Sjögren's syndrome combined with cold agglutinin disease: A case report / 北京大学学报(医学版)

Sjögren's syndrome(SS)is a chronic autoimmune disease that affects exocrine glands, especially salivary and lacrimal glands. The main clinical manifestations are dry mouth and dry eyes, but also multi-organ and multi-system can be involved. Cold agglutinin disease(CAD)is an autoimmune disease characterized by red blood cell agglutination in the blood vessels of extremities caused by cold agglutinin at low temperature, resulting in skin microcirculation disturbance, or hemolytic anemia. Cold agglutinin disease is divided into two categories, primary cold agglutinin disease and secondary cold agglutinin disease. Primary cold agglutinin disease is characterized with cold agglutinin titer of 1 ∶4 000 or more and positive Coomb's test. However, the Coomb's test is not necessarily positive and the cold agglutinin titer is between 1 ∶32 and 1 ∶4 000 in secondary cold agglutinin disease. Here, we reported an elderly patient admitted to hospital due to fever. He was diagnosed with respiratory infection, but he showed incompletely response to the anti-infection treatment. Further laboratory tests showed the patient with positive ANA and anti-SSA antibodies. Additionally, the patient complained that he had dry mouth and dry eyes for 1 year. Schirmer test and salivate gland imaging finally confirmed the diagnosis Sjogren's syndrome. During the hospital stay, the blood clots were found in the anticoagulant tubes. Hemolytic anemia was considered as the patient had anemia with elevated reticulocytes and indirect bilirubin. In addition, further examination showed positive cold agglutination test with a titer of 1 ∶1 024, and cold agglutinin disease was an important type of cold-resistant autoimmune hemolytic anemia. Furthermore, the patient developed cyanosis after ice incubating at the tip of the nose. Hence, the patient was diagnosed as CAD and he was successfully treated with glucocorticoids instead of anti-infection treatments. Hence, the patient was diagnosed with SS combined with secondary CAD. SS combined CAD are rarely reported, and they are both autoimmune diseases. The abnormal function of B lymphocytes and the production of autoantibodies might be the common pathogenesis of them. Cold agglutinin disease can lead to severe hemolytic anemia, even life-threatening. In clinical practice, timely recognizing and dealing with CAD might promote the prognosis of the patient.

Early prediction of severe COVID-19 in patients with Sjögren's syndrome / 北京大学学报(医学版)

OBJECTIVE@#To investigate the predictive value of blood cell ratios and inflammatory markers for adverse prognosis in patients with primary Sjögren's syndrome (PSS) combined with coronavirus disease 2019 (COVID-19).@*METHODS@#We retrospectively collected clinical data from 80 patients with PSS and COVID-19 who visited the Rheumatology and Immunology Department of the First Affiliated Hospital of Nanchang University from December 2022 to February 2023. Inclusion criteria were (1) meeting the American College of Rheumatology (ACR) classification criteria for Sjögren's syndrome; (2) confirmed diagnosis of COVID-19 by real-time reverse transcription polymerase chain reaction or antigen testing for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2); (3) availability of necessary clinical data; (4) age > 18 years. According to the clinical classification criteria of the "Diagnosis and Treatment Protocol for Novel Coronavirus Pneumonia (trial the 10th Revised Edition)", the patients were divided into the mild and severe groups. Disease activity in primary Sjögren' s syndrome was assessed using the European League Against Rheumatism (EULAR) Sjögren' s syndrome disease activity index (ESSDAI). Platelet-lymphocyte ratio (PLR), C-reactive protein-lymphocyte ratio (CLR), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and other laboratory data were compared between the two groups within 24-72 hours post-infection.@*RESULTS@#The mild group consisted of 66 cases with an average age of (51. 52±13. 16) years, and the severe group consisted of 14 cases with an average age of (52.64±10.20) years. Disease activity, CRP, platelets, PLR, and CLR were significantly higher in the severe group compared with the mild group (P < 0.05). Univariate analysis using age, disease activity, CRP, platelets, PLR, and CLR as independent variables indicated that disease activity, CRP, PLR, and CLR were correlated with the severity of COVID-19 (P < 0.05). Multivariate logistic regression analysis further confirmed that PLR (OR=1.016, P < 0.05) and CLR (OR=1.504, P < 0.05) were independent risk factors for the severity of COVID-19 in the critically ill patients. Receiver operator characteristic (ROC) curve analysis showed that the area under the curve (AUC) for PLR and CLR was 0.708 (95%CI: 0.588-0.828) and 0.725 (95%CI: 0.578-0.871), respectively. The sensitivity for PLR and CLR was 0.429 and 0.803, respectively, while the highest specificity was 0.714 and 0.758, respectively. The optimal cutoff values for PLR and CLR were 166.214 and 0.870, respectively.@*CONCLUSION@#PLR and CLR, particularly the latter, may serve as simple and effective indicators for predicting the prognosis of patients with PSS and COVID-19.

