Operational characteristics of ultrasound in the diagnosis of Sjögren's syndrome.

BACKGROUND AND OBJECTIVE: To determine the operational characteristics of salivary gland ultrasound (SGU) in the diagnosis of Sjögren's syndrome (SS) in a population of colombian patients with dry symptoms. MATERIALS AND METHODS: Study of diagnostic tests in patients with dry symptoms who consecutively attended the rheumatology consultation (2018-2020). Sociodemographic and clinical data were obtained through a survey, paraclinical and ophthalmological tests, minor salivary gland biopsy, unstimulated salivary flow and SGU (score 0-6 based on De Vita) were done. Sensitivity, specificity, positive (PPV) and negative (NPV) predictive values (Stata 15®) were calculated. The receiver operating characteristics (ROC) curve was developed. RESULTS: 102 patients were included (34 SS and 68 non-SS), mean age 55.69 (±11.93) years, 94% women. Positive ultrasound (score of 2 or more) was more frequent in the SS group, (70.6% vs. 22.1%, P<0.0001). The sensitivity was the same for grade 2 and 3 (70.59%), with a higher specificity (89.71%) for grade 3 (PPV 77.42% NPV 85.92). The ROC curve from the sum of the glands by means of ultrasound was better than those of the independent glands. The ROC curve of the ultrasound presented a greater area under the curve (0.72 [0.61-0.82]) than that of the histological analysis (focus score) (0.68 [0.59-0.78]), P=0.0252. CONCLUSION: Salivary gland ultrasound is a useful and reliable method for the classification of SS. Its use could be considered in the future within the SS classification criteria.

Prevalence of neonatal lupus in a small group of antibody-carrying mothers and frequency of complete atrioventricular block.

BACKGROUND: Neonatal lupus (NL) is extremely rare and is caused by the transplacental passage of maternal IgG autoantibodies against Ro, La, and/or RNP proteins into the fetal circulation, which can cause congenital complete atrioventricular block (CCAB), permanent skin lesions, and liver involvement. OBJECTIVE: To know the prevalence of NL in patients with CCAB and the clinical course in long-term follow-up. METHODS: From January 1992 to December 2017, patients with CCAB were included. The presence of anti-SSA/Ro and anti-SSB/La antinuclear antibodies in maternal serum confirmed NL. RESULTS: Eight patients were included with a follow-up of 10 ± 6 years; NL was concluded in 62.5%; two were male. One of them was diagnosed in utero, two at birth, and a pacemaker was implanted in them, one at 12 years of age and another at 15. The other two cases were diagnosed at 18 and 26 years of age, and permanent pacemakers were implanted 8 and 5 years later, respectively. In one case, a definitive pacemaker was not implanted in a newborn with only 1 year of follow-up. At delivery, 60% of the mothers were free of rheumatic disease, and altogether, they all had 19 children; none of them presented NL manifestations. CONCLUSIONS: CCAB is rare and frequently associated with a maternal autoimmune disease, practically all of them will require a definitive pacemaker at some point in their lives.

ANTECEDENTES: El lupus neonatal (LN) es extremadamente raro y es ocasionado por el paso transplacentario de auto-anticuerpos maternos IgG contra las proteínas Ro, La y/o RNP a la circulación fetal que puede ocasionar bloqueo aurículo-ventricular completo congénito (BAVCC) permanente, lesiones dérmicas y afectación hepática. OBJETIVO: Conocer la prevalencia de LN en paciente con BAVCC y la evolución clínica en un seguimiento a largo plazo. MÉTODOS: De enero de 1992 a diciembre 2017 se incluyeron paciente con BAVCC. La presencia de anticuerpos antinucleares anti-SSA/Ro y anti-SSB/La en suero materno confirmó LN. RESULTADOS: Ocho pacientes fueron incluidos con seguimiento de 10 ± 6 años, el 62.5 % con LN; dos fueron del sexo masculino. Uno diagnosticado in útero, dos al nacimiento, en ellos se implantó marcapaso; uno a los 12 años de edad y otro a los 15. Los otros dos casos fueron diagnosticados a los 18 y 26 años, se implantó marcapaso definitivo en ellos 8 y 5 años después respectivamente. En un caso no se implantó marcapaso definitivo; un recién nacido con solo un año de seguimiento. Al dar a luz, el 60 % de las madres estaban libres de enfermedad reumática y en conjunto todas tuvieron 19 hijos, ninguno de ellos presentó manifestaciones de LN. CONCLUSIONES: El BAVCC es raro y frecuentemente está asociado a una enfermedad autoinmune materna, prácticamente todos requerirán de marcapaso definitivo en alguna época de su vida.

