminar discussion. This paper summarized the whole process of the evaluation of the advantages of TCM treatment of SS. First， because TCM atomization is widely used in clinical practice and enriches TCM administration methods， this therapy is included after other non-drug therapies were taken as characteristic therapies. Second， the evaluation indicators of therapeutic effect should be determined with consideration to international acceptance and the current research status. Third， the expression method should be accurate， standardized， and objective， highlight the natural advantages of TCM， and avoid arbitrary extension. This paper provides a reference for clinicians to explore other diseases responding specifically to TCM.
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La encefalitis autoinmune es un trastorno inmunomediado que compromete distintos territorios del parénquima cerebral, involucrando frecuentemente la materia gris profunda o la corteza, con o sin compromiso de la materia blanca, meninges o médula espinal. Se asocia frecuentemente con enfermedades autoinmunes o paraneoplásicas, y constituye un reto diagnóstico. Reportamos el caso de una mujer de 55 años con antecedente de síndrome de Sjögren que consultó a Emergencias por cefalea y confusión. El líquido cefalorraquídeo (LCR) presentaba leucocitosis con neutrofilia. En la resonancia magnética nuclear (RMN) cerebral se evidenciaron múltiples imágenes de comportamiento restrictivo, de señal hiperintensa en T2 y FLAIR, a predominio córtico-subcortical a nivel occipital bilateral, hemisferio cerebeloso derecho y parietal derecho. Se descartaron infecciones y neoplasias. El panel de anticuerpos para encefalitis autoinmune aquaporina-4 y anti-MOG en LCR fue negativo. Recibió metilprednisolona endovenosa con mejoría progresiva de los síntomas.
Autoimmune encephalitis is an immune-mediated disorder that affects different areas of the brain parenchyma, often involving deep gray matter or the cortex, with or without involvement of white matter, meninges, or spinal cord. It is frequently associated with autoimmune or paraneoplastic diseases and is a diagnostic challenge. We report the case of a 55-year-old woman with history of Sjögren's syndrome who presented to the emergency department with headache and episodes of confusion. Cerebrospinal fluid (CSF) analysis showed leukocytosis with neutrophilia. Brain MRI revealed multiple restricted diffusion lesions with hyperintense signal on T2 and FLAIR sequences, predominantly in the bilateral occipital region, right cerebellar hemisphere, and right parietal region. Infections and neoplasms were ruled out. The panel of antibodies for autoimmune encephalitis, including Aquaporin-4 and anti-MOG in CSF, was negative. She received intravenous methylprednisolone, leading to symptom improvement.
Subject(s)
Female , Central Nervous SystemABSTRACT
Introducción: recientemente, en Europa y en idioma inglés, se ha desarrollado el Clinical EULAR Sjögren's Syndrome Disease Activity Index (ClinESSDAI) para evaluar la actividad en pacientes con síndrome de Sjögren primario (SSp). Objetivos: validar el ClinESSDAI en pacientes con SSp en Argentina. Materiales y métodos: estudio de corte transversal. Se utilizó la versión en castellano del EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) validada en Argentina. Para evaluar la validez del constructo, se usó la escala visual análoga (EVA) desarrollada por un reumatólogo experto por dominio del ClinESSDAI y de la EVA global para el puntaje total del ClinESSDAI, mientras que otro profesional en la materia realizó el ESSDAI y ClinESSDAI. Para analizar la reproducibilidad, se estudió a un subgrupo de pacientes, sin mediar cambios en el tratamiento ni en la condición clínica, 10 días después de la evaluación basal. Todos los médicos examinaron a los pacientes desconociendo la evaluación de los demás colegas. Resultados: se incluyeron 47 pacientes con SSp. La correlación entre la EVA global y el ClinESSDAI fue muy buena (Rho 0,7), así como la correlación de la EVA y el ClinESSDAI de cada dominio. El coeficiente de correlación intraclase (CCI) entre el ESSDAI y el ClinESSDAI fue de 0,98. La reproducibilidad fue de 0,93. Conclusiones: el ClinESSDAI es una herramienta válida y reproducible en nuestra población, equiparable al ESSDAI.
