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.

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)

Systemic autoimmune diseases and work outcomes in Brazil: a scoping review

ABSTRACT OBJECTIVE To review articles that assessed work-related outcomes such as workability, work productivity, presenteeism, absenteeism, sick leave, return to work, and employment status of Brazilian patients with rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, Sjögren's syndrome, and systemic autoimmune myopathies. METHODS This study was conducted in Medline databases (PubMed), SciELO, and Lilacs through a combination of descriptors of interest. Studies published until December 2020 were considered in the search strategy. RESULTS Eight out of 90 articles met the eligibility criteria and were included in this review. The studies are highly heterogeneous. Most of them are cross-sectional, and all of them address rheumatoid arthritis or systemic lupus erythematosus. A common denominator among these studies is the high proportion of patients outside the labor market. CONCLUSIONS In general, the studies show unfavorable labor outcomes and impaired participation in the Brazilian workforce among the samples of patients assessed. There is a need to better understand several topics about Brazilian patients with systemic autoimmune diseases and their work context, as well as to conduct studies focusing on rarer diseases and on the themes of return and reintegration to work.

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.

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.

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)

Reativação da BCG após vacina contra COVID-19: relato de caso / BCG reactivation after COVID-19 vaccine: case report

A reativação da BCG pode ocorrer em diversos contextos: associada a quadros infecciosos, imunossupressão, autoimunidade e pós-vacinações. Além disso, especialmente em crianças abaixo de 5 anos de idade, deve ser valorizada como um achando presente em cerca de 50% dos casos de Doença de Kawasaki. Neste artigo, relatamos o primeiro caso publicado na literatura de uma paciente adulta jovem, a qual manifestou uma reativação de BCG após receber a primeira dose de vacina contra COVID-19 (AztraZeneca/Oxford/Biomanguinhos). Dentro das primeiras 24h após a administração da vacina, a paciente desenvolveu febre alta, sudorese, dor local, mialgia difusa e cefaleia. Após dois dias, iniciou eritema e enduração no local da cicatriz da vacina BCG. Ela tem como comorbidade a urticária crônica espontânea, porém estava assintomática sem crises há mais de 1 ano. Tem como antecedente familiar relevante o óbito materno por síndrome complexa de sobreposição de autoimunidade (lúpus eritematoso sistêmico, síndrome de Sjögren e síndrome do anticorpo antifosfolípide). Após ser medicada com anti-inflamatórios não esteroides (AINE) e corticoterapia tópica de moderada potência por 3 dias, houve resolução completa da reativação da BCG. A paciente, após 3 meses, recebeu a segunda dose da vacina e não manifestou nenhum sintoma. Acredita-se que a reativação da BCG ocorra devido a um mecanismo de reação cruzada entre HSP do indivíduo, elicitadas como mediadores da imunidade inata frente à inflamação vacinal, com alguns epítopos do M. bovis. Recomendase que seja investigada alguma condição imunossupressora ou autoimune nos pacientes que manifestem reativação da BCG, principalmente em adultos, na qual a doença de Kawasaki é bastante rara. As vacinas, incluindo as contra COVID-19, também podem desencadear o surgimento deste fenômeno imunológico ainda pouco compreendido.