Efficacy of disease-modifying antirheumatic drugs in primary Sjögren's syndrome-related interstitial lung disease.

OBJECTIVES: To evaluate the treatment modalities and their effects in primary Sjögren's syndrome (pSS) patients with interstitial lung disease (ILD). METHODS: In this chart review study, patients diagnosed with pSS-related ILD (pSS-ILD) between January 2004 and August 2022 were screened. Glucocorticoid use and administered disease-modifying antirheumatic drugs (DMARDs) were determined. The difference between forced vital capacity (FVC) and diffusion capacity of the lungs for carbon monoxide (DLCO) before and after treatment was evaluated. RESULTS: ILD was present in 44 of 609 patients (7.2%) diagnosed with pSS. In 27 patients included in the study, steroid usage was 81.5%. There was a statistically insignificant increase in FVC% (from 80.20±22.1 to 81.6±23.0) and a decrease in DLCO% (53.7±15.3-52.2±19.3) with DMARD treatment (p=0.434 and p=0.652, respectively). There was no significant difference between the treatment groups (azathioprine [AZA], mycophenolate mofetil [MMF], and rituximab [RTX]) in terms of the change in FVC% and DLCO% compared with baseline levels. The effect of treatment on FVC and DLCO was similar in UIP and NSIP patterns. CONCLUSIONS: AZA, MMF, and RTX have similar effects on pulmonary functions in pSS-ILD and provide disease stabilization.

[Translated article] Physicochemical and microbiological stability study of two new preservative-free methylprednisolone eye drops.

OBJECTIVE: To study the physicochemical and microbiological stability over 90 days of two preservative-free methylprednisolone sodium succinate (MTPSS) 1 and 10 mg/mL eye drops for use in ocular pathologies such as Sjögren's syndrome and dry eye syndrome. METHOD: The two eye drops were prepared from injectable MTPSS (Solu-moderin® and Urbason®), water for injection and normal saline solution. In accordance with ICH (International Council for Harmonization of Technical Requirements for Pharmaceuticals for Human Use) guidelines, they were then stored in triplicate under refrigerated conditions (5±3 °C), at room temperature (25±2 °C), and at 40 °C (±2 °C). In accordance with the USP (United States Pharmacopeia), physicochemical controls of the active ingredient content were carried out by HPLC-UV (High Performance Liquid Chromatography with Ultraviolet detection), together with controls of pH, osmolality, and visual examination. Microbiological sterility was also tested under refrigerated conditions up to 30 days in open containers and up to 90 days in closed ones. RESULTS: The eye drops stored at 5 °C were the most stable; in the 1 mg/mL eye drops, degradation of the drug fell below 90% from day 21, and in the 10 mg/mL eye drops, from day 42. pH change did not vary by ≥1 unit in formulations stored at 5 °C, unlike the other formulations. Changes in osmolality did not exceed 5% on day 90 in any storage conditions. Samples of non refrigerate eye drops at 10 mg/mL, presented a white precipitate from day 14 and 28, respectively. Non-refrigerated 1 mg/mL eye drops presented suspended particles on day 90. There were no color changes. Microbiological analysis showed that sterility was maintained for over 90 days in the closed containers, although microbial contamination was detected from day 21 in the open containers. CONCLUSIONS: 1 mg/mL MTPSS eye drops show physicochemical and microbiological stability for 21 days under refrigeration, compared to 42 days for 10 mg/mL eye drops stored under the same conditions. However, since they do not include preservatives in their composition, they should not be used for more than 7 days after opening.

Physicochemical and microbiological stability study of two new preservative-free methylprednisolone eye drops. / Estudio de estabilidad fisicoquímica y microbiológica de dos nuevos colirios de metilprednisolona sin conservantes.