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.

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.

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)

Salivary rheumatoid factor in primary and secondary Sjögren's syndrome / Factor reumatoide salival en síndrome de Sjögren primario y secundario

Sjögren's syndrome is a chronic autoimmune disease, characterized by the presence of hyposalivation and xerophthalmia, which in addition to other factors is diagnosed by the presence of rheumatoid factor in blood. The objective of the present study is to evaluate the presence of rheumatoid factor (IgG-IgM) in the saliva of patients with primary and secondary Sjögren's syndrome. Materials and methods: Forty samples from patients with primary and secondary Sjögren's syndrome previously diagnosed by the Arthritis and Rheumatism Specialist Center of the Autonomous University of Nuevo Leon were analyzed. Samples were taken from the saliva using the Carlson-Crittenden device to evaluate the IgG-IgM immunocomplex using the ELISA method. Results: No significant difference was found between the presence of IgM in primary (0.099±0.016) and secondary Sjögren syndrome (0.098±0.017), however, a high presence of IgG was found in the group of patients with secondary Sjögren's syndrome (0.134±0.054). Conclusion: The search for diagnostic tools using salivary biomarkers has come with economic and clinical advantages, however, in the present study no significant changes were found in salivary rheumatoid factor between both groups.

El síndrome de Sjögren es una enfermedad autoinmune crónica, caracterizada por la presencia de hiposalivación y xeroftalmia, la cual además de otros factores es diagnosticada por la presencia del factor reumatoide en sangre. El objetivo del presente estudio es evaluar la presencia del factor reumatoide (IgG-IgM) en saliva parotídea de pacientes con síndrome de Sjögren primario y secundario. Materiales y métodos: Se analizaron 40 muestras de pacientes con síndrome de Sjögren primario y secundario previamente diagnosticados por el Centro de Especialistas en Artritis y Reumatismo de la Universidad Autónoma de Nuevo León, a los cuales se les tomó una muestra de saliva parotídea mediante el dispositivo Carlson-Crittenden para evaluar mediante el método ELISA el inmunocomplejo IgG-IgM. Resultados: No se encontró diferencia significativa entre la presencia de IgM en el síndrome de Sjögren primario (0.099±0.016) y secundario (0,098±0,017), sin embargo en cuanto a la presencia de la IgG se encontró elevada en el grupo de pacientes con síndrome de Sjögren secundario (0,134±0,054). Conclusión: La búsqueda de herramientas diagnósticas mediante biomarcadores salivales ha traído consigo ventajas económicas y clínicas, sin embargo en el presente estudio no se encontró un cambio significativo en el factor reumatoide salival entre ambos grupos.

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.