BCG reactivation can occur in different contexts: associated with infectious conditions, immunosuppression, autoimmunity and post-vaccinations. Also, especially in children below of 5 years of age, should be valued as a finding present in about 50% of cases of Kawasaki disease. In this article, we report the first case published in the literature of a young adult patient, who manifested a reactivation of BCG after receiving the first dose of vaccine against COVID-19 (AztraZeneca/Oxford/Biomanguinhos). Within the first 24 hours after the administration of the vaccine, the patient developed high fever, sweating, local pain, diffuse myalgia and headache. After 2 days, erythema and induration at the site of the BCG vaccine scar began. she has how comorbidity to chronic spontaneous urticaria, but she was asymptomatic without crises for more than 1 year. The relevant family history is maternal death due to the complex syndrome of autoimmunity overlap (systemic lupus erythematosus, Sjögrens syndrome, and anti-phospholipid antibody). After being medicated with NSAID and moderate topical corticosteroid therapy potency for 3 days, there was complete resolution of BCG reactivation. The patient, after 3 months, received the 2nd dose of the vaccine and had no symptoms. It is believed that the reactivation of BCG occurs due to a cross-reaction mechanism between the individuals HSP, elicited as mediators of innate immunity against vaccine inflammation, with some epitopes of M. bovis. It is recommended that any immunosuppressive or autoimmune condition be investigated in patients that manifest BCG reactivation, especially in adults, in which Kawasaki disease is quite rare. Vaccines, including those against COVID-19, can also trigger of this immunological phenomenon still poorly understood.

Submaxilitis aguda bilateral asociada con listeriosis en un paciente inmunodeprimido / Acute bilateral submaxillitis associated to listeriosis in immunosuppressed patient

Acute bilateral submaxillitis is a rare event, except when it is caused by sialolithiasis. It has been described secondary to allergic, infectious, suppurative or viral processes, autoimmune such as Sjögren's syndrome, drugs such as thiopurines, nitrofurantoin, phenylbutazone, captopril, and after upper airway procedures such as upper endoscopy, orotracheal intubation bronchoscopy and ERCP (endoscopic retrograde cholangiopancreatography for choledocholithiasis). Treatment with tumour necrosis factor-alpha (TNF-alpha) antagonist drugs is associated with an increased risk of reactivation of intracellular bacterial infections, so that listeriosis has been described in pathologies that require such treatment, such as rheumatic, dermatological and intestinal diseases, which present other comorbidities or are immunocompromised. Listeriosis mainly causes bacteremia and meningitis, when symptomatic, and infects immunosuppressed persons, where it has a lethality despite 30% antibiotherapy. We present the clinical case of a male immunosuppressed patient, secondary to a treatment with azathioprine and prednisone followed by adalimumab, for indeterminate inflammatory bowel disease, superinfected by CMV, who after eating meat contaminated by Listeria monocytogenes, suffered a picture of listeriosis bacteremia, which improved with antibiotic treatment, followed by a transient acute bilateral submaxillitis, which subsided with symptomatic treatment (oral hydration). It is the only case described in the literature, in which an immunosuppressed patient treated with adalimumab, suffers from acute bilateral submaxillitis in the context of listeriosis, caused by the mumps virus.

La submaxilitis aguda bilateral es un evento raro, salvo cuando está causada por sialolitiasis. Se la ha descrito secundaria a procesos alérgicos, infecciosos, supurativos, virales o autoinmunes como el síndrome de Sjögren; a la administración de fármacos como tiopurinas, nitrofurantoina, fenilbutazona, captopril, y tras procedimientos sobre la vía aérea superior, como endoscopia digestiva alta, broncoscopia, intubación orotraqueal y colangiopancreatografía retrógrada endoscópica por coledocolitiasis (CPRE). El tratamiento con fármacos antagonistas del factor de necrosis tumoral alfa (TNF-alfa) se asocia con riesgo acentuado de reactivación de infecciones bacterianas intracelulares, de forma que se ha descrito la listeriosis en afecciones que requieren dicho tratamiento, como enfermedades reumáticas, dermatológicas y del intestino en sujetos que presentaban otras comorbilidades o estaban inmunocomprometidos. La listeriosis provoca bacteriemia y meningitis predominantemente, cuando es sintomática, e infecta a los sujetos inmunodeprimidos, en los que, a pesar de la antibioticoterapia, tiene una letalidad del 30%. Se presenta el caso clínico de un paciente varón, inmunodeprimido, secundario a tratamiento por azatioprina y prednisona seguido de adalimumab, por enfermedad inflamatoria intestinal indeterminada, sobreinfectada por citomegalovirus, que tras la ingesta de carne contaminada por Listeria monocytogenes, sufrió un cuadro de bacteriemia por listeriosis que mejoró con tratamiento con antibióticos, seguido de submaxilitis aguda bilateral transitoria, que cedió con tratamiento sintomático (hidratación oral). Es el único caso descrito en la literatura en el que un paciente inmunodeprimido tratado con adalimumab presenta submaxilitis aguda bilateral en el contexto de la listeriosis, provocada por el virus de la parotiditis.