OBJECTIVE: To study the physicochemical and microbiological stability over 90 days of two preservative-free methylprednisolone sodium succinate (MTPSS) 1 mg/ml and 10 mg/ml eye drops for use in ocular pathologies such as Sjögren's syndrome and dry eye syndrome. METHOD: The two eye drops were prepared from injectable MTPSS (Solu-moderin® and Urbason®), water for injection and normal saline solution. In accordance with ICH (International Council for Harmonisation of Technical Requirements for Pharmaceuticals for Human Use) guidelines, they were then stored in triplicate under refrigerated conditions (5 ±3 °C), at room temperature (25 ± 2 °C), and at 40 °C (±2 °C). In accordance with the USP (United States Pharmacopeia), physicochemical controls of the active ingredient content were carried out by HPLC-UV (High Performance Liquid Chromatography with Ultraviolet detection), together with controls of pH, osmolality, and visual examination. Microbiological sterility was also tested under refrigerated conditions up to 30 days in open containers and up to 90 days in closed ones. RESULTS: The eye drops stored at 5 °C were the most stable; in the 1 mg/ml eye drops, degradation of the drug fell below 90% from day 21, and in the 10 mg/ml eye drops, from day 42. pH change did not vary by ≥1 unit in formulations stored at 5 °C, unlike the other formulations. Changes in osmolality did not exceed 5% on day 90 in any storage conditions. Samples of non refrigerate eye drops at 10 mg/ml, presented a white precipitate from day 14 and 28 respectively. Non-refrigerated 1 mg/ml eye drops presented suspended particles on day 90. There were no color changes. Microbiological analysis showed that sterility was maintained for over 90 days in the closed containers, although microbial contamination was detected from day 21 in the open containers. CONCLUSIONS: 1 mg/ml MTPSS eye drops show physicochemical and microbiological stability for 21 days under refrigeration, compared to 42 days for 10 mg/ml eye drops stored under the same conditions. However, since they do not include preservatives in their composition, they should not be used for more than 7 days after opening.

Ecografía de glándula salival en el Síndrome de Sjögren primario

Introducción: El Síndrome de Sjögren primario es una enfermedad autoinmune que se caracteriza por un proceso inflamatorio que afecta fundamentalmente a las glándulas exocrinas. Existe un interés creciente en el uso de la ecografía de glándulas salivales como una herramienta no invasiva para el diagnóstico del Síndrome de Sjögren primario (SSp). Objetivo: Describir los hallazgos ecográficos de glándulas salivales y de biopsia glandular en pacientes con sospecha de Síndrome de Sjögren primario. Materiales y métodos: Se realizó un estudio observacional de corte transversal en el que se incluyó a pacientes adultos con sospecha de SSp. En todos los casos se realizó ecografía y biopsia de glándulas salivales. Se registraron variables epidemiológicas, clínicas, de imagen (i.e. ultrasonido) y el resultado anatomopatológico de las biopsias de glándulas salivales. Se realizó un análisis descriptivo de las variables. Resultados: Se incluyeron a 23 pacientes con sospecha de SSp. La edad promedio de los pacientes fue 47,7±11,1 años, y el 96,0% fueron de sexo femenino. En todas las ecografías se constataron hallazgos compatibles con SSp, mientras que en el 96% de las biopsitas se objetivaron datos compatibles con SSp. Conclusión: En esta serie de pacientes, se pudo constatar un alto porcentaje de casos en los que se objetivó cambios compatibles con SSp, tanto en la ecografía de glándulas salivales como en la anatomía patológica.

Introduction : Primary Sjögren's Syndrome is anautoimmune disease that is characterized by an inflammatory process that primarily affects the exocrine glands. There is growing interest in the use of salivary gland ultrasound as a non-invasive tool for the diagnosis of primary Sjögren's Syndrome (pSS). Objective: To describe the ultrasound findings of salivary glands and glandular biopsy in patients with suspected primary Sjögren's Syndrome. Materials and methods: A cross-sectional observational study including adult patients with suspected pSS . In all cases, ultrasound and salivary gland biopsy were performed. Epidemiological, clinical, imaging (i.e. ultrasound) variables as well as pathology results of salivary gland biopsies were recorded. A descriptive analysis of the variables was carried out. Results: 23 patients with suspected pSS were included. The average age of the patients was 47.7±11.1 years, and 96.0% were female. All ultrasounds showed findings compatible with pSS, while 96% of the biopsies reported results compatible with pSS. Conclusion: In this group of patients, a high percentage of cases showed data compatible with pSS, both in the ultrasound and biopsy of the salivary glands.