The evaluation of the Myxovirus Resistance 1 protein in serum and saliva to monitor disease activation in primary Sjögren's syndrome

OBJECTIVE: Primary Sjögren's syndrome (pSjS) is a chronic autoimmune disease that causes dry eye and mouth. No laboratory parameters to monitor the activation of this disease have been identified. Therefore, any possible relationships between salivary and blood myxovirus resistance 1 (MX1) and pSjS must be prospectively studied. METHODS: Thirty female patients with pSjS, 30 women with rheumatoid arthritis (RA) without secondary Sjögren's syndrome (SjS) and 28 healthy control women were enrolled in this investigation. Analyses of MX1 by the enzyme-linked immunosorbent assay (ELISA) method, SS-A (Ro) and SS-B (La) tests by the strip immunoblot method, anti-nuclear antibody (ANA) tests by immunofluorescence and the measurement of serum rheumatoid factor (RF), C3, C4, immunoglobulin A (IgA), immunoglobulin M (IgM), and immunoglobulin G (IgG) were performed. RESULTS: The serum level of MX1 in patients without Raynaud phenomenon was higher than in those with Raynaud phenomenon (p:0.029, p<0.05, statistically significant). There was a statistically significant positive association between hemoglobin levels and MX1 serum levels. No statistically significant association was found among the other parameters. Low MX1 levels were shown to be associated with both a low disease activity score based on the European League Against Rheumatism (EULAR) Sjögren's Syndrome Disease Activity Index (ESSDAI) and hydroxychloroquine use in all patients. CONCLUSION: MX1 levels have a considerable impact on the assessment of the disease activity in SjS. We believe that more-comprehensive studies should be performed on patients with pSjS who do not use hydroxychloroquine to prove this relationship and that MX1 levels should be used as a routine marker for the assessment of pSjS disease activity. Further studies are needed to create awareness of the role that MX1 has in the diagnosis of pSjS, which may help to uncover novel pathways for new therapeutic modalities.

Diagnostic potential of saliva proteome analysis: a review and guide to clinical practice

Abstract: Proteomic techniques have become popular in medicine and dentistry because of their widespread use in analyzing bodily fluids such as blood, saliva, urine, and gingival crevicular fluids as well as hard tissues such as enamel, dentine, and cementum. This review is a guide to proteomic techniques in general dentistry, summarizing techniques and their clinical application in understanding and diagnosing diseases and their use in identifying biomarkers of various diseases.

Combinación poco habitual de hepatocarcinoma con síndrome de Sjögren / Uncommon combination of hepatocarcinoma with Sjögren's syndrome

El síndrome de Sjögren es una enfermedad reumática que aumenta el riesgo de padecer enfermedades malignas. Dentro de estas se identifican a los linfomas como las que con mayor frecuencia se presentan. El hepatocarcinoma es una de las enfermedades más agresivas y su incidencia ha ido en aumento asociado al alto consumo de alcohol. Se presenta el caso de una paciente de 56 años de edad con diagnóstico de un síndrome de Sjögren, con manifestaciones clínicas y anátomopatológicas que permiten llegar al diagnóstico de un hepatocarcinoma, asociación muy infrecuente. El hepatocarcinoma provoca un deterioro progresivo del estado de salud de los pacientes. Conocer sus síntomas y signos es de vital importancia con el fin de llegar al diagnóstico precoz de la enfermedad y evitar así las complicaciones que de él se derivan. No se encuentra relación etiopatogénica entre estas dos afecciones(AU)

Sjögren's syndrome is a rheumatic disease that increases the risk of suffering from malignant diseases. Lymphomas are identified among them as most frequent. Hepatocarcinoma is one of the most aggressive conditions and the incidence has been increasing associated with high alcohol consumption. We present the case of a 56-year-old patient with diagnosis of Sjögren's syndrome that presents clinical and anatomo-pathological manifestations allowing the diagnosis of a very rare hepatocarcinoma. This entity causes a progressive deterioration of the patient health status. It is of vital importance knowing the symptoms and signs in order to reach early diagnosis, thus avoid complications. There is no etiopathogenic relationship between these two conditions(AU)

Comportamiento clínico-oftalmológico del síndrome de ojo seco / Clinical-ophthalmologic behavior of the dry eye syndrome

