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.

Pregnancy-associated neuromyelitis optical spectrum disorder combined with primary Sjögren's syndrome: A critical illness case report / 北京大学学报(医学版)

Central nervous system involvement in primary Sjögren's syndrome (pSS) is less common and usually presents as white matter lesions, neuromyelitis optica spectrum disorder (NMOSD), or transverse myelitis. NMOSD is an immune-mediated inflammatory demyelinating disease of the central nervous system with a high rate of relapse and significant disability. Studies have shown that patients with pSS combined with NMOSD have more severe symptoms and poorer prognosis. Here, we present a case of critical illness in pregnancy-associated NMOSD combined with Sjögren's syndrome. The patient was a 30-year-old pregnant woman with a history of Sjögren's syndrome who was diagnosed with NMOSD. She received combination therapy with steroids, intravenous immunoglobulin (IVIG), and hydroxychloroquine during pregnancy, resulting in partial resolution of numbness below the waist. However, due to irregular medication adherence outside the hospital setting, she developed weakness in her right lower limb accompanied by inability to move it, while her left lower limb still had some mobility but occasional numbness along with urinary and fecal incontinence. Ten days later, she was admitted to the emergency department where an emergency cesarean section was performed to deliver a healthy baby boy. However, her condition worsened postpartum as she developed high fever accompanied by bilateral lower limb paralysis and weakness along with loss of voluntary control over urination and defecation. The patient underwent ano-ther course of treatment consisting of steroids and IVIG; however there was limited improvement in symptoms observed after this intervention. Following administration of rituximab for the first time, the patient developed urinary tract infection which was successfully managed before continuing regular infusions. In later stages the patient could walk slightly with a limp and regained control over urination and defecation, allowing her to resume normal activities. This case suggests that combination therapy with steroids, IVIG, and hydroxychloroquine should be considered for the patients with pregnancy-associated NMOSD combined with Sjögren's syndrome. Rituximab can significantly improve symptoms such as postpartum paralysis in patients with NMOSD, however, there may be a risk of infection associated with its use.

Clinical characteristics of fetal cardiac disease in patients with anti-SSA antibody positive / 北京大学学报(医学版)

OBJECTIVE@#To investigate the clinical manifestations and laboratory indicators of anti-Sjögren's-syndrome-related antigen A (SSA) antibody associated fetal cardiac disease.@*METHODS@#Pregnant women hospitalized at Peking University People's Hospital from January 2013 to July 2023 were included. Eleven patients with anti-SSA antibody positive were eventually diagnosed with fetal cardiac di-sease. And patients with anti-SSA antibody positive without fetal cardiac disease were selected as controls. Clinical manifestations, laboratory indications and drug usage were compared between the two groups.@*RESULTS@#Among these 11 patients, congenital heart block was confirmed in seven, which was the most common manifestations of fetal cardiac malformation. The proportion of the patients diagnosed with autoimmune disease before pregnancy in fetal cardiac malformation group was significantly lower than that in the control group (P=0.032), while most of the patients in the fetal cardiac malformation group received immune-related examinations for the first time because of this time's fetal cardiac diagnosis. While most of the patients in the control group received routine examinations because of autoimmune diseases diagnosed before pregnancy. During pregnancy, the white blood cell level [(9.29±2.58)×109/L vs. (7.10±1.90×109/L, t=3.052, P=0.004], erythrocyte sedimentation rate [(49.50 (48.00, 51.00) mm/h vs. 23.00 (15.00, 30.25) mm/h, Z=-2.251, P=0.024], IgA level [3.46 (2.30, 5.06) g/L vs. 2.13 (1.77, 2.77) g/L, Z=-2.181, P=0.029], and antinuclear antibody (ANA) titers [1∶320 (1∶160, 1∶320) vs. 1∶80 (1∶40, 1∶160), Z=-3.022, P=0.003] were significantly higher in fetal cardiac malformation group than in the control group. The proportion of positive anti-SSB antibody during pregnancy did not show a statistically significant difference between the two groups (37.5% vs. 7.7%, P=0.053). There was no significant difference in hydroxychloroquine dosage and initiation time between the two groups. The dosage of prednisone in the second and third trimesters was significantly higher in the cardiac malformation group than that in the control group, but there was no significant difference in the first trimester.@*CONCLUSION@#Fetal cardiac disease is rare in pregnant women with anti-SSA antibody. White blood cell, erythrocyte sedimentation rate, IgA, the titer of ANA positivity were higher in the fetal heart disease group during pregnancy. Since congenital heart block is difficult to reverse, its prevention and monitoring are more important than remedial treatment.

Comparison of clinical and immunological characteristics between primary Sjögren's syndrome patients with positive and negative anti-SSB antibody / 北京大学学报(医学版)