Introduction: the Clinical EULAR Sjögren's Syndrome Disease Activity Index (ClinESSDAI) has recently been developed in Europe and in the English language to evaluate activity in patients with primary Sjögren's syndrome (pSS). Objectives: validate the ClinESSDAI in patients pSS in Argentina. Materials and methods: a cross-sectional study. The Spanish version of the ESSDAI, validated in Argentina, was used. To evaluate construct validity, the Visual Analog Scale (VAS) was used, performed by an expert rheumatologist per ClinESSDAI domain, and the global VAS was used for the total score of the ClinESSDAI, while another professional performed the ESSDAI and ClinESSDAI. To evaluate reproducibility, a subgroup of patients was evaluated without changes in treatment or clinical condition 10 days after the baseline evaluation. All physicians were blind to each other's evaluation. Results: 47 patients with pSS were included. The correlation between global VAS and ClinESSDAI was very good (Rho 0.7), as well as the correlation of the VAS and ClinESSDAI of each domain. The intraclass correlation coefficient (ICC) between ESSDAI and ClinESSDAI was 0.98. The reproducibility was 0.93. Conclusions: the ClinESSDAI is a valid and reproducible tool in our population, comparable to the ESSDAI.
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Abstract Sjogren's syndrome (SS) is a complex autoimmune disease characterized by lymphocytic infiltration of salivary and lacrimal glands, resulting in sicca symptoms. Additionally, SS presents with neurological manifestations that significantly impact the nervous system. This review aims to provide a comprehensive overview of the neurological aspects of SSj, covering both the peripheral and central nervous system involvement, while emphasizing diagnosis, treatment, and prognosis.
Resumo A síndrome de Sjogren (SS) é uma doença autoimune complexa caracterizada pela infiltração linfocítica das glândulas salivares e lacrimais, resultando em sintomas sicca. Além disso, a SS apresenta manifestações neurológicas que afetam significativamente o sistema nervoso. Esta revisão tem como objetivo fornecer uma visão abrangente dos aspectos neurológicos da SSj, abordando tanto o envolvimento do sistema nervoso periférico quanto do central, com ênfase no diagnóstico, tratamento e prognóstico.
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Sjögren's syndrome is a systemic autoimmune disease characterized by dry eyes and mouth due to the involvement of exocrine glands. However, it can manifest with GI symptoms that cover a broad spectrum from esophageal and intestinal dysmotility, achalasia, hypochlorhydria, and chronic atrophic gastritis to pancreatic enzyme deficiency, biliary dysfunction, and liver cirrhosis, which varies in its clinical manifestations and is often associated with erroneous approaches. This article reviews the GI manifestations of Sjögren's syndrome. It presents the case of a woman in her eighth decade of life with this syndrome. She showed asymptomatic hepatobiliary disease, documented abnormalities in liver profile tests, and a subsequent diagnosis of primary sclerosing cholangitis, for which she received initial treatment with ursodeoxycholic acid. During her condition, the patient has had three episodes of cholangitis, requiring endoscopic retrograde cholangiopancreatography with no findings of stones, with scant biliary sludge and discharge of purulent bile precipitated by her underlying liver disease. The association between Sjögren's syndrome and primary sclerosing cholangitis is rare and calls for special consideration.