Sjögren: unique surname, two men, four syndromes and one disease / Sjögren: sobrenome único, dois homens, quatro síndromes e uma doença

Abstract Henrik and Torsten Sjögren (/'ogrƏn/ or SHOH-grƏn) were two Swedish physicians living in the same period, but completely unrelated, except for their notable contributions to Medicine. The first one described keratoconjunctivitis sicca, afterward called Sjögren's syndrome, and a fishing net aspect retinal pigmentation affecting visual acuity, nowadays known as Sjögren reticular dystrophy. The last one contributed to the understanding of Spielmeyer-Sjögren disease, Marinesco-Sjögren, and Sjögren-Larsson syndromes, all related to genetic disorders and neurological symptoms. In this paper, we aim to describe each disorder, in order to avoid any misunderstanding in diagnosis and for historical record.

Resumo Henrik e Torsten Sjögren (/'ogrƏn/ or SHOH-grƏn) foram dois médicos suecos que viveram na mesma época, mas não tinham nenhuma relação entre si, exceto por suas notáveis contribuições à medicina. O primeiro descreveu a ceratoconjuntivite sicca, posteriormente chamada de síndrome de Sjögren, e uma pigmentação da retina com aspecto de rede de pesca que afeta a acuidade visual, hoje conhecida como distrofia reticular de Sjögren. O último contribuiu para a compreensão da doença de Spielmeyer-Sjögren, das síndromes de Marinesco-Sjögren e Sjögren-Larsson, todas relacionadas a distúrbios genéticos e sintomas neurológicos. Neste artigo, pretendemos descrever cada desordem, a fim de evitar qualquer mal-entendido no diagnóstico e para registro histórico.

Prevalence of neonatal lupus in a small group of antibody-carrying mothers and frequency of complete atrioventricular block / Prevalencia de lupus neonatal en un pequeño grupo de madres portadoras de anticuerpos y frecuencia de bloqueo auriculoventricular completo

Abstract Background: Neonatal lupus (NL) is extremely rare and is caused by the transplacental passage of maternal IgG autoantibodies against Ro, La, and/or RNP proteins into the fetal circulation, which can cause congenital complete atrioventricular block (CCAB), permanent skin lesions, and liver involvement. Objective: To know the prevalence of NL in patients with CCAB and the clinical course in long-term follow-up. Methods: From January 1992 to December 2017, patients with CCAB were included. The presence of anti-SSA/Ro and anti-SSB/La antinuclear antibodies in maternal serum confirmed NL. Results: Eight patients were included with a follow-up of 10 ± 6 years; NL was concluded in 62.5%; two were male. One of them was diagnosed in utero, two at birth, and a pacemaker was implanted in them, one at 12 years of age and another at 15. The other two cases were diagnosed at 18 and 26 years of age, and permanent pacemakers were implanted 8 and 5 years later, respectively. In one case, a definitive pacemaker was not implanted in a newborn with only 1 year of follow-up. At delivery, 60% of the mothers were free of rheumatic disease, and altogether, they all had 19 children; none of them presented NL manifestations. Conclusions: CCAB is rare and frequently associated with a maternal autoimmune disease, practically all of them will require a definitive pacemaker at some point in their lives.