Prevalência de lesão periapical radiolúcida nos pacientes com síndrome de Sjögren primária / Prevalence of radiolucid periapical injury in patients with primary Sjögren syndrome

Introdução: Pacientes com Síndrome de Sjögren Primária (SSP) apresentam maior incidência de cárie dentária. Como consequência da cárie, podem ocorrer lesões periapicais radiolúcidas (LPR). Objetivo: Determinar a prevalência de LPR nos pacientes com SSP e caracterizar radiologicamente os dentes associados às LPR. Métodos: Estudo transversal envolvendo pacientes com SSP, recrutados em uma Clínica de Reumatologia (HUCAM-ES), que foram submetidos, inicialmente, a um questionário socioeconômico e avaliação clínica do fluxo salivar. Posteriormente, radiografias foram obtidas por um sistema digital intrabucal, com posicionador para a técnica periapical do paralelismo. Realizou-se a estatística descritiva por meio da média e desvio-padrão para as variáveis quantitativas, e frequências absoluta e relativa para as variáveis qualitativas. Realizou-se o teste qui-quadrado com IC95% e o teste t de Student para comparação dos pacientes com e sem lesão periapical. Resultados: No total, 24 pacientes fizeram parte do estudo, todos do sexo feminino (20 - 71 anos). O número médio de dentes/paciente foi de 23,3 ± 3,1, com prevalência de LPR dentária de 7,86%. Do total de pacientes, 70,8% tinham pelo menos uma LPR. Na comparação dos dentes com e sem LPR, os dentes com LPR apresentaram maior porcentagem de canal tratado e cárie. Conclusão: De acordo com os resultados, pode-se concluir que as LPR encontradas nos pacientes com SSP são uma condição frequente, principalmente quando associadas a dentes com canal(is) obturado(s) (AU).

Introduction: Patients with Primary Sjögren Syndrome (PSS) have a higher incidence of dental caries. As a conse- quence, radiolucent periapical lesions (RPL) may appear. Objective: To determine the prevalence of RPL in patients with PSS and to radiologically characterize the teeth associated with RPL. Methodology: Cross-sectional study involving patients with PSS, recruited at the Rheumatology Clinic (HUCAM-ES), who were initially submitted to a socioeconomic questionnaire and clinical evaluation of salivary flow. Subsequently, radiographs were obtained by a digital intraoral system with positioner for the periapical parallelism technique. Descriptive statistics were calculated by the mean and standard deviation for quantitative variables, and absolute and relative frequencies for qualitative variables. Chi-square test with 95% CI and Student t test were applied to compare patients with and without periapical lesion. Results: Over-all, 24 patients participated in the study and all were females (20-71 years). The mean number of teeth per patient was 23.3±3.1, with prevalence of dental RPL of 7.86%. Among all patients, 70.8% had at least one RPL. When comparing teeth with and without RPL, teeth with RPL showed higher percentage of treated canal and caries. Conclusion: According to the results, it can be concluded that RPL in patients with PSS is a frequent condition, especially when associated with teeth with obturated root canals (AU).