Introducción: el síndrome de ojo seco o queratoconjuntivitis seca, constituye uno de los trastornos oculares de mayor frecuencia en la actualidad. Objetivo: evaluar el comportamiento clínico del síndrome de ojo seco en la consulta de córnea. Material y Métodos: se realizó un estudio observacional, descriptivo, prospectivo en 103 pacientes con diagnóstico de síndrome de ojo seco que acudieron al Servicio de Córnea del Hospital Universitario Clínico Quirúrgico Comandante Faustino Pérez, de Matanzas, en el período comprendido entre noviembre de 2013 a noviembre de 2016. Se analizaron las variables clínico-epidemiológicas, examen oftalmológico, test de Schirmer y BUT. Resultados: el grupo etáreo que predominó fue el de 60 a 69 años y el género femenino. La medicación sistémica fue el factor de riesgo que en mayor porciento se presentó. El síntoma predominante fue la sequedad ocular, mientras que la disminución del menisco lagrimal estuvo presente en gran parte de los pacientes. Tanto el BUT, como el test de Schirmer se presentaron alterados en el 81,5 y 65% de los investigados respectivamente. Conclusiones: el síndrome de ojo seco es una enfermedad frecuente en la práctica oftalmológica, el reconocimiento de sus elementos clínicos contribuye al diagnóstico preciso de la misma (AU).

Introduction: the dry eye syndrome or dry keratoconjunctivitis is, nowadays, one of the most frequent ocular disorders. Objective: to assess the clinical behavior of the dry eye syndrome in the cornea consultation. Materials and methods: a prospective, descriptive, observational study was carried out in 103 patients with diagnosis of dry eye syndrome who came to the Service of Cornea of the Clinical Surgical University Hospital "Comandante Faustino Pérez", of Matanzas, between November 2013 and November 2016. The clinical epidemiological variables, the ophthalmologic examination, the Schirmer´s test and BUT were analyzed. Results: the age group that predominated was the 60-69 years group, and female gender. Systematic medication was the risk factor showing higher percent. The predominant symptom was eye dryness, while the reduction of the lachrymal meniscus was present in most of the patients. The BUT as much as the Schirmer´s test were altered in 81.5 % and 65 % of the studied persons respectively. Conclusions: the dry eye syndrome is a disease frequently found in the ophthalmological practice; the recognition of its clinical elements contributes to its precise diagnosis (AU).

Prótesis total con reservorio salival en un paciente con síndrome de Sjögren / Complete Denture with Salivary Reservoir in a Patient with Sjogren's Syndrome

Antecedentes: El síndrome de Sjögren es una enfermedad autoinmune que empieza a manifestarse hacia los 45 años de edad y cuya incidencia aumenta con la edad. La sintomatología en la cavidad oral se caracteriza por boca seca o xerostomía. Gran cantidad de pacientes que la padecen son portadores de prótesis totales debido a la pérdida dental en edades avanzadas. Objetivo: Describir el caso de un paciente con síndrome de Sjögren a quien se le elaboró una prótesis con reservorio salival para disminuir síntomas orales. Métodos: Se realizaron ensayos de laboratorio para elaborar los reservorios buscando resistencia estructural de la prótesis; facilidad de colocación y retiro de las tapas de los reservorios para permitir la limpieza de los depósitos; estética y que contuvieran la mayor cantidad posible de saliva artificial. Los diseños se probaron en el paciente. Resultados: Después de realizar ensayos de prueba de la prótesis en ambos maxilares, el paciente comentó sobre la facilidad de retiro de las tapas, ausencia de nauseas, fonética normal, higiene adecuada, comodidad, solución parcial al problema de xerostomía y duración aproximada de tres horas una vez llenos los reservorios. Conclusión: Los prototipos finales proporcionaron mejores condiciones de los reservorios y aumentaron la cantidad de saliva. Se recomienda realizar estudios clínicos controlados para confirmar los hallazgos de este caso.