OBJECTIVE@#To analyze the differences of clinical manifestations and laboratory features between primary Sjögren's syndrome (pSS) patients with positive and negative anti-Sjögren's syndrome type B (SSB) antibody.@*METHODS@#The clinical data of pSS patients hospitalized in Department of Rheumato-logy and Immunology, Peking University Third Hospital were retrospectively analyzed to investigate the differences of clinical and laboratory features between anti-SSB positive and negative groups. The t test, Mann-Whitney U test, Chi-square test and Fisher's exact probability were used for analysis.@*RESULTS@#A total of 142 pSS patients were enrolled in this study, including 137 females and 5 males with a mean age of (54.8±13.3) years. The anti-SSB positive group included 44 patients accounting for 31.0% of the pSS patients. The anti-SSB positive pSS patients were younger at disease onset and at visit [age at visit: (50.9±14.5) years vs. (56.5±12.4) years; age at onset: (42.2±14.8) years vs. (49.5±15.3) years, P < 0.05]. The patients with anti-SSB positive more frequently presented with rash (29.5% vs. 14.3%, P < 0.05), enlargement of parotid glands (27.3% vs. 8.2%, P < 0.05), renal tubular acidosis (15.9% vs. 4.2%, P < 0.05), immune thrombocytopenia (9.1% vs. 1.0%, P < 0.05), rheumatoid factor (RF) positive (85.0% vs. 49.4%, P < 0.05), higher RF and antinuclear antibody (ANA) titers (median: 89.8 IU/mL vs. 20.5 IU/mL; median: 320 vs. 160, P < 0.05), anti-Sjögren's syndrome type A (SSA) antibody positive (97.7% vs. 64.3%, P < 0.05), elevation of γ globulin (71.4% vs. 38.5%, P < 0.05), higher levels of IgG (median: 21.0 g/L vs. 15.6 g/L, P < 0.05), higher proportions of CD3-CD19+ cells [(21.0±11.9)% vs. (13.7±9.6)%, P < 0.05] and lower proportions of CD3+ cells [(67.2±14.4)% vs. (76.6%±13.1)%, P < 0.05] than those negative. However, the anti-SSB positive group was less likely to show anti-mitochondrial antibodies (AMA)-M2 positivity (10.5% vs. 35.6%, P < 0.05). Glucocorticoids (90.9% vs. 73.5%, P < 0.05) and immunosuppressants (54.5% vs. 36.7%, P < 0.05) were more frequently used in anti-SSB positive pSS patients than those negative.@*CONCLUSION@#The anti-SSB positive pSS patients were younger at disease onset while more frequently presenting with various symptoms, higher levels of other antibodies and activation of B cells than those negative. Glucocorticoids and immunosuppressants were more frequently used, indicating that anti-SSB positive group presented with a more severe clinal phenotype.

Clinical features of oral management to oral complications of Sjögren's syndrome / 北京大学学报(医学版)

OBJECTIVE@#To understand the clinical characteristics about sequence diagnosis and treatment of oral complications in patients with Sjögren's syndrome (SS) through retrospective analysis, and to provide some guidance for clinical work.@*METHODS@#Some SS patients who underwent oral sequence management in the Department of General Dentistry, Peking University School and Hospital of Stomatology from January 2015 to September 2021 were enrolled. For the SS patients included in this study, a comprehensive oral examination was performed, including parotid region examination, oral mucosal exa-mination, dentition examination, dental examination, periodontal examination, unstimulated salivary flow rate, Candida infection and radiological imaging examination. According to the examination results, the patients were given fluoride application, antifungal treatment, root canal therapy, direct filling repair, and indirect repair treatment in sequence and the results recorded.@*RESULTS@#A total of 9 patients with SS, with 4 primary SS patients (pSS) and 5 secondary SS patients (sSS) were enrolled in the study. For all the 9 patients, the average age was (49.2±16.2) years and the median xerostomia duration 5 years. The unstimulated salivary flow rate of the 9 patients was all less than 1 mL/10 min. Eight of the 9 cases was diagnosed as oral Candidiasis, with positive salivary Candida culture result (>200 cfu/mL), and 1 of the 9 cases was not. The average decay, missing, filling teeth (DMFT) was 24.8±4.2; the average decay, missing, filling tooth surfaces (DMFS) was 59.2±21.9, the average incisal caries was 2.5±1.3, and the average number of crown restorations at baseline was 4.5±3.6. All the 9 SS patients were applied with topical fluoride usage, and 8 were prescribed with antifungal treatment. One sSS patient was conducted with filling restoration treatment, one pSS patient was conducted with full mouth rehabilitation, and the remaining 7 patients were conducted with direct filling combined with fixed repair treatment. The average 3.2 full crown restorations in 6 patients had to be removed and restored because of secondary caries, and 3 of the 9 patients underwent implant denture restorations finally.@*CONCLUSION@#Management of oral complications in SS patients needs to be carried out in sequence. A comprehensive examination and diagnosis should be carried out first, followed by infection control, and then restoration of oral function at last.

Utilidad de la proteína C reactiva en el posoperatorio inmediato de un paciente reumático con apendicitis aguda / Usefulness of C-reactive protein in the immediate postoperative period of rheumatic patients with acute appendicitis

Las enfermedades reumáticas son un grupo de afecciones que se caracterizan por la presencia de manifestaciones y complicaciones sistémicas derivadas del proceso inflamatorio mantenido. Los reactantes de fase aguda, como es el caso de la proteína C reactiva constituyen un marcador de actividad de la enfermedad. Sin embargo, su utilidad se magnifica en los pacientes reumáticos que tienen que ser sometidos a una intervención quirúrgica, circunstancia en la que se tornan un marcador eficiente que muestra la magnitud del proceso inflamatorio. Se presenta el caso de una paciente femenina de 53 años edad, con diagnóstico de artritis reumatoide y síndrome de Sjögren secundario, que tuvo que ser sometida a intervención quirúrgica por diagnóstico de apendicitis aguda. Se realizó seguimiento clínico y de laboratorio utilizando la proteína C reactiva como marcador de elección para monitorear la intensidad del proceso inflamatorio resultante de la intervención quirúrgica y de los efectos de esta en la actividad clínica de las enfermedades reumáticas de base. Después del tratamiento quirúrgico y medicamentoso, la paciente fue dada de alta hospitalaria sin manifestaciones articulares, digestivas ni alteraciones de los exámenes de laboratorio(AU)