El síndrome de Sjögren es una enfermedad autoinmune sistémica que se caracteriza por la sequedad ocular y bucal debido a la afección de glándulas exocrinas; sin embargo, puede manifestarse con síntomas gastrointestinales que abarcan un espectro amplio desde la dismotilidad esofágica e intestinal, acalasia, hipoclorhidria y gastritis crónica atrófica hasta enzimodeficiencia pancreática, disfunción biliar y cirrosis hepática, que tiene variación en sus manifestaciones clínicas y se asocia con abordajes erróneos en muchas ocasiones. En este artículo se hace una revisión acerca de las manifestaciones gastrointestinales de síndrome de Sjögren y se presenta el caso de una mujer en la octava década de la vida con este síndrome, que cursa con enfermedad hepatobiliar asintomática, documentación de alteración en pruebas de perfil hepático y diagnóstico ulterior de colangitis esclerosante primaria, por lo que recibió un tratamiento inicial con ácido ursodesoxicólico. Durante el curso de su enfermedad ha presentado 3 episodios de colangitis, con requerimiento de colangiopancreatografía retrógrada endoscópica sin hallazgos de cálculos, con escaso barro biliar y salida de bilis purulenta, precipitada por su enfermedad hepática de base. La asociación entre el síndrome de Sjögren y la colangitis esclerosante primaria es infrecuente y justifica una consideración especial.
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El síndrome de Sjögren (SS) es una enfermedad autoinmune que afecta a las glándulas salivales y lagrimales. Se expone el caso clínico de una paciente de 67 años de género femenino que consulta por dolor en múltiples dientes; relata sensación de boca seca (xerostomía) y sequedad ocular (xeroftalmia). Al examen intraoral, se observan múltiples caries en superficies atípicas, mucosas secas, saliva espumosa y notoria depapilación lingual. Se sospecha de SS, derivando a medicina interna y confirmándose el diagnóstico. En paralelo, se inicia el tratamiento odontológico, realizando adaptaciones en los procedimientos para aliviar la sintomatología del SS, especialmente durante tratamientos endodónticos.
Sjögren's syndrome (SS) is an autoimmune disease that affects the salivary and lacrimal glands. The clinical case of a 67-year-old female patient who consulted for pain in multiple teeth is exposed. Additionally, she reports a sensation of dry mouth (xerostomia) and dry eyes (xerophthalmia). During the intraoral examination, the following findings are noticed: multiple cavities on atypical surfaces, dry mucous membranes, foamy saliva, and atrophic glossitis. SS is suspected, referring to internal medicine and confirming the diagnosis. In parallel, dental treatment is initiated, making adaptations in the procedures to alleviate the symptoms of SS, especially during endodontic treatments.
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Sjogren’s syndrome is a chronic and slowly progressing autoimmune disease characterized by lyphocytic infiltration of exocrine glands resulting in Sicca syndrome (xerostomia and keratocunjuntivitis sicca). The disease can present alone or along with other autoimmune diseases leading to significant organ specific and systemic disease. Middle aged women (Female: Male: 9:1) are primarily affected. Extraglandular (systemic) manifestations are seen in one third of patients with Sjogren’s syndrome. Among the extraglandular manifestations, renal involvement is commonly seen. Renal involvement in the form of tubulointerstitial nephritis (TIN) is more common compared to glomerular involvement. Distal renal tubular acidosis (RTA) is more common manifestation of TIN presenting as mild hypokalemia, metabolic acidosis, and rarely with hypokalemic periodic paralysis. We report three cases of hypokalemic periodic paralysis with metabolic acidosis, two in respiratory paralysis, diagnosed as distal RTA. On further evaluation of distal RTA, the patient diagnosed to have Sjogren’s syndrome and managed accordingly. Our report shows that Sjogren’s syndrome is a rare but important cause of hypokaemic periodic paralysis due to RTA.
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Purpose: The objective of the study was to compare the efficacy and safety of two concentration of autologous serum (AS) 20% vs 50% in recalcitrant moderate?to?severe dry eye patients. Methods: A double?blind prospective, interventional, and randomized study was done on 44 patients (80 eyes) clinically diagnosed with moderate?to?severe dry eye disease (DED) that was refractory to conventional treatment, and all patients were treated with AS20% or AS50% for 12 weeks. We documented Ocular Surface Disease Index (OSDI), tear film breakup time (TBUT), OXFORD corneal staining score (OSS), and Schirmer test (ST) at baseline, 2,4,8, and 12 weeks. These parameters were compared in both groups and between the groups by using Student’s t?test. The study included 11 males and 33 females. Results: Out of 80 eyes, 33 eyes had moderate and 47 had severe DED. The age of patients in AS20% was 44.73 ± 14.37 years, and in AS50% was 46.41 ± 14.47 years. The most common etiology associated with DED was secondary Sjogren syndrome. In moderate DED, both the groups showed significant improvement in both subjective and objective parameters. But in severe DED, the AS20% group failed to show any significant improvement objectively, though subjective improvement was present. Conclusion: In refractory severe DED patients, AS50% is better option for treatment and in moderate DED both concentrations of autologous serum are effective.