Background: Sjögren's syndrome is an autoimmune disease that manifests after the age of 45 years and whose incidence increases with age. Symptoms in the oral cavity includes dry mouth or xerostomia. Large number of Sjögren's patients wear complete dentures due to tooth loss at advanced ages. Objective: To describe the case of a Sjögren's patient for whom dentures with salivary reservoirs were made to reduce oral symptoms. Methods: Lab tests were conducted to find an optimum denture design with reservoirs. Requirements included: structural resistance, easy placement and removal of reservoir caps to allow cleaning, good esthetics, and containing the greatest amount of artificial saliva. Models were tried in the patient. Results: Several models were tried for both jaws. At the end, the patient reported about easiness to remove caps, absence of nausea, normal phonetics, adequate hygiene, comfort, and partial solution to the problem of xerostomia with an approximate duration of three hours once the reservoir was full. Conclusion: Final prototypes provided the best characteristics of reservoirs and stored the highest amounts of saliva. It is important to conduct clinical controlled trials to confirm the findings of this case.

Formes familiales du syndrome de Gougerot-Sjögren : étude de 22 familles multiplex

Introduction : Les facteurs génétiques du SGS, outre les modèles animaux, sont déterminés par le biais de leurs formes familiales. L'objectif de ce travail était d'étudier les aspects phénotypiques des formes familiales du SGS. Patients et méthodes : Etude réalisée dans le service de Rhumatologie du CHU Aristide Le Dantec de Dakar entre Janvier 2013 et Mars 2016, où nous avons colligé les observations de familles multiplex de SGS répondant aux critères de consensus de 2002.Résultats : Vingt-deux familles ont été colligées à partir de 22 propositus (17 femmes et 5 hommes), d'âge moyen de 31,5 ans au début apparent de la maladie. Le SGS chez ces propositus était primitif dans 8 cas et secondaire à une PR dans 14 cas. Les familles totalisaient 921 membres. Soixante- quinze (54 femmes et 21 hommes), y compris les cas index présentaient un SGS (54 primitifs et 21 secondaires), soit une prévalence de 8,14 %. Les apparentés de premiers degré atteints étaient au nombre de 46 (85%). Les autres maladies auto- immunes associées étaient une PR (16 cas), un lupus systémique (1 cas), une polymyosite (1 cas), une sclérodermie systémique (1 cas), un vitiligo (1 cas) et une maladie de Basedow (1 cas). Les autres affections répertoriées étaient : lymphome oculaire (1 cas), cancer du col de l'utérus (1 cas). L'évolution sous traitement fut favorable, sauf chez 1 cas décédé. Conclusion : Le caractère familial du SGS chez nos malades plaide en faveur de l'implication de facteurs génétiques dans le déterminisme de la maladie

Manifestaciones bucales del síndrome de Sjögren. Presentación de un caso / Oral manifestations of the Sjögren's syndrome. Presentation of a case

El síndrome de Sjögren es una enfermedad autoinmunitaria, caracterizada por la infiltración linfoplasmocitaria de las glándulas exocrinas con destrucción epitelial, provocando un síndrome seco. El objetivo de esta presentación de caso fue profundizar en el conocimiento de la enfermedad, ya que constituye la clave para su diagnóstico oportuno. Se presentó a una paciente femenina de 54 años, con antecedentes de lupus eritematoso sistémico. El interrogatorio, el examen clínico general y bucal, los estudios de laboratorio e histológico, así como la sospecha clínica de la enfermedad, permitieron realizar el diagnóstico del síndrome de Sjögren asociado a lupus eritematoso sistémico. En esta paciente concurrieron las siguientes manifestaciones bucales: xerostomía, halitosis, sensación de sed constante, dificultades para la deglución y para hablar, labios secos y pálidos, lengua depapilada, ardor bucal, intolerancia al uso de la prótesis, queilitis angular y candidiasis bucal. El manejo de la enfermedad tiene un enfoque multidisciplinario; el estomatólogo cumple un papel esencial en su diagnóstico y tratamiento, lo cual permite mejorar la calidad de vida de estos pacientes.