Rheumatic diseases are a group of conditions characterized by the presence of systemic manifestations and complications derived from the sustained inflammatory process. Acute phase reactants, such as C-reactive protein, constitute a marker of disease activity. However, its usefulness is magnified in those rheumatic patients who have to undergo surgery, being an efficient marker that shows the magnitude of the inflammatory process. We present the case of a 53-year-old female patient diagnosed with rheumatoid arthritis and secondary Sjögren's syndrome who had to undergo surgery due to a diagnosis of acute appendicitis. Clinical and laboratory follow-up was performed using C-reactive protein as the marker of choice to monitor the intensity of the inflammatory process resulting from the surgical intervention and its effects on the clinical activity of underlying rheumatic diseases(AU)

Long-term follow-up of a case of choroidal neovascularization secondary to reticular pigmentary retinal dystrophy / Seguimento de longo-prazo de um caso de neovascularização de coroide secundária à distrofia reticular pigmentar da retina

ABSTRACT Reticular pigmentary retinal dystrophy, also known as Sjögren's reticular dystrophy, is a rare condition characterized by macular lesions with a reticular pattern, which are best seen on fluorescein angiogram. Choroidal neovascularization secondary to this type of dystrophy is even less common. This report describes a case of reticular pigmentary retinal dystrophy with vision loss due to neovascular membrane, which responded well to treatment with anti-vascular endothelial growth factor.

RESUMO A distrofia reticular pigmentar da retina, também conhecida como distrofia reticular de Sjögren, é uma doença rara, caracterizada por lesões maculares com um padrão reticular, que são mais bem visualizadas na angiografia com fluoresceína. A neovascularização de coroide secundária a este tipo de distrofia é ainda menos comum. Este relato descreve um caso de distrofia reticular pigmentar da retina, com perda de visão devido à membrana neovascular, que respondeu bem ao tratamento com fator de crescimento endotelial antivascular.

Subconjunctival adalimumab for treatment of dry eye disease in Sjögren's syndrome / Tratamento de olho seco com adalimumabe subconjuntival em pacientes portadores da síndrome de Sjögren

ABSTRACT Objective To describe the use of subconjuctival administration of the anti-tumor necrosis factor agent adalimumab for treatment of dry eye in patients with Sjögren's syndrome, and to investigate conjunctival healing. Methods Prospective, nonrandomized, noncomparative interventional case series including consecutive patients with Sjögren's syndrome and dry eye disease treated with subconjunctival adalimumab, who were refractory to conventional treatment. Patients with infectious ocular surface involvement or structural changes in the tear pathway or eyelids were excluded. Data recorded included age, sex, lissamine green staining pattern, Schirmer test results, intraocular pressure, conjunctival mobility, tear break up time and findings of biomicroscopic evaluation, following fluorescein dye instillation. The Ocular Surface Disease Index questionnaire validated for the Portuguese language was used for subjective assessment of patients. Results Eleven eyes of eight patients were studied. Mean patient age was 53±13.4 years. Patients were treated with subconjunctival injection of 0.03 mL of adalimumab and followed for 90 days thereafter. There were no statistically significant objective improvement (objective tests results; p>0.05) and no statistically significant changes in intraocular pressure (p=0.11). Questionnaire responses revealed a significant improvement in ocular symptoms (p=0.002). Conclusion Based on the Ocular Surface Disease Index questionnaire, subconjunctival administration of adalimumab improved dry eye symptoms. However, objective assessments failed to reveal statistically significant improvements.

RESUMO Objetivo Descrever o uso subconjuntival do antifator de necrose tumoral adalimumabe para o tratamento do olho seco em pacientes com síndrome de Sjögren e avaliar a cicatrização conjuntival. Métodos Série de casos intervencionista com desenho prospectivo, não randomizado, não comparativo. O medicamento adalimumabe foi aplicado em região subconjuntival em pacientes com síndrome de Sjögren e olho seco que eram resistentes a outras terapias convencionais. Pacientes com patologias oculares de origem infecciosa ou com alterações estruturais nas vias lacrimais e pálpebras foram excluídos do estudo. Os dados coletados incluíram idade, sexo, teste com lisamina verde, teste de Schirmer, pressão intraocular, mobilidade conjuntival, teste de ruptura do filme lacrimal, e avaliação biomicroscópica com colírio de fluoresceína. Além disso, o questionário Ocular Surface Disease Index validado para a língua portuguesa foi aplicado com objetivo de avaliar subjetivamente a resposta dos pacientes ao tratamento. Resultados Onze olhos de oito pacientes foram estudados. A idade média dos pacientes foi de 53±13,4 anos. A dose aplicada de adalimumabe subconjuntival foi de 0,03mL, e a duração do seguimento foi de 90 dias após a injeção. Não houve melhora estatisticamente significativa nos testes objetivos (todos apresentaram p>0,05). A pressão intraocular também não sofreu variações estatisticamente significativas (p=0,11). Entretanto, por meio do questionário, foi registrada melhora significativa dos sintomas oculares (p=0,002). Conclusão O uso do adalimumabe subconjuntival melhorou os sintomas de olho seco, avaliados por meio do questionário Ocular Surface Disease Index, mas não houve melhora estatisticamente significativa na avaliação objetiva.