Perfil clínico y epidemiológico de los pacientes con diagnóstico de neuromielitis óptica / Clinical and epidemiological profile of patients with neuromyelitis optica diagnosis

INTRODUCCIÓN. El trastorno del espectro de neuromielitis óptica, enfermedad inflamatoria, desmielinizante, afecta al sistema nervioso central, frecuente en poblaciones no caucásicas como la ecuatoriana. El retraso en su diagnóstico y tratamiento provoca discapacidad que se puede prevenir. OBJETIVO. Determinar el perfil clínico y epidemiológico de los pacientes con diagnóstico de trastorno del espectro de neuromielitis óptica. MATERIALES Y MÉTODOS. Estudio descriptivo transversal. Población de 45 Historias Clínicas y una muestra de 41 de pacientes con diagnóstico de trastorno del espectro de neuromielitis óptica atendidos en la Unidad de Neurología del Hospital de Especialida-des Carlos Andrade Marín, período enero 2005 a diciembre 2019. Se realizó análisis univarial. Se aplicó el programa estadístico International Business Machines Statistical Package for the Social Sciences, versión 25. RESULTADOS. El 76,0% (31; 41) fueron mujeres. Datos promedios: edad 48,9 años; diagnóstico definitivo demoró 4,12 años, desde el inicio de los síntomas; tiempo de diagnóstico fue 3,17 años; 3,7 brotes en total; el 87,8% (36; 41) con un fenotipo recurrente. La media de duración de la enfermedad fue de 6,8 años. En el 70,7% (29; 41), se identificaron anticuerpos anti-AQP4 en suero mediante inmunofluorescencia directa, el 51,2% requirieron para la marcha apoyo uni o bilateral. El 43,9% (18; 41) debutó con neuritis óptica; el 31,7% (13; 41) presentaron mielitis como primer síntoma y el 24,4% (10; 41) la combinación de neuritis óptica y mielitis fueron los síntomas iniciales. CONCLUSIÓN. Se determinó el perfil clínico y epi-demiológico de los pacientes con diagnóstico de trastorno del espectro de neuromielitis óptica. Existió demora en el diagnóstico definitivo de los pacientes desde el inicio de los síntomas, lo que se tradujo en un aumento de la discapacidad.

INTRODUCTION. Neuromyelitis optica spectrum disorder, an inflammatory, demyelinating disease, affects the central nervous system, common in non-Caucasian popu-lations such as Ecuadorians. The delay in its diagnosis and treatment causes disabi-lity that can be prevented. OBJECTIVE. To determine the clinical and epidemiological profile of patients diagnosed with neuromyelitis optica spectrum disorder. MATERIALS AND METHODS. Cross-sectional descriptive study. Population of 45 Medical Records and a sample of 41 patients with a diagnosis of neuromyelitis optica spectrum disor-der seen at the Neurology Unit of the Carlos Andrade Marín Specialties Hospital, period from January 2005 to December 2019. Univariate analysis was performed. The statistical program International Business Machines Statistical Package for the Social Sciences, version 25 was used. RESULTS. 76,0% (31; 41) were women. Average data: age 48,9 years; definitive diagnosis took 4,12 years from the onset of symptoms; time to diagnosis was 3,17 years; 3,7 outbreaks in total; 87,8% (36; 41) with a recurrent phenotype. The average disease duration was 6,8 years. In 70,7% (29; 41), anti-AQP4 antibodies were identified in serum by direct immunofluorescence, 51,2% required uni- or bilateral su-pport for walking. Optic neuritis started in 43,9% (18; 41); 31,7% (13; 41) had myelitis as the first symptom and 24,4% (10; 41) the combination of optic neuritis and myelitis were the initial symptoms. CONCLUSION. The clinical and epidemiological profile of patients diagnosed with neuromyelitis optica spectrum disorder was determined. There was delay in the conclusive diagnosis of patients from the beginning of symptoms, which resulted in increased disability.