Resumen Antecedentes: El lupus neonatal (LN) es extremadamente raro y es ocasionado por el paso transplacentario de auto-anticuerpos maternos IgG contra las proteínas Ro, La y/o RNP a la circulación fetal que puede ocasionar bloqueo aurículo-ventricular completo congénito (BAVCC) permanente, lesiones dérmicas y afectación hepática. Objetivo: Conocer la prevalencia de LN en paciente con BAVCC y la evolución clínica en un seguimiento a largo plazo. Métodos: De enero de 1992 a diciembre 2017 se incluyeron paciente con BAVCC. La presencia de anticuerpos antinucleares anti-SSA/Ro y anti-SSB/La en suero materno confirmó LN. Resultados: Ocho pacientes fueron incluidos con seguimiento de 10 ± 6 años, el 62.5 % con LN; dos fueron del sexo masculino. Uno diagnosticado in útero, dos al nacimiento, en ellos se implantó marcapaso; uno a los 12 años de edad y otro a los 15. Los otros dos casos fueron diagnosticados a los 18 y 26 años, se implantó marcapaso definitivo en ellos 8 y 5 años después respectivamente. En un caso no se implantó marcapaso definitivo; un recién nacido con solo un año de seguimiento. Al dar a luz, el 60 % de las madres estaban libres de enfermedad reumática y en conjunto todas tuvieron 19 hijos, ninguno de ellos presentó manifestaciones de LN. Conclusiones: El BAVCC es raro y frecuentemente está asociado a una enfermedad autoinmune materna, prácticamente todos requerirán de marcapaso definitivo en alguna época de su vida.

Autologous serum eye drops diluted with 0.5% methylcellulose and 0.9% saline solution: a clinical comparative study / Colírio de soro autólogo diluído com metilcelulose 0,5% e solução salina 0,9%: um estudo clínico comparativo

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.

Síndrome de Sjögren: identificación de nuevos biomarcadores y mecanismos moleculares implicados en su fisiopatogénesis

Introducción: El Síndrome de Sjögren (SS) es una enfermedad autoinmune de carácter sistémico, que afecta principalmente al sistema glandular exocrino, generando un funcionamiento anormal de las glándulas lacrimales y salivales. Objetivo: proporcionar una actualización sobre la identificación de nuevos biomarcadores y mecanismos moleculares implicados en la fisiopatogénesis del SS. Método: Revisión narrativa de la literatura en diferentes bases de datos, mediante la búsqueda de términos descritos incluidos en los tesauros MESH y DeCs, para artículos publicados a partir del año 2018. Resultados: presentamos evidencia que destaca la identificación de nuevos biomarcadores y mecanismos implicados en la fisiopatogénesis del SS, describiendo las vías de: linfocitos B, catepsina S, cistatina C, quimioquina C-X3-C modificada de ligando 1, quimiocina regulada por activación del timo, células T, proteína morfogenética ósea 6, estimulación del receptor de oxitocina, receptor de zinc, calponina-3. Conclusión: los avances en la tecnología facilita el análisis detallado de la genética y fisiopatogénesis del SS, impulsando el desarrollo de terapias específicas. La búsqueda de biomarcadores no invasivos responde a las limitaciones de los métodos existentes y la invasividad de las biopsias salivales, prometiendo mejoras diagnósticas y terapéuticas.

Introduction: Sjögren's Syndrome (SS) is a systemic autoimmune disease that primarily affects the exocrine glandular system, leading to abnormal lacrimal and salivary gland function. Objective: To provide an update on identifying new biomarkers and molecular mechanisms involved in the pathogenesis of SS. Method: Narrative review of the literature in various databases, searching for terms included in the MESH and DeCs thesauri, for articles published since 2018. Results: We present evidence highlighting the identification of new biomarkers and mechanisms involved in the pathogenesis of SS, describing pathways of B lymphocytes, cathepsin S, cystatin C, modified C-X3-C chemokine ligand 1, thymus activation-regulated chemokine, T cells, bone morphogenetic protein 6, oxytocin receptor stimulation, zinc receptor, and calponin-3. Conclusion: Advances in technology facilitate detailed analysis of the genetics and pathogenesis of SS, driving the development of specific therapies. The search for non-invasive biomarkers is driven by the limitations of existing methods and the invasiveness of salivary gland biopsies, promising diagnostic and therapeutic improvements.

20% Autologous serum vs. 0.05% cyclosporine and preservative-free artificial tears in the treatment of Sjögren related dry eye / Soro autólogo a 20% versus ciclosporina a 0,05 e lubrificantes oculares sem conservantes no tratamento da síndrome do olho seco relacionada à Sjögren

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.