Tumor de Klatskin en una paciente con diagnóstico de síndrome de Sjögren / Klatskin tumor in a patient diagnosed with Sjögren's syndrome

El proceso inflamatorio crónico de las enfermedades reumáticas constituye un factor que causa afectación en otros órganos y sistemas de órganos. La afectación puede llegar a producir metaplasia de la mucosa de distintos tejidos y condicionar la aparición de enfermedades neoplásicas. Los pacientes con síndrome de Sjögren tienen un 40 por ciento de aumento de riesgo de padecer una enfermedad tumoral con respecto a los pacientes sanos o con otras enfermedades crónicas no inflamatorias. El objetivo del presente estudio es dar a conocer las características clínico-imagenológicas que posibilitaron el diagnóstico de un tumor de Klatskin en una paciente femenina de 57 años de edad con antecedentes de 5 años de evolución del síndrome de Sjögren. Se realizó una colangiopancreatografía retrógrada endoscópica para eliminar el tumor. A los tres días de la intervención quirúrgicamente la paciente muestra una evolución favorable por lo que se decide dar alta hospitalaria con seguimiento por consulta externa de especialidades de cirugía general y reumatología indistintamente. Durante todo el procedimiento se mantuvo el tratamiento para la enfermedad de base de la paciente. Actualmente la paciente se encuentra incorporada a sus actividades cotidianas y con una evolución favorable de su estado de salud(AU)

The chronic inflammatory process of rheumatic diseases constitutes a factor that causes affectation in other organs and organ systems. The affectation can produce metaplasia of the mucosa of different tissues and condition the appearance of neoplastic diseases. Patients with Sjögren's syndrome have a 40 percent increased risk of developing a tumor disease compared to healthy patients or other non-inflammatory chronic diseases. The objective of this study is to present the clinical-imaging characteristics that made the diagnosis of a Klatskin tumor possible in a 57-year-old female patient with a 5-year history of Sjögren's syndrome. Endoscopic retrograde cholangiopancreatography was performed to remove the tumor. Three days after the surgical intervention, the patient showed a favorable evolution, so it was decided to discharge from the hospital with follow-up by outpatient consultation of specialties of general surgery and rheumatology indistinctly. Treatment for the patient's underlying disease was maintained throughout the procedure. Currently the patient is incorporated into her daily activities and with a favorable evolution of her state of health(AU)

Atypical presentation of Sjogren's syndrome with MALT lymphoma mimicking chronic venous ulcers / Apresentação atípica da síndrome de Sjogren com linfoma MALT que mimetiza úlceras crônicas venosas

Abstract Chronic ulcerations of the lower extremities are quite a common condition amongst adults, most often caused by chronic venous insufficiency. Irrespective of the main underlying cause, chronic limb ulcerations are usually associated with significant symptoms, impairing daily functioning. Improper or delayed diagnosis and inadequate treatment increase the risk of serious complications, including limb amputations. Malignancies can develop secondary to chronic leg ulcers. About 2.4% of ulcers arising from chronic venous stasis undergo malignant transformation. Squamous cell carcinoma is the most common type of malignancy found in chronic leg ulceration biopsies. Basal cell carcinoma, sarcoma, and melanoma have all been documented infrequently. In the case described here, we found lymphoma of the marginal zone of mucosa-associated lymphoid tissue (MALT), which is an extremely rare cutaneous neoplasm of the lower extremities, but one that may have an association with autoimmune diseases.

Resumo Úlceras crônicas dos membros inferiores são uma condição bastante comum entre adultos, na maioria das vezes causadas por insuficiência venosa crônica. Independente da sua causa principal, úlceras crônicas dos membros estão geralmente associadas a sintomas significativos, prejudicando o funcionamento diário. O diagnóstico inadequado ou tardio e o tratamento inadequado aumentam o risco de complicações graves, inclusive o risco de amputação do membro. Malignidades podem se desenvolver secundariamente em úlceras crônicas da perna. Em torno de 2,4% das úlceras decorrentes de estase venosa crônica sofrem transformação maligna. O carcinoma de células escomosas é o tipo de malignidade mais comum que pode ser encontrada em biópsias de úlceras crônicas da perna. Carcinoma basocelular, sarcoma e melanoma foram raramente documentados. No caso aqui descrito, encontramos linfoma da zona marginal do tecido linfoide associado à mucosa (mucosa-associated lymphoid tissue, MALT), o qual é uma neoplasia cutânea dos membros inferiores extremamente rara mas que pode estar associada a doenças autoimunes.

Congenital complete atrioventricular heart block in a pregnant woman with sjögren syndrome: prenatal care follow-up and the challenge of intrauterine treatment / Bloqueio atrioventricular completo congênito em uma mulher grávida com síndrome de Sjögren: seguimento pré-natal e desafio do tratamento intrauterino

Abstract The present report describes a case of complete atrioventricular block (CAVB) diagnosed at 25 weeks of gestation in a pregnant woman with Sjögren's syndrome and positive anti-Ro/SSA antibodies. Fluorinated steroids (dexamethasone and betamethasone) and terbuline were used to increase the fetal heart rate, but the fetal heart block was not reversible, and the administration of drugs was discontinued due to maternal collateral effects. Follow-up fetal echocardiograms were performed, and the fetus evolved with pericardial effusion, presence of fibroelastosis in the right ventricle, and ventricular dysfunction. Interruption of pregnancy by cesarean section was indicated at 34 weeks of gestation, and a cardiac pacemaker was implanted in the male newborn immediately after birth. Therapy for fetuses with CAVB is controversial mainly regarding the use or not of corticosteroids; however, monitoring of the atrioventricular interval by fetal echocardiography should be performed in fetuses from pregnant women with positive autoantibodies anti-Ro/SSA and/or anti-La/SSB to prevent the progression to CAVB.