Expresión clínica del Síndrome de Sjögren primario en pacientes adultos diagnosticados a edad menor o igual a 35 años versus mayores de 35 años / Clinical expression of primary Sjögren's syndrome in adult patients diagnosed at an age less than or equal to 35 years versus those older than 35 years

Objetivo: Describir y comparar las manifestaciones clínicas en pacientes adultos diagnosticados con Síndrome de Sjögren primario (SSp) a edad menor o igual a 35 años versus mayores a 35 años. Materiales y métodos: Se incluyeron pacientes mayores de 18 años de edad, con diagnóstico de SSp de acuerdo a los criterios de clasificación ACR - EULAR 2002/2016, registrados en la base de datos GESSAR (Grupo de Estudio Síndrome de Sjögren Sociedad Argentina de Reumatología). Resultados: Se incluyeron 665 pacientes. Cien (15,04%) con edad al diagnóstico ≤ 35 años, 92% mujeres. El promedio de edad del grupo > 35 años, fue de 54 + 11 años, 96% mujeres. Se encontraron diferencias estadísticamente significativas entre < 35 años vs > 35 años, en xeroftalmia (90,72% vs 95,64%, p: 0,04) y xerodermia (42,35% vs 57,36%, p: 0,03) y en los siguientes dominios del ESSDAI (EULAR Activity Index for primary Sjögren's syndrome): sistema nervioso periférico (4,05 vs 11,32, p: 0,03), respiratorio (6% vs 15,40%, p: 0,01) y renal (6% vs 1,59%, p: 0,02). Conclusión: Nuestro estudio sugiere un menor compromiso glandular en pacientes con SSp diagnosticados a menor edad, sin un patrón diferencial característico en cuanto al compromiso sistémico.

Objective: To describe and compare the clinical manifestations, in adult patients diagnosed with primary Sjögren's Syndrome at age less than or equal to 35 years versus those over 35 years of age. Materials and Methods: We analyzed the data of patients older than 18 years, with diagnosis of primary Sjögren's syndrome (American - European criteria 2002), included in the GESSAR database (Sjögren Syndrome Study Group of the Argentine Society of Rheumatology). Results: 665 patients were included. One hundred of them with an age at diagnosis less than or equal to 35 years and with a mean age at diagnosis of 29 + 4 years, 92% of them women. The average age at diagnosis of the group over 35 years was 54 + 11 years, 96% women. Statistically significant differences were found between less than or equal to 35 years vs over 35 years, in xerophthalmia (90.72% vs 95.64%, p: 0.04) and xeroderma (42.35% vs 57.36% , p: 0.03), and in the following domains of ESSDAI (EULAR Activity Index for primary Sjögren's syndrome): peripheral nervous system (4.05 vs 11.32, p: 0.03), respiratory (6% vs 15.40%, p: 0.01) and renal (6% vs 1.59%, p: 0.02). Conclusion: Our study suggests less glandular involvement in patients with pSS diagnosed at a younger age, without a characteristic differential pattern regarding systemic involvement.

Síndrome de Sjögren en un adolescente: caso Clínico / Sjögren syndrome. report of one case

Sjögren Syndrome (SS), a slowly Progressive disease that has unified, validated diagnostic criteria, with excellent evidence and performance in adults, but not in adolescents. We report a 17 year old teenager with a family history of SS in his sister and mother. He presented with fever and fatigue. He had an elevated C reactive protein, leukopenia, positive antinuclear and anti-Rho antibodies and rheumatoid factor. A scintigraphy showed a severe salivary gland dysfunction. The syndrome in this patient had a pediatric clinical behavior despite his proximity to adulthood.

Perspectivas da saúde orofacial em portadores de doenças reumáticas: uma revisão da literatura / Perspectives of orofacial health in people with rheumatic diseases: a literature review

Este estudo objetivou verificar as principais manifestações bucofaciais que as doenças reumáticas podem causar. Foram selecionadas, assim, 05 doenças reumáticas: Artrite Reumatóide (AR), Síndrome de Sjogren (SS), Lúpus Eritematoso Sistêmico (LES), Esclerose Sistêmica (ES) e a Síndrome de Behçet (SB). Estas, por sua vez, foram escolhidas por apresentarem como sinais e sintomas clínicos problemas orofaciais. Foi elaborado, dessa maneira, uma revisão bibliográfica de trabalhos com vintênio de 2000 a 2020 nas seguintes bases: LILACS, MEDLINE e SCIELO. Propõe-se, portanto, a introdução de um Cirurgião-dentista na equipe multidisciplinar de Reumatologia para diagnosticar e tratar as especificidades bucofaciais que acometem os portadores de problemas reumáticos... (AU)