Validación del ClinESSDAI para Argentina / Validation of ClinESSDAI for Argentina

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.

Sjogren-Larsson syndrome brain volumetric reductions demonstrated with an automated software / Reduções volumétricas cerebrais da síndrome de Sjogren-Larsson demonstradas com um software automatizado

Abstract Background Sjogren-Larsson syndrome (SLS) is a neurocutaneous disease with an autosomal recessive inheritance, caused by mutations in the gene that encodes fatty aldehyde dehydrogenase (ALDH3A2), clinically characterized by ichthyosis, spastic diplegia, and cognitive impairment. Brain imaging plays an essential role in the diagnosis, demonstrating a nonspecific leukoencephalopathy. Data regarding brain atrophy and grey matter involvement is scarce and discordant. Objective We performed a volumetric analysis of the brain of two siblings with SLS with the aim of detecting deep grey matter nuclei, cerebellar grey matter, and brainstem volume reduction in these patients. Methods Volume data obtained from the brain magnetic resonance imaging (MRI) of the two patients using an automated segmentation software (Freesurfer) was compared with the volumes of a healthy control group. Results Statistically significant volume reduction was found in the cerebellum cortex, the brainstem, the thalamus, and the pallidum nuclei. Conclusion Volume reduction in grey matter leads to the hypothesis that SLS is not a pure leukoencephalopathy. Grey matter structures affected in the present study suggest a dysfunction more prominent in the thalamic motor pathways.

Resumo Antecedentes A Síndrome de Sjogren-Larsson (SSL) é uma doença neurocutânea de herança autossômica recessiva, causada por mutações no gene que codifica a aldeído graxo desidrogenase (ALDH3A2), caracterizada clinicamente por ictiose, diplegia espástica e comprometimento cognitivo. A imagiologia cerebral desempenha um papel essencial no diagnóstico, demonstrando uma leucoencefalopatia inespecífica. Dados sobre atrofia cerebral e envolvimento da substância cinzenta são escassos e discordantes. Objetivo Realizamos uma análise volumétrica do cérebro de dois irmãos com SLS com o objetivo de detectar núcleos profundos de substância cinzenta, substância cerebral cinzenta e redução do volume do tronco encefálico nestes pacientes. Métodos Os dados de volume obtidos da ressonância magnética (RM) cerebral dos dois pacientes usando um software de segmentação automática (Freesurfer) foram comparados com os volumes de um grupo controle saudável. Resultados Redução de volume estatisticamente significativa foi encontrada no córtex do cerebelo, no tronco cerebral, no tálamo e nos núcleos pálidos. Conclusão A redução do volume da substância cinzenta leva à hipótese de que a SSL não é uma leucoencefalopatia pura. As estruturas da substância cinzenta afetadas no presente estudo sugerem uma disfunção mais proeminente nas vias motoras talâmicas.

Sjogren's syndrome: a neurological perspective / Síndrome de Sjogren: uma perspectiva neurológica

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.

Encefalitis autoinmune seronegativa asociada a síndrome de Sjögren primario / Autoimmune seronegative encephalitis associated to primary Sjögren's disease

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.

Gastrointestinal and Hepatic Involvement in Primary Sjögren's Syndrome: Case Report and Literature Review

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.

Síndrome de Sjögren y endodoncia, consideraciones a propósito de un caso / Sjögren's syndrome and endodontics, clinical case and considerations

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.

Assessment of depression, stress, anxiety and oral health-related quality of life in individuals with sjögren's syndrome: case control pilot study / Evaluación de la depresión, el estrés, la ansiedad y la calidad de vida relacionada con la salud bucal en personas con síndrome de Sjögren: estudio piloto de casos y controles

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.