Resumo Este relato descreve um caso de bloqueio atrioventricular completo (BAVC) diagnosticado com 25 semanas de gestação em uma mulher com síndrome de Sjögren e anticorpos anti-Ro/SSA positivos. Esteroides fluoretados (dexametasona e betametasona) e terbulina foram utilizados para aumentar a frequência cardíaca fetal, mas o bloqueio cardíaco fetal não foi reversível, e a administração dos medicamentos foi interrompida devido a efeitos colaterais maternos. Ecocardiogramas fetais de acompanhamento foram realizados, e o feto evoluiu com derrame pericárdico, presença de fibroelastose no ventrículo direito, e disfunção ventricular. A interrupção da gravidez por cesariana foi indicada com 34 semanas, e um marca-passo cardíaco foi implantado no recém-nascido do sexo masculino imediatamente após o nascimento. A terapia para fetos com BAVC é controversa, principalmente no que diz respeito ao uso ou não de corticosteroides; no entanto, o monitoramento do intervalo atrioventricular pela ecocardiografia fetal deve ser feito em fetos de mulheres grávidas com autoanticorpos positivos anti-Ro/SSA e/ou anti-La/SSB para impedir a progressão para o BAVC.

Vasculitis crioglobulinémica con compromiso renal y síndrome de Sjögren / Cryoglobulinemic vasculitis with renal involment and Sjögren's syndrome

La vasculitis crioglobulinémica es una vasculitis de vasos pequeños que se da en presencia de crioglobulinas séricas. Las crioglobulinemias mixtas son las más frecuentes y se asocian a infecciones crónicas, típicamente al virus de la hepatitis C, así como a enfermedades linfoproliferativas y autoinmunes, más a menudo al síndrome de Sjögren. Las manifestaciones clínicas incluyen púrpura de los miembros inferiores, neuropatía periférica, artralgias y glomerulonefritis. La presencia de vasculitis crioglobulinémica en el contexto de un síndrome de Sjögren es marcadora de peor pronóstico. Presentamos el caso de una paciente con vasculitis crioglobulinémica con compromiso cutáneo y renal, que condujo al diagnóstico de un síndrome de Sjögren primario. (AU)

Cryoglobulinemic vasculitis is a small-sized vasculitis that occurs in the context of serum cryoglobulins. Mixed cryoglobulinemias are the most frequent and are associated with chronic infections, typically hepatitis C and autoimmune diseases, most commonly Sjögren's syndrome. Clinical manifestations include purpura of lower limbs, peripheral neuropathy, arthralgias and glomerulonephritis. The presence of cryoglobulinemic vasculitis in the context of Sjögren's syndrome is a marker of poor prognosis. We present the case of a patient with cryoglobulinemic vasculitis associated to cutaneous and renal involvement that led us to the diagnosis of primary Sjögren's syndrome. (AU)

Association between three autoimmune diseases: vitiligo, primary biliary cirrhosis, and Sjögren's syndrome

Abstract Although the association of multiple autoimmune diseases has already been widely described, no reports of the association between vitiligo, primary biliary cirrhosis and Sjogren's syndrome were retrieved in the SciELO and PubMed databases. The authors describe the case of a female patient who was diagnosed with primary biliary cirrhosis and Sjogren's syndrome at age 54. At age 58, she developed vitiligo restricted to the face, associated with significant impairment of self-esteem and quality of life. Antinuclear antibody was negative at the onset of the condition, but became positive after phototherapy initiation. In general, the occurrence of multiple autoimmune diseases in the same patient is known as a mosaic of autoimmunity. However, specific mechanisms appear to interconnect primary biliary cirrhosis and Sjogren's syndrome, such as PDC-E2-mediated generalized epithelitis.

Síndrome Sjögren y penfigoide de las membranas mucosas en paciente con antecedente de timoma: Reporte de caso y revisión de literatura / Sjögren Syndrome and Mucous Membrane Pemphigoid in a Patient with a History of Thymoma: Case Report and Literature Review

RESUMEN Los timomas son las neoplasias mediastínicas más comunes en adultos. Los pacientes con timoma son frecuentemente diagnosticados con síndromes paraneoplásicos y generalmente están asociados con varias condiciones autoinmunes. El síndrome de Sjögren (SS) es un trastorno inflamatorio autoinmune, que se caracteriza por la destrucción mediada por linfocitos de las glándulas exocrinas, que conduce a la ausencia de secreción glandular, en particular las salivales y las lagrimales; la hiposalivación puede variar desde una ligera reducción en el flujo de saliva con molestias transitorias, a un grave deterioro de la salud oral y malestar psicológico. El penfigoide de membranas mucosas (PMM) es una enfermedad autoinmune crónica, muestra predominante afectación de la mucosa, afectando con mayor frecuencia la cavidad oral, seguida de la conjuntiva, la cavidad nasal y el esófago; intraoralmente incluye gingivitis descamativa, vesículas, erosiones cubiertas por pseudomembranas y úlceras. El objetivo de este trabajo es presentar el primer caso de una paciente con antecedente de timoma, la cual, conjuntamente presentó SS y PMM; además evidenciar la importancia del manejo multidiciplinario para un diagnóstico preciso y temprano, ya que así, aumenta la eficiencia y la eficacia de la estrategia de tratamiento.