This study aimed to verify as main bucofacial manifestations that rheumatic diseases can cause. Thus, 05 rheumatic diseases were selected: Rheumatoid Arthritis (RA), Sjogren's Syndrome (SS), Systemic Lupus Erythematosus (SLE), Systemic Sclerosis (ES) and Behçet's Syndrome (SB). These, in turn, were chosenbecause they present as orofacial clinical signs and symptoms. In this way, a bibliographic review of works with twenty years from 2000 to 2020 was prepared on the following bases: LILACS, MEDLINE and SCIELO. Therefore, it is proposed to introduce a dental surgeon in the multidisciplinary team of Rheumatology to diagnose and treat as orofacial specificities that affect patients with rheumatic problems... (AU)

Lacrimal gland atrophy and dry eye related to isotretinoin, androgen, and prolactin: differential diagnosis for Sjögren's syndrome / Atrofia das glândulas lacrimais e olho seco relacionados aisotretinoína, androgênio e prolactina: diagnóstico diferencial com a síndrome de Sjögren

ABSTRACT This report is of three cases of sicca syndrome, initially suspected to be Sjögren's syndrome, which was ruled out by clinical and laboratory investigations. The patients were a 24-year-old woman, a 32-year-old man, and a 77-year-old woman with chronic symptoms of sicca syndrome, including dry eye syndrome. The first case was associated with the use of isotretinoin, a retinoic acid. The second was associated with the use of anabolic androgenic steroids, and the third was related to a prolactin- secreting pituitary adenoma. All cases manifested sicca, including dry eye syndrome, after those events, and the manifestations persisted. Magnetic resonance imaging revealed bilateral atrophy of the lacrimal gland. The medical history, ocular examinations, laboratory exams, and magnetic resonance images confirmed dry eye syndrome; however, the exams were all negative for Sjögren's syndrome. The lacrimal gland was absent on magnetic resonance imaging in all three cases. The clinical history revealed that the signs and symptoms appeared after chronic exposure to retinoic acid, anabolic androgenic steroids, and a prolactin-secreting pituitary adenoma, respectively. Chronic isotretinoin, anabolic androgenic steroids, and prolactin-secreting pituitary adenoma or, in this last case, its inhibitory treatment, can cause lacrimal gland atrophy, sicca syndrome, and dry eye syndrome, and a differential diagnosis of Sjögren's syndrome. Further studies on doses, time, and other susceptibilities to the long-lasting adverse effects of retinoic acid, anabolic androgenic steroids, and the repercussions of prolactin-secreting pituitary adenoma are necessary to confirm and expand upon these associations.

RESUMO O relato descreve três casos de síndrome de sicca, inicialmente suspeitos de serem a síndrome de Sjögren, que fo­ram negados pela investigação clínica e laboratorial. O primeiro associado ao uso de isotretinoína, um ácido retinóico, o segundo ao uso de esteroides androgênicos anabolizantes e o terceiro relacionado ao adenoma da hipófise secretora da prolactina, todos manifestaram sicca, incluindo a síndrome do olho seco após esses eventos e as manifestações persistem. A ressonância magnética revelou atrofia bilateral da glândula lacrimal. Eles eram uma mulher de 24 anos, um homem de 32 anos e uma mulher de 77 anos com sintomas crônicos da síndrome de sicca, incluindo a síndrome do olho seco. A história médica, o exame ocular, os exames laboratoriais e a ressonância magnética foram confirmados como síndrome do olho seco, no entanto, todos os exames foram negativos para a síndrome de Sjögren. A glândula lacrimal estava ausente na ressonância magnética nos três casos. A história clínica revelou que sinais e sintomas se manifestaram após exposição crônica ao ácido retinóico, esteróides anabolizantes androgênicos e adenoma secretivo da prolactina hipofisária, respectivamente. Isotretinoína crônica, esteroides anabólicos androgênicos e adenoma hipofisário secretor de prolactina ou, neste último caso, seu tratamento inibitório pode ser a causa da atrofia da glândula lacrimal, síndrome da sicca e síndrome do olho seco e diagnóstico diferencial da síndrome de Sjögren. Estudos adicionais sobre doses, duração e outras suscetibilidades aos efeitos adversos duradouros do ácido retinóico, esteroides androgênicos anabólicos e repercussões do adenoma da hipófise secretora da prolactina são necessários para confirmar e detalhar essas associações.