The Sjögren’s syndrome is an auto-immune disease, characterized by the limpho-plasmocytic infiltration of the exocrine glands with epithelial destruction, provoking a dry syndrome. The objective of this case presentation was deepening in this disease knowledge, because it is the key for its opportune diagnosis. We presented a female patient, aged 54 years, with antecedents of systemic lupus erythematosus. The anamnesis, oral and general examination, histological and laboratory studies, and also the clinical suspicion, allowed arriving to the diagnosis of Sjögren’s syndrome associated to systemic lupus erythematosus. This patient showed the following oral manifestations: xerostomia, halitosis, sensation of constant thirst, difficulties for deglutition and speaking, dry and pale lips, depapillated tongue, oral burning, intolerance to prosthesis usage, angular cheilitis and oral candidiasis. The disease management has a multidiscipline approach; the dentist plays an essential role in the disease’s diagnosis and treatment, allowing improving the life quality of these patients.

Aspectos relevantes da síndrome de Sjõgren para o cirurgião-dentista / Sjogren’s syndrome’s relevant aspects for the dental surgeon

A síndrome de Sjõgren (SS), conhecida como síndrome sicca, é uma doença autoimune caracterizada pela hipofunção das glândulas salivares e lacrimais, cuja prevalência na população mundial é de aproximadamente 0,5% a 1%. Por ser uma doença autoimune complexa e de difícil diagnóstico, é sub-diagnosticada e sub-tratada segundo o consenso realizado em 2012 pelo Colégio Americano de Reumatologia (ACR). O Cirurgião-Dentista pode desempenhar papel importante na detecção de possíveis alterações compatíveis com a síndrome, além de auxiliar no tratamento de diversas patologias orais decorrentes da síndrome. Este trabalho tem como objetivo explanar aspectos importantes referentes ao diagnóstico e tratamento da síndrome aqui discutida. A SS apesar de ser considerada uma doença de evolução lenta, em estágios avançados pode ser fatal, principalmente por aumentar as chances dos pacientes virem a desenvolver linfoma não Hodking. O tratamento odontológico dos pacientes com SS deve principalmente ser profilático, com a recomendação do uso de repositores de saliva e controle rígido da higiene bucal.

Sjogren’s syndrome (SS), known as the sicca syndrome, is an autoimmune disease characterized by salivary and lacrimal glands hypofunction which prevalence in the world population is approximatel y around 0,5% to 1%. For being a complex autoimmune disease and with difficult diagnosis, it is sub diagnosed and miss treated according to the consensus occurred in 2012 by the American College of Rheumatology (ACR). The surgeon-dentist (SD) may play a important role on the detection of possible changes compatible to the syndrome, besides can help in the treatment of many oral pathologies caused by the syndrome. This work has the main purpose to explain the important aspects regards to the correct diagnosis and treatment of this syndrome.The SS besides been considered a slow evolution disease, in advanced stages it can be fatal,mainly for increasing the patient’s chances of developing non-Hodking lymphoma. The dental treatment of patients with SS must be prophylactic, with the recomedations of the use of salivary replenishing and careful control of the oral hyigiene.

Avaliação dos efeitos do tratamento periodontal não cirúrgico sobre os sinais e sintomas da síndrome de Sjõgren primária / Evaluation of periodontal non-surgical treatment effects on the signs and symptoms on primary Sjõgren's syndrome

A síndrome de Sjõgren primária (SSp) é uma doença crônica autoimune sistêmica que pode levar à hipossalivação e afetar negativamente o ambiente oral. Os objetivos deste estudo foram detectar a influência da SSp nos níveis de biomarcadores inflamatórios na saliva e no fluido gengival nas amostras de pacientes com periodontite crônica, avaliar o efeito do tratamento periodontal não cirúrgico sobre os valores do índice clínico de avaliação da atividade sistêmica de pacientes com SSp e do índice reportado pelo paciente com SSp. Amostras de fluido gengival, saliva e os parâmetros clínicos periodontais que consistiram de medida da profundidade de sondagem (PS), nível clínico de inserção (NCI), sangramento à sondagem (SS) e índice de placa (IP) foram coletadas no início do estudo e 45 dias após a terapia periodontal não-cirúrgica de pacientes sistemicamente saudáveis com periodontite crônica (PC, n = 7) e pacientes com SSp e periodontite crônica (SP, n = 7). Pacientes periodontalmente saudáveis com SSp (SC, n = 7) e sistemicamente saudáveis (C, n = 7) também foram avaliados no início do estudo. Os grupos C, PC e SC foram pareados em gênero, idade e critério socioeconômico com o grupo SP. Os níveis de interleucina-8 (IL-8), IL-10 e IL-1ß foram avaliados por ensaio multiplex. Os níveis de atividade da doença foram medidos usando o Gold Standard da literatura chamado Índice Eular de atividade da síndrome de Sjõgren (ESSDAI). Já para avaliação dos sintomas reportados pelo paciente com SSp foi utilizado o Índice Eular reportado pelo paciente com Sjõgren (ESSPRI).