ABSTRACT Thymomas are the most common mediastinal neoplasms in adults. Patients with thymoma are frequently diagnosed with paraneoplastic syndromes and are generally associated with several autoimmune conditions. Sjögren's syndrome (SS) is an autoimmune inflammatory disorder, characterized by the lymphocyte-mediated destruction of the exocrine glands, which leads to the absence of glandular secretion, particularly salivary and lacrimal; hyposalivation can vary from a slight reduction in the flow of saliva with transient discomfort, to a serious deterioration of oral health and psychological distress. Mucous membrane pemphigoid (MMP) is a chronic, autoimmune disease that shows predominant mucosal involvement, most commonly affecting the oral cavity, followed by the conjunctiva, the nasal cavity and the esophagus; intraorally includes scaly gingivitis, vesicles, erosions covered by pseudomembranes and ulcers. The objective of this work is to present the first case of a patient with a history of thymoma, which, jointly presented SS and MMP; also highlight the importance of multidisciplinary management for an accurate and early diagnosis, as this increases the efficiency and efficacy of the treatment strategy.

Frecuencia del síndrome de Sjögren en pacientes con lupus eritematoso del Hospital Central del Instituto de Previsión Social en el año 2017 que acuden a tratamiento odontológico / Frequency of sjögren's syndrome in patients with lupus erythematosus at the Central Hospital of the Social Welfare Institute in 2017 who seek dental treatment

La xerostomía, síndrome de boca seca o síndrome de Sjögren se define como la disminución del flujo salival en condiciones de reposo. El origen de esta endad clínica es mulcausal, pudiendo ser el resultado de una alteración localizada sobre las glándulas productoras de la saliva, o bien el resultado de un desequilibrio o alteración de índole sistémica. La evaluación del grado de disfunción de las glándulas salivares ha constuido un objevo básico por ello el propósito de este estudio fue determinar frecuencia del síndrome de Sjögren en pacientes con lupus eritematoso del hospital central del Instuto de Previsión Social en el año 2017 que acuden a tratamiento odontológico. Se realizó un descripción observacional retrospecvo para lo cual se recurrió a la historial clínico previo permiso del jefe de reumatología para acceder a los datos . Como conclusión general se obtuvo que existe una predominancia de pacientes de entre 46 a 50 años de edad representada por el 39% y de sexo femenino en el 83% de los casos. Se demuestra la frecuencia mencionando que acudieron 74 pacientes y de los cuales 18 (24%) personas presentan lupus eritematoso y síndrome de Sjögren, y entre estos el 78% acude a tratamiento odontológico.

Xerostomia, dry mouth syndrome or Sjögren's syndrome is defined as decreased salivary flow under resng condions. The origin of this clinical enty is mulcausal, and may be the result of an alteraon located on the glands producing saliva, or the result of an imbalance or alteraon of a systemic nature. The evaluaon of the degree of dysfuncon of the salivary glands has been a basic objecve and therefore the purpose of this study was to determine the frequency of Sjögren syndrome in paents with lupus erythematosus from the central hospital of the Social Welfare Instute in 2017 who seek dental treatment. A retrospecve observaonal descripon was made, for which the clinical history was used with the prior permission of the head of rheumatology to access the data. The general conclusion was that there is a predominance of paents between 46 and 50 years of age represented by 39% and female in 83% of cases. The frequency is demonstrated by menoning that 74 paents aended and of which 18 (24%) people present with lupus erythematosus and Sjögren's syndrome, of which 78% aend dental treatment

Recommendations from the Brazilian society of rheumatology for the diagnosis of Sjögren's syndrome (Part I): glandular manifestations (systematic review)

Abstract Background: Primary Sjögren's syndrome (pSS) is a systemic immune-mediated disease whose main characteristic is exocrine gland inflammation and, subsequent reduction in tear and saliva production. A delayed diagnosis is common due to the nonspecific clinical manifestations of disease. The aim of the present study was to develop recommendations for the diagnosis of glandular manifestations of pSS based on evidence and expert opinion. Main body of the abstract: We conducted a systematic literature review to retrieve the best evidence available on the accuracy of diagnostic tests for pSS. We also held two in-person meetings with experts (rheumatologists, pathologists, ophthalmologists and dentists) to establish their level of agreement using the Delphi method. Ultimately, we generated 18 recommendations that aim to facilitate the diagnosis of the glandular manifestations of pSS. Conclusion: The diagnosis of glandular manifestations of pSS is complex and multidisciplinary. It requires specific knowledge in the field of ophthalmology, immunology, pathology and imaging, making it compulsory for the rheumatologist to work with professionals from these different areas in order to improve accuracy and early diagnosis. Glandular dysfunction tests, ANA, RF, Anti-Ro, protein electrophoresis, urinalysis, blood count, C-Reactive protein, complement, testing for syphilis and viruses (HCV, HIV) and SGUS should be investigated when dryness or systemic manifestation are present. Minor salivary gland biopsy is recommended for all anti-Ro negative or incomplete criteria cases.