Abdominal symptoms during Sjogren's syndrome: a pilot study

Abstract Background: Abdominal symptoms in patients with primary Sjögren syndrome (pSS) are poorly documented. The objective of the study was to describe the abdominal symptoms of patients with pSS and to assess their association with characteristics of the disease. Methods: One hundred and fifty patients with pSS were evaluated using a composite global symptom score for abdominal symptoms and their severity. Data concerning the clinical and biological characteristics of pSS and abdominal disorders were also collected. Results: Of the patients with pSS, 95% suffered from abdominal symptoms (median global symptom score 7.5 ±5.5 points out of 30). More than half of the patients experienced abdominal tension (68%), upper abdominal pain (54%), abdominal discomfort (58%) and/or constipation (54%). Regarding the pSS activity, in relation to European League Against Rheumatism (EULAR) Sjogren syndrome disease activity index score items, general and central nervous system involvement wereassociated with a high global symptom score. The EULAR Sjogren Syndrome Patient Reported Index (ESSPRI) symptom score was positively correlated with the global symptom score (p < 0.01). Multivariate analysis showed a significant association between a high global symptom score and SSA seronegativity, gastroparesis, and ESSPRI score (p < 0.01 for each). Conclusions: The majority of patients with pSS suffered abdominal symptoms. There is currently no therapeutic recommendation because of the lack of information on the underlying pathophysiological mechanisms. Trial registration: NCT03157011. Date of registration: July 17, 2017.(AU)

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

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

El síndrome de Sjögren y su influencia en la calidad de vida / Sjögren's syndrome and its influence on quality of life

INTRODUCCIÓN: las afecciones reumáticas constituyen un grupo de enfermedades que además de afectar el aparato locomotor, también afectan otros órganos y sistemas, lo que genera discapacidad funcional y disminución de la percepción de calidad de vida; dentro de estas afecciones se incluye el síndrome de Sjögren. OBJETIVO: determinar la percepción en la calidad de vida en pacientes con síndrome de Sjögren. METODOLOGÍA: estudio descriptivo, retrospectivo y con enfoque mixto; universo de 457 pacientes con diagnóstico de síndrome de Sjögren. Muestra constituida por 417 pacientes, se aplicó el cuestionario de láminas Coop/Wonca para determinar la percepción de calidad de vida y la versión cubana del Health Assessment Questionnaire para determinar capacidad funcional, a la vez también se utilizó el coeficiente de correlación de Pearson para determinar asociación entre las variables del estudio. RESULTADOS: La puntuación de percepción en la calidad de vida global fue de 21,78% y las dimensiones más afectadas fueron el dolor, las actividades sociales y cotidianas; siendo diferentes entre ambos sexos. CONCLUSIÓN: existió correlación positiva notoria entre la presencia de discapacidad funcional y las puntuaciones de calidad de vida relacionada con la salud; mientras mayor fue las puntuaciones de discapacidad peor fue la percepción de calidad de vida de los pacientes.