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Purpose: To understand the bacterial microbiome changes associated with Sjogren’s syndrome (SS) and non?Sjogren’s syndrome (NSS) aqueous?deficient dry eyes compared to healthy eyes. Methods: Bacterial microbiome was generated from the deoxyribonucleic acid of tear film samples in healthy (n = 33), SS (n = 17), and NSS (n = 28) individuals. Sequencing of the V3?V4 region of the 16S rRNA gene was performed on the Illumina HiSeq2500 platform. Quantitative Insights Into Microbial Ecology (QIIME) pipeline was used to assign taxa to sequences. Statistical analysis was performed in R to assess the alpha diversity and beta diversity indices. Significant changes between the healthy, SS, and NSS cohorts were depicted by principal coordinate analysis (PCoA), differential abundance, and network analysis. Results: Tear microbiome was generated in healthy, SS, and NSS samples. Phyla Actinobacteria, Firmicutes, and Bacteroidetes showed significant changes in SS and NSS compared to healthy. Genera Lactobacillus and Bacillus were predominantly present in all samples. PCoA and heat map analysis showed distinct clusters for SS and NSS from the healthy cohort. Genera Prevotella, Coriobacteriaceae UCG?003, Enterococcus, Streptomyces, Rhodobacter, Ezakiella, and Microbacterium significantly increased in abundance in SS and NSS compared to a healthy cohort. Bacteria–bacteria interaction in SS, NSS, and healthy cohorts was predicted by CoNet network analysis. This analysis predicted a major hub of interaction for the pro?inflammatory bacterium Prevotella in the SS and NSS cohorts. Conclusion: The results of the study indicate significant changes in the phyla and genera in SS and NSS compared to healthy. Both discriminative analysis and network analysis indicated a possible association of predominant pro?inflammatory bacteria with SS and NSS.
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Dry eye disease (DED) is a broad term that includes a diverse group of clinical disorders. Aqueous-deficient dry eye (ADDE), a subtype of DED, is characterized by decreased tear production by the lacrimal gland. It can be seen in up to one-third of individuals with DED and can be comorbid with a systemic autoimmune process or occur secondary to an environmental insult. Since ADDE can be a source of long-term suffering and severe visual impairment, early identification and adequate treatment are imperative. Multiple etiologies can underlie ADDE, and it is critical to identify the underlying cause to not only improve the ocular health but also to improve the overall quality of life and well-being of affected individuals. This review discusses the various etiologies of ADDE, highlights a pathophysiology-based approach for evaluating underlying contributors, outlines various diagnostic tests, and reviews treatment options. We present the current standards and discuss ongoing research in this field. Through this review, we propose a treatment algorithm that would be useful for an ophthalmologist in diagnosing and managing individuals with ADDE.