Linfoma no Hodgkin de amígdalas en un paciente con lupus eritematoso sistémico / Non-Hodgkin's lymphoma of tonsils in a patient with systemic lupus erythematous

Introducción: las enfermedades reumáticas aumentan el riesgo de aparición de distintas comorbilidades y estado de salud inadecuado en los pacientes. Dentro de estas comorbilidades las más peligrosas, por la frecuencia que se producen y por el desenlace final de las mismas lo constituyen las enfermedades neoplásicas. Objetivos: socializar los elementos clínicos, de laboratorio e histopatológicos que permiten la sospecha clínica y el diagnóstico de linfoma no Hodgkin en pacientes con enfermedades reumáticas. Caso clínico: paciente femenina de 54 años de edad, con diagnóstico de lupus eritematoso sistémico y síndrome de Sjögren secundario que acude con manifestaciones clínicas dadas por sudores nocturnos profusos, toma del estado general, fiebre vespertina y adenopatías cervicales. Se le realiza el diagnóstico de linfoma no Hodgkin en amígdala derecha. Conclusiones: las enfermedades reumáticas aumentan el riesgo de aparición de enfermedades neoplásicas. El seguimiento periódico, la adherencia farmacológica y el monitoreo constante de manifestaciones generales y elementos de sospecha de procesos malignos, son las acciones fundamentales que se pueden realizar para prevenir o diagnósticas precozmente la aparición de afecciones neoplásicas en pacientes reumáticos(AU)

Introduction: rheumatic diseases increase the risk of the appearance of different comorbidities and inadequate health status in patients. Within these comorbidities the most dangerous, by the frequency that occur and by the final outcome of them are neoplastic diseases. Objectives: to socialize the clinical, laboratory and histopathological elements that allow clinical suspicion and the diagnosis of non-Hodgkin's lymphoma in patients with rheumatic diseases. Case report: A 54-year-old female patient with a diagnosis of systemic lupus erythematous and secondary Sjogren's syndrome who presented with clinical manifestations due to profuse nocturnal sweats, general condition, afternoon fever and cervical lymphadenopathy. He is diagnosed with non-Hodgkin's lymphoma in the right amygdala. Conclusions: rheumatic diseases increase the risk of the appearance of neoplastic diseases. The periodic follow-up, the pharmacological adherence and the constant monitoring of general manifestations and elements of suspicion of malignant processes, are the fundamental actions that can be performed to prevent or early diagnosis the appearance of neoplastic affections in rheumatic patients(AU)

Impact of environmental pollution on the ocular surface of Sjögren's syndrome patients / Impacto da poluição ambiental na superfície ocular de pacientes com síndrome de Sjögren

ABSTRACT Purpose: To evaluate the effect of air pollution on the ocular surface of patients with Sjögren's syndrome. Methods: We investigated the ocular surfaces of thirty patients with Sjögren's syndrome and thirty healthy volunteers (control group) living in the Metropolitan Area of Buenos Aires. We used nitrogen dioxide as an indicator of exposure to air pollution. An ocular symptoms questionnaire was answered by all subjects, who also underwent a complete ocular surface ophthalmic examination-including an Ocular Surface Disease Index questionnaire, biomicroscopy, tear breakup time, Schirmer 1 test, corneal and conjunctival vital staining with fluorescein and lissamine green, tear lysozyme concentration, and impression cytology. Results: In almost all ocular surface test findings, we found a positive and significant correlation between higher levels of exposure to air pollution and higher levels of ocular surface damage in both the control group and Sjögren's syndrome patients. In Sjögren's syndrome patients, the Ocular Surface Disease Index questionnaire, tear breakup time, vital staining and impression cytology showed a significant correlation between high levels of air pollution and ocular surface disease. In the control group, the Ocular Surface Disease Index questionnaire, tear breakup time, and impression cytology showed a significant correlation between high levels of air pollution and ocular surface disease. Conclusions: Here we demonstrated that in patients with dry eye syndrome associated with Sjögren, abnormalities of the ocular surface and eye irritation related to air pollution are more severe than those in the control group. We believe that measuring air quality should be not only an integral part of the evaluation of ocular surface disease but also a therapeutic consideration.

RESUMO Objetivo: Avaliar o efeito da poluição do ar na superfície ocular de pacientes com síndrome de Sjögren. Métodos: Foram investigadas as superfícies oculares de trinta pacientes com síndrome de Sjögren e trinta voluntários saudáveis (grupo controle) residentes na Região Metropolitana de Buenos Aires. Usamos o dióxido de nitrogênio como um indicador de exposição à poluição do ar. Um questionário de sintomas oculares foi respondido por todos os indivíduos, que também foram submetidos a um exame oftalmológico completo da superfície ocular - incluindo um questionário do Índice da Doença da Superfície Ocular, biomicroscopia, tempo de ruptura do filme lacrimal, teste de Schirmer 1, coloração da córnea e conjuntiva com fluoresceína e lissamina verde, concentração de lisozima lacrimal e citologia de impressão. Resultados: Em quase todos os achados do teste de superfície ocular, encontramos uma correlação positiva e significativa entre níveis mais altos de exposição à poluição do ar e níveis mais elevados de danos na superfície ocular tanto no grupo controle quanto nos pacientes com síndrome de Sjögren. Em pacientes com síndrome de Sjögren, o questionário do Índice da Doença da Superfície Ocular, tempo de ruptura do filme lacrimal, coloração vital e citologia de impressão mostraram uma correlação significativa entre altos níveis de poluição do ar e doença da superfície ocular. No grupo controle, o questionário do Índice de Doenças da Superfície Ocular, tempo de ruptura do filme lacrimal e citologia de impressão mostraram uma correlação significativa entre altos níveis de poluição do ar e doença da superfície ocular. Conclusões: Aqui demonstramos que, pacientes com síndrome de olho seco associada a Sjögren, as anormalidades da superfície ocular e a irritação ocular relacionadas à poluição do ar são mais graves do que aquelas no grupo controle. Acreditamos que a medição da qualidade do ar não deve ser apenas uma parte integral da avaliação da doença da superfície ocular, mas também uma consideração terapêutica.