Características Clínicas y Epidemiológicas en Pacientes Diagnosticados con Síndrome de Sjögren en un Hospital Universitario de Cuarto Nivel

El síndrome de Sjögren es la segunda enfermedad autoinmune crónica más prevalente; factores hereditarios y medioambientales interactúan provocando una "epitelitis autoinmune" que daña el tejido glandular exocrino principalmente, pero también a otros órganos; debido a esta heterogeneidad clínica, el diagnóstico y tratamiento constituyen un desafío. Estudios demuestran que afecta al 0,1-0,6 % de la población, con predilección por el sexo femenino (9:1). En Colombia existen pocos estudios epidemiológicos descriptivos y de caracterización en este síndrome. Caracterizar los parámetros clínicos y epidemiológicos de pacientes con síndrome de Sjögren. Se realizó un estudio observacional descriptivo de corte transversal, se revisaron historias clínicas de 409 pacientes con el síndrome que asistieron al Hospital Universitario San Ignacio entre 2012 y 2019. Los datos se analizaron usando estadística descriptiva. La edad promedio de diagnóstico fue 59 años, la relación mujer: hombre 9:1, (91,7 % y 8,3 % respectivamente). Hubo mayor prevalencia en pacientes de 51 a 60 años (131) seguido por los de 61-70 años (114). La manifestación clínica más común fue la xerostomía (90 %); la biopsia de glándula salival menor fue el examen complementario más solicitado (65 %). El compromiso óseo-articular, fue la enfermedad concomitante más asociada. La etiología de la enfermedad es poco conocida, sin embargo, su caracterización, así como la identificación de medios diagnósticos y terapéuticos ayuda en la comprensión de la clínica; dicha información permitirá el desarrollo de tratamientos cada vez más efectivos y específicos.

Sjögren syndrome is the second most common chronic autoimmune disease, environmental and hereditary mechanisms causes an autoimmune epithelitis that affect the exocrine glandular tissue mainly, but also other organs; because this broad spectrum of clinical characteristics, the diagnostic and treatment become a challenge. Studies has shown that 0.1-0.6 % of the population is affected, with predominance of females over males (9:1). In Colombia there are few descriptive epidemiological studies and characterization of the affected population. Clinical and epidemiological characterization of Sjögren syndrome patients. A descriptive cross-sectional observational study was conducted, 409 clinical histories of patients with the syndrome at San Ignacio University Hospital from 2012 to 2019 were analized. The data collected were subjected to a descriptive statistic. The average age of diagnosis was 59 years, the ratio female: male 9:1 (91.7 % and 8.3 % respectively), the highest frequency of the syndrome was found in the 51 to 60 years old group (131) followed by the 61 to 70 years old group (114). The most common clinical manifestation was xerostomia (90 %), minor salivary gland biopsy was the most frequent test requested (65 %). The osseoarticular involvement was the most frecuent concomitant disease. Although the etiology of Sjögren syndrome is little known, its characterization, as well as the identification of the diagnostic and therapeutic means used, is of great help in understanding the disease; this information will allow the development of increasingly effective and specific treatments. More studies are required, in different locations, which will highlight the differences between populations.

Sindrome de Sjögren: una revisión de la literatura / Sjögren's syndrome: A review of the literature

El síndrome de Sjögren es un trastorno crónico autoinmune que afecta a las glándulas secretoras, principalmente salivales y lagrimales; además, puede presentar manifestaciones sistémicas extraglandulares. El objetivo de esta revisión fue revisar la literatura sobre los aspectos generales del síndrome de Sjögren, para lo cual se realizó una búsqueda en bases de datos entre el 15 de enero y el 15 de marzo del 2020, en donde se obtuvieron 29 artículos sobre los cuales se hizo la revisión. El síndrome de Sjögren tiene una importante prevalencia entre las enfermedades autoinmunes más comunes, caracterizada por presentar xerostomía y xeroftalmia. Los criterios diagnósticos tienen alta sensibilidad y especificidad y su tratamiento es sintomático.

Sjögren's syndrome is a chronic autoimmune disorder that affects the secretory glands, mainly salivary and lacrimal glands; and also can present extraglandular systemic manifestations. The objective of this review was to check the literature about the general aspects of Sjorgen's syndrome, for which a search of the literature was carried out between January 15 to March 15, 2020, 29 articles were obtained on which did the review. Sjögren's syndrome is highly prevalent among the most common autoimmune diseases, characterized by xerostomia and xerophthalmia. The diagnostic criteria have high sensitivity and specificity, and their treatment is symptomatic.