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Objective: Sjögren's syndrome (SS) is a chronic auto-immune inflammatory systemic disease, in which the infiltration of mo-nonuclear cells in the exocrine glands leads to physiological and morphological changes. This pilot case-control study aims to describe the profile, evaluate the oral condition, quality of life (QoL) and psychological condition, through complete clinical examination, OHIP-14 and DASS-21 questionnaires. Materials and Methods: The study was conducted with seven individuals with a final diagnosis of SS (case group [CG]), and seven individuals with symptoms of dry mouth (control group [GCO]), consulting at the institution from January to November 2021. participants were selected by free demand and those previously seen at the institution with a diagnosis of SS between 19 and 70 years of age. The questionnaire OHIP-14 was applied to assess the patient's quality of life, where seven dimensions are assessed, subdivided into 14 questions through the Lickert scale (0 to 4) assigned by the individual and which quantifies the impact of oral health on QoL. The questionnaire DASS-21 assessed the psychological condition of the patient, which presents seven questions for each emotional state (depression, anxiety, and stress), totaling 21 questions. The general clinical condition, evolution of SS, oral clinical condition, and the profile of this population were related to QoL factors and psychological conditions, using these assessment instruments. Results: There was no statistically significant difference between the groups regarding stimulated salivary flow. The only symptom with a statistically significant difference in the CG was difficulty in phonation (p< 0.001). The dimensions related to functional limitation and physical pain showed the most expressive results (p=0.004) (p=0.025), showing a strong negative impact on the QoL of the CG individuals, and the dimension related to disability was the least affected (p=0.684). The analysis of depression, anxiety, and stress did not show statistically significant results between the groups; however, in the CG, 5 (71.42%) individuals showed a severe degree of depression, anxiety, and stress. Conclusions: Individuals in the case group showed some changes, with a strong negative impact on QoL compared to the control group.
Objetivo: El síndrome de Sjögren (SS) es una enfermedad inflamatoria sistémica crónica autoinmune, en la que la infiltración de células mononucleares en las glándulas exocrinas provoca cambios fisiológicos y morfológicos. Este estudio piloto de casos y controles tiene como objetivo describir el perfil, evaluar la condición bucal, calidad de vida (CdV) y condición psicológica, mediante examen clínico completo, cuestionarios OHIP-14 y DASS-21. Materiales y Métodos: El estudio se realizó con 7 individuos con diagnóstico final de SS, grupo de casos (CG) y 7 individuos con síntomas de sequedad bucal, grupo control (GCO) atendidos en la institución de enero a noviembre de 2021. Los participantes fueron seleccionados por libre demanda y entre los atendidos previamente en la institución con diagnóstico de SS entre 19 y 70 años de edad. Para evaluar la calidad de vida del paciente se aplicó el cuestionario OHIP-14, donde se evalúan siete dimensiones, sub-divididas en 14 preguntas a través de la escala de Likert (0 a 4) asignada por el individuo y que cuantifica el impacto de la salud bucal en la calidad de vida. El cuestionario DASS-21 evaluó la condición psicológica del paciente, el cual presenta siete preguntas para cada estado emocional (depresión, ansiedad y estrés), totalizando 21 preguntas. El estado clínico general, la evolución del SS, el estado clínico bucal y el perfil de esta población se relacionaron con factores de calidad de vida y condiciones psicológicas, mediante estos instrumentos de evaluación. Resultados: En cuanto al flujo salival estimulado, no hubo diferencias estadísticamente significativas entre los grupos. El único síntoma que mostró diferencia estadísticamente significativa en el CG fue la dificultad en la fonación (p< 0,001). Las dimensiones relacionadas con limitación funcional y dolor físico mostraron los resultados más expresivos (p=0,004) (p=0,025), mostrando un fuerte impacto negativo en la CdV de los individuos del GC, y la dimensión relacionada con discapacidad fue la menos afectada (p=0,684). El análisis de depresión, ansiedad y estrés no mostró resultados estadísticamente significativos entre los grupos; sin embargo, en el GC, 5 (71,42%) individuos presentaron un grado severo de depresión, ansiedad y estrés. Conclusión: Se puede concluir que los individuos del grupo de casos mostraron algunos cambios, con un fuerte impacto negativo en la calidad de vida en comparación con el grupo de control.