INTRODUCTION: rheumatic affections constitute a group of diseases that, in addition to affecting the locomotor system, also affect other organs and organ systems, which generates functional disability and a decrease in the perception of quality of life; these conditions include Sjögren's syndrome. MATERIALS AND METHODS: descriptive, retrospective study with a mixed approach; universe of 457 patients diagnosed with Sjögren's syndrome. Sample made up of 417 patients, the Coop / Wonca slide questionnaire was applied to determine the perception of quality of life and the Cuban version of the Health Assessment Questionnaire to determine functional capacity. Pearson's correlation coefficient was used to determine association between the study variables. RESULTs: the perception score in the global quality of life was 21.78% and the most affected dimensions were pain, social and daily activities; being different between both sexes. CONCLUSIONS: there was a strong positive correlation between the presence of functional disability and health-related quality of life scores; the higher the disability scores, the worse the perception of quality of life of the patients.

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.

Disfunción temporomandibular en pacientes con síndrome de Sjögren / Temporomandibular dysfunction in patients with Sjögren's syndrome

Introducción: El síndrome de Sjögren es una enfermedad reumática, inflamatoria y crónica caracterizada por dolor articular y resequedad de las mucosas. La articulación temporomandibular (ATM) y las glándulas parótidas y salivales suelen estar afectadas. Objetivo: Determinar la prevalencia de la afectación de la articulación temporomandibular en pacientes con síndrome de Sjögren. Métodos: Estudio básico, no experimental, descriptivo y de corte transversal que incluyó a 78 pacientes con diagnóstico de síndrome de Sjögren atendidos en la Unidad Móvil número 2 de Alausi entre 2017 y 2019. Para identificar la afectación de la ATM se realizaron ecosonografías. Se usó la prueba de correlación de Pearson para establecer la relación entre las características de la enfermedad y la presencia de afectación articular. Resultados: El promedio de edad fue de 46,18 años. Predominaron los pacientes entre 40 y 60 años (62,82 por ciento), el sexo femenino (85,90 por ciento), y el tiempo de evolución de la enfermedad entre 3 y 5 años (50,00 por ciento). El 79,49 por ciento presentó síndrome de Sjögren secundario, y la artritis reumatoide fue la causa más frecuente (54,84 por ciento). El 65,38 por ciento tenía algún tipo de afectación de la ATM, y el hallazgo más común fue la disminución del espacio articular (56,86 por ciento). Conclusiones: Existió una elevada prevalencia de afectación de la ATM en los pacientes con síndrome de Sjögren, y el hallazgo principal fue la disminución del espacio articular. Se encontró una correlación positiva media entre el tiempo de evolución de la enfermedad y la presencia de afectación de la ATM(AU)

Introduction: Sjögren's syndrome is a rheumatic, inflammatory and chronic disease characterized by the presence of joint pain and dryness of the mucous membranes. The temporomandibular joint and the parotid and salivary glands usually have a high frequency of involvement. Objective: To determine the prevalence of temporomandibular involvement in patients with Sjögren's syndrome. Methods: Descriptive, correlational and explanatory study that included 78 patients diagnosed with Sjögren's syndrome treated in the mobile unit number 2 of Alausi. Echocardiography of the temporomandibular joints was performed to identify the involvement of the joint. Pearson's correlation test was used to establish a relationship between the characteristics of the disease and the presence of joint involvement. Results: Average age of 46.18 years with a predominance of patients between 40 and 60 years of age (62.82 percent), of the female sex (85.90 percent) and with time of evolution of the disease between 3 and 5 years (50.00 percent). 79.49 percent of the cases presented secondary Sjögren's syndrome, rheumatoid arthritis being the most frequent disease (54.84 percent). 65.38 percent of the patients presented some type of involvement of the temporomandibular joint, with the decrease in joint space being the most frequent finding (56.86 percent). Conclusions: There was a high prevalence of temporomandibular joint involvement in patients with Sjögren's syndrome, with the decrease in joint space being the finding that was most frequently identified. A positive average correlation was found between the time of evolution of the disease and the presence of temporomandibular joint involvement(AU)