Oral Nodular Lesions in Patients with Sjögren's Syndrome: Unusual Oral Implications of a Systemic Disorder

Abstract Sjögren's syndrome (SS) is a systemic chronic autoimmune disorder affecting the lacrimal and salivary glands. SS may manifest as primary SS (pSS) or secondary SS (sSS), the latter occurring in the context of another autoimmune disorder. In both cases, the dry eyes and mouth affect the patient's quality of life. Late complications may include blindness, dental tissue destruction, oral candidiasis and lymphoma. This paper reports two cases of SS, each of them presenting unusual oral nodular lesion diagnosed as relapsed MALT lymphoma and mucocele. The importance of the diagnosis, treatment and management of the oral lesions by a dentist during the care of SS patients is emphasized, as the oral manifestations of SS may compromise the patient's quality of life.

Resumo A síndrome de Sjögren (SS) é uma doença autoimune crônica sistêmica que afeta as glândulas lacrimal e salivar. A SS pode se manifestar como SS primária (SSp) ou SS secundária (SSs), a última ocorrendo em conjunto com outra desordem autoimune. Em ambos os casos, os olhos secos e a boca seca afetam a qualidade de vida do paciente. As complicações tardias podem incluir cegueira, destruição dos tecidos dentários, candidíase oral e linfoma. Este artigo relata dois casos de SS, cada um apresentando lesão nodular oral incomum diagnosticada como linfoma MALT reincidente e mucocele. A importância do diagnóstico, tratamento e manejo das lesões orais por um cirurgião-dentista durante o atendimento de pacientes com SS é enfatizada, pois as manifestações orais da SS podem comprometer a qualidade de vida do paciente.

Are the women with Sjögren's Syndrome satisfied with their sexual activity? / As mulheres com síndrome de Sjögren estão satisfeitas com sua atividade sexual?

ABSTRACT Objective: Females with Sjögren's Syndrome (SS) often experience vaginal dryness and dyspareunia, along with glandular and extraglandular symptoms. We aimed to evaluate sexual function and life quality in women with SS. Methods: Forty-six premenopausal women with SS and 47 age-matched controls were studied. Age, duration of the disease, medications, and comorbid diseases were noted. Participants completed 36-Item Short Form Health Survey (SF-36) and Female Sexual Function Index (FSFI). Patients were asked about vaginal discharge and itching in the last month, and if they informed their rheumatologists about any sexual problems. Gynecologic examinations were performed and vaginal smears were taken on each participant. Results: The median total scores of FSFI were significantly lower in the SS group than the controls [17.12 (2.4-27.8) and 27.4 (16.9-36.0), respectively, p < 0.001]. In the SS group, 37 (80.4%) and in the control group 18 (38.3%) of patients were sexually dissatisfied (p < 0.001). Vaginal dryness and lubricant use were significantly increased in patients with SS compared to controls (p < 0.001). Life quality scores were significantly lower in patients with SS than the controls (p < 0.001). Vaginal dryness was negatively correlated with FSFI total (r = −0.312, p = 0.035) and subscores except desire and arousal. Physical functioning, role physical and role emotional scores were positively correlated with total FSFI scores (r = 0.449, p = 0.002, r = 0.371, p = 0.011, r = 0.299, p = 0.043, respectively). Conclusions: Women with SS experience less satisfaction with sexual activity, which can be affected by age, vaginal dryness, physical pain, and impaired function due to the disease. Therefore, rheumatologists should pay attention to these symptoms and management.

RESUMO Objetivo: As mulheres com síndrome de Sjögren (SS) muitas vezes experimentam secura vaginal e dispareunia, juntamente com sintomas glandulares e extraglandulares. Este estudo objetivou avaliar a função sexual e a qualidade de vida de mulheres com SS. Métodos: Estudaram-se 46 mulheres pré-menopáusicas com SS e 47 controles pareados por idade. Avaliaram-se a idade, a duração da doença, os medicamentos usados e as comorbidades. As participantes preencheram o questionário de qualidade de vida 36-Item Short Form Health Survey(SF-36) e o Female Sexual Function Index (FSFI). As pacientes foram perguntadas quanto à presença de corrimento e prurido vaginal no último mês e se haviam informado a seus reumatologistas sobre quaisquer problemas sexuais. Fizeram-se exames ginecológicos e esfregaços vaginais de todas as participantes. Resultados: A mediana do escore total do FSFI foi significativamente menor no grupo SS do que no grupo controle [17,12 (2,4 a 27,8) e 27,4 (16,9 a 36), respectivamente, p < 0,001]. Nos grupos SS e controle, 37 (80,4%) e 18 (38,3%) das pacientes estavam sexualmente insatisfeitas, respectivamente (p < 0,001). A presença de secura vaginal e o uso de lubrificantes foram significativamente mais frequentes em pacientes com SS em relação aos controles (p < 0,001). Os índices de qualidade de vida foram significativamente menores nas pacientes com SS do que nos controles (p < 0,001). A secura vaginal esteve negativamente correlacionada com o FSFI total (r = −0,312 p = 0,035) e com todos os seus subescores, exceto desejo e excitação. Os escores de capacidade funcional, aspecto físico e aspecto emocional se correlacionaram positivamente com a pontuação total do FSFI (r = 0,449, p = 0,002; r = 0,371, p = 0,011; r = 0,299, p = 0,043, respectivamente). Conclusões: As mulheres com SS têm menor satisfação com a atividade sexual, o que pode ser afetado pela idade, secura vaginal, dor física e função prejudicada em razão da doença. Portanto, os reumatologista devem prestar atenção a esses sintomas e seu tratamento.