Subject(s)
Humans , Male , Female , Adult , Middle Aged , Aged , Young Adult , Anxiety/epidemiology , Quality of Life/psychology , Sjogren's Syndrome/epidemiology , Depression/epidemiology , Sjogren's Syndrome/complications , Case-Control StudiesABSTRACT
Neurological manifestations such as polyneuropathy are reported in 8-49% of cases with Sjögren's Syndrome (SjS), but central nervous system involvement is seldom described. We report a 46-year-old woman with a history of SjS with distal renal tubular acidosis and autoimmune thyroiditis. She consulted in the emergency room for a five-days history of holocranial headache and explosive vomiting. Fundoscopy showed bilateral papilledema. Brain computed tomography (CT) without contrast showed diffuse encephalic edema, with effacement ofsulci and restriction ofperitruncal cisterns. Brain AngioCT ruled out thrombosis, and brain magnetic resonance (MRI) was without structural alterations or hydrocephalus. Lumbar puncture had increased cerebrospinal fluid output pressure but without cytochemical alterations, and negative gram, cultures and filmarray. The diagnosis of Intracranial Hypertension Syndrome (ICHTS) ofprobable autoimmune etiology in the context of SjS was proposed, and management with high-dose corticosteroids was initiated with favorable clinical and imaging response.
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Objective:Primary biliary cholangitis (PBC) and Primary Sj?gren′s syndrome (pSS) are autoimmune epithelial inflammatory diseases that share many common clinical symptoms. The aim of this study was to investigate the differences and diagnostic value of Autotaxin (ATX) in PBC and SS.Methods:The clinical data of 237 cases diagnosed with PBC, PBC secondary to SS, pSS and healthy individuals(HC) between September 2020 and September 2021 were retrospectively analyzed. The levels of ATX in each group were measured by enzyme-linked immunosorbent assay (ELISA), and the corresponding sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and area under the curve ( AUC), etc were analyzed. Normally distributed data were expressed as mean ±SD and non-normally distributed as median (IQR). The differences and correlations between ATX and the biochemical tests in each group were assessed by applying the Mann-Whitney U test, Spearman correlation analysis, etc. P<0.05 was considered statistically significant difference. Results:The results showed that ATX was positive in 33.9%, 33.3% and 53.3% for PBC, PBC secondary SS, and pSS, respectively, with the specificities of 93.1%, 100% and 93.2%, respectively. The highest accuracy was achieved in pSS and the sensitivity and specificity were 86.5% and 93.2%, which were higher than those in PBC group(56.8%, 93.1%), respectively. Compared with HC [32.6(21.8, 60.5)ng/ml], ATX levels in PBC[59.3(48.6, 86.3)ng/ml, U=1 750.50, P<0.001], PBC-SS [73.6 (53.3,102.4)ng/ml; U=199.00, P<0.001], and pSS [152.6 (97.4,192.1)ng/ml, U=264.00, P<0.001] were elevated with significant difference ( P<0.05). ATX levels showed a decreasing trend from the pSS group to the HC group. ATX in PBC group[AUC(95% CI)= 0.73(0.651,0.812), P<0.001], PBC secondary SS group [AUC(95% CI)=0.82(0.730, 0.912), P<0.001], and pSS group [AUC(95% CI)=0.94(0.898, 0.984), P<0.001] had prediction accuracy. ATX was associated with total protein ( r=-0.31, P=0.041) level and glutaminase (r=-0.26, P=0.024) level. Conclusion:ATX has diagnostic value in both PBC and SS, and with higher sensitivity and specificity for the latter.
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Metabolomics is an emerging branch of system biology, which comprehensively and systematically analyzes the changes of metabolite levels in the human body after being affected by pathophysiological and other factors. In recent years, metabolomics has been widely used in the study of clinical diseases. Primary Sj?gren′s syndrome (pSS) is a chronic systemic autoimmune disease that can affect multiple organs and systems. The pathogenesis of pSS has not been fully clarified, and early diagnosis and treatment are still difficult, and the research on its pathogenesis is still a challenge at present. Metabolomics provides new avenues for disease research, and there are more and more metabolomics studies of pSS. This review mainly introduces metabolomics and metabolomics research progress of pSS.
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Objective:To explore the correlation between Traditional Chinese Medicine (TCM) syndrome types of Sj?gren syndrome (SS) and blood test parameters, immunological function and disease activity.Methods:A retrospective cross-sectional study was conducted. The clinical data of 242 SS inpatients in the Rheumatology and Immunology Department of Jiangsu Province Hospital of TCM from February 2021 to June 2022 were analyzed retrospectively. We compared the general data (gender, age, course of disease, BMI), blood parameters [WBC, hemoglobin (Hb), PLT, neutrophil count (NEUT), lymphocyte count(LYMPH), neutrophil/lymphocyte ratio (NLR)], immunological indicators (globulin, IgG, IgA, IgM, rheumatoid factor (RF), anti-SSA antibody, anti-SSB antibody, anti-Ro-52 antibody) .The distribution difference of disease activity [Disease Activity Index of Sjogren's syndrome (ESSDAI) and Patient Report Index of Sjogren's syndrome (ESSPRI)], the correlation between each syndrome type and blood routine parameters, immunological indicators and inflammatory indicators was analyzed by binary logistic regression.Results:They were divided into 82 cases of qi yin deficiency syndrome, 61 cases of yin deficiency and fluid deficiency syndrome, 59 cases of yin deficiency and blood stasis syndrome, 32 cases of yin deficiency and heat toxin syndrome, and 8 cases of other syndrome types. Because the number of other syndrome types was small, they were not included in this study. Logistic regression analysis showed that the positive rate of anti SSA antibody was negatively correlated with IgM [ OR (95% CI)=0.570 (0.407, 0.798)] ( P<0.01). The positive rates of anti SSB antibody and anti Ro-52 antibody were negatively correlated with LYMPH [ OR (95% CI)=0.445 (0.223, 0.886), 0.457 (0.224, 0.932), respectively] ( P<0.05). The positive rates of anti SSB antibody and anti Ro-52 antibody were positively correlated with IgG [ OR (95% CI)=1.171 (1.034, 1.325), 1.159 (1.014, 1.325), respectively] ( P<0.05). Qi Yin deficiency syndrome was positively correlated with WBC [ OR (95% CI)=2.590 (1.120, 5.987)] ( P<0.05), and negatively correlated with LYMPH [ OR (95% CI)=0.090 (0.017, 0.470)] and IgA [ OR (95% CI)=0.728 (0.553, 0.959)] ( P<0.05). Yin deficiency and fluid deficiency syndrome were negatively correlated with PLT [ OR (95% CI)=0.991 (0.984, 0.998)], ESSPRI [ OR (95% CI)=0.705 (0.506, 0.983)], ESSDAI [ OR (95% CI)=0.716 (0.534, 0.960)] ( P<0.05). Yin deficiency and blood stasis syndrome was positively correlated with IgA [ OR (95% CI)=1.184 (1.028, 1.363)] ( P<0.05), and negatively correlated with anti SSB antibody positive rate [ OR (95% CI)=0.247 (0.093, 0.659)] ( P<0.05). Yin deficiency heat toxin syndrome was positively correlated with IgA [ OR (95% CI)=1.368 (1.037, 1.803)] ( P<0.05), and negatively correlated with anti SSB antibody positive rate [ OR (95% CI)=0.278 (0.085, 0.909)] ( P<0.05). Conclusion:The level of immunoglobulin, inflammatory index and disease activity of yin deficiency and blood stasis syndrome and yin deficiency and heat toxin syndrome are high, and blood system damage and exocrine gland disease are easy to occur, which can provide clinical basis for the combination of disease and syndrome differentiation and treatment of SS.
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ObjectiveTo investigate the effect of different doses of Jiedu Tongluo Shengjin prescription （JTSP） on serum interleukin-6 （IL-6）， tumor necrosis factor-α （TNF-α） and forkhead box P3 （FoxP3） in submandibular gland of NOD/Ltj mice with Sjögren's syndrome， and to explore the mechanism of JTSP on immune regulation in NOD/Ltj mice. MethodThirty NOD/Ltj mice （eight weeks old） were randomly divided into model group， JTSP low-dose group， JTSP medium-dose group， JTSP high-dose group and hydroxychloroquine group， and were administrated with normal saline， JTSP 9， 18， and 36 g·kg-1， and hydroxychloroquine 60 mg·kg-1 daily， respectively from the age of 12 weeks. Six ICR mice were given an equal amount of normal saline by gavage as the control group. During the experiment， daily water consumption and saliva secretion of mice at the age of 9， 12， 16 weeks were recorded. After 4 weeks of administration， submandibular gland and spleen tissues were dissected to calculate corresponding indexes. The pathological morphology of submandibular gland was observed by hematoxylin-eosin （HE） staining. Meso Scale Discovery （MSD） and immunohistochemistry were employed to detect the serum levels of IL-6， TNF-α and IL-10， and the expression and distribution of FoxP3 in submandibular gland， respectively. The protein expression of FoxP3 in mouse submandibular gland was determined by Western blot， and the mRNA expressions of FoxP3 and TNF-α were determined by real-time polymerase chain reaction （Real-time PCR）. ResultCompared with the control group， the model group presented increased daily water consumption， decreased saliva secretion， lowered submandibular gland index， elevated pathological score of submandibular gland， up-regulated serum IL-6 and TNF-α and mRNA expression of TNF-α while down-regulated serum IL-10 and protein and mRNA expressions of FoxP3 in submandibular gland （P<0.05）. Compared with the conditions in model group， daily water consumption in JTSP groups was reduced while saliva secretion was increased， especially in medium-dose and high-dose groups （P<0.05）， and there was an increase in the submandibular gland index of JTSP medium-dose group （P<0.05） while a decrease in the spleen index of JTSP high-dose group （P<0.05）. Additionally， JTSP groups had lower pathological score of submandibular gland than the model group （P<0.05）， especially high-dose group， as well as lower serum IL-6 and TNF-α and mRNA expression of TNF-α while higher serum IL-10 （P<0.05）. JTSP at medium and high doses up-regulated the protein and mRNA expressions of FoxP3 in submandibular gland （P<0.05）. ConclusionJTSP may inhibit the secretion of inflammatory cytokines by regulating the stability of FoxP3+ regulatory T （Treg） cells， thus alleviating the systemic immune inflammation in Sjögren's syndrome.
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Sjögren's syndrome （SS）， a disorder of immune system， is one of the dominant diseases treated by traditional Chinese medicine （TCM）. China Association of Chinese Medicine organized experts in the field of TCM and western medicine rheumatology and pharmacology to discuss the advantages and optimal regimens of TCM for the treatment of SS. The experts generally agreed on the low early diagnosis rate of SS and the lack of targeted therapeutic drugs. In addition， autoimmune abnormality is the key factor in the occurrence of SS and deficiency of both Qi and Yin is the core pathogenesis. SS has unique tongue manifestations， which is expected to allow for the early diagnosis and treatment with integrated traditional Chinese and western medicine. TCM has advantages in treating SS in terms of alleviating clinical symptoms and systemic involvement， individualized treatment， relieving sleep and mood disorders， preventing the occurrence in the early stage， and enhancing the effectiveness and reducing toxicity in the treatment by integrated TCM and western medicine. In general， TCM has advantages in different stages of SS. Internal and external use of TCM， acupuncture， and acupotome are all available options. The optimal regimens should be determined on the basis of pattern identification， stage of disease， and the advantages of TCM. Clinical characteristics and biomarkers of SS should be studied to classify patients， so as to design precision evidence-based TCM regimens for SS. On the basis of unique tongue manifestations of SS， models for early diagnosis and poor prognosis identification of SS should also be established to achieve early prevention and treatment and to improve the prognosis. In the future， we should vigorously carry out high-quality evidence-based medical research on the treatment of SS by TCM and integrated traditional Chinese and western medicine and develop relevant guidelines to optimize and standardize current diagnosis and treatment， thereby laying a basis for clarifying and explaining the advantages of TCM in treating SS.