Os parâmetros clínicos melhoraram após a terapia periodontal (p <0,05). No entanto, o NCI em pacientes com SSp não melhorou significativamente após a terapia (p> 0,05). Houve um aumento nos níveis de IL-1ß, IL-8 e diminuição dos níveis de IL-10 nas amostras de saliva de pacientes do grupo SC em comparação ao grupo C (p <0,05). Já em relação ao fluido gengival, pacientes do grupo SC tiveram maiores níveis de IL-1ß em comparação com o grupo C (p<0,05). Além disso, o tratamento periodontal não cirúrgico resultou num aumento dos níveis de IL-10 no fluido gengival no grupo SP e grupo PC em relação ao valor basal (p <0,05). O fluxo salivar foi significativamente aumentado após o tratamento periodontal apenas em pacientes do grupo SP (p = 0,039). Além disso, o tratamento periodontal não influenciou o índice ESSDAI (p = 0,35) e levou a uma diminuição significativa no índice ESSPRI (p = 0,03). Os presentes dados demonstraram que a SSp influencia os níveis salivares e de fluido gengival de biomarcadores inflamatórios em favor de um perfil próinflamatório, no entanto, este perfil parece não aumentar susceptibilidade dos indivíduos SSp à destruição periodontal. Além disso, os presentes dados demonstraram que o tratamento periodontal não-cirúrgico tem um impacto positivo sobre o fluxo salivar e sobre o índice ESSPRI de pacientes com SSp. Sugere-se assim que o tratamento periodontal pode melhorar a qualidade de vida de indivíduos com SSp.

Primary Sjõgren's syndrome (pSS) is a chronic systemic autoimmune disease that might lead to hyposalivation and negatively affect the oral environment. The aims of this study were to detect the influence of pSS on the levels of inflammatory biomarkers in salivary and gingival crevicular fluid (GCF) samples of patients with chronic periodontitis and to evaluate the effect of non-surgical periodontal treatment on the disease activity index of patients with pSS, and on the reported index of patients with pSS. GCF and salivary samples and clinical parameters consisting of measuring probing depth (PD), clinical attachment level (CAL), bleeding on probing (BOP) and plaque index (PI) were collected at baseline and 45 days after non-surgical periodontal therapy from systemically healthy patients with chronic periodontitis (PC, n=7) and patients with pSS with chronic periodontitis (SP, n=7). Periodontally healthy patients with pSS (SC, n=7) and systemically healthy (C, n=7) were also evaluated at baseline. The groups C, PC and SC were pared on gender, years and socioeconomic status with the SP group. The levels of interleukin-8 (IL-8), IL-10, and IL-1ß were measured by using multiplex immunoassays. Disease activity levels were measured by using the Gold Standard called Eular Sjõgren's syndrome disease activity index (ESSDAI). Also to evaluate the symptoms reported by the pacients with pSS we used the Eular Sjõgren's syndrome patient reported index (ESSPRI).

The clinical parameters improved significantly after periodontal therapy (p<0.05). However, CAL in pSS patients was not statistically improved after therapy (p>0.05). There was an increased expression of IL-1ß, IL-8 and decreased levels of IL-10 in the salivary samples of patients in the group SC compared to the group C (p<0.05). The GCF, patients in the group SC had bigger levels of IL-1ß in comparation with the C group (p<0.05). Moreover, nonsurgical periodontal treatment resulted in increased levels of IL-10 on GCF in the groups SP and PC in relation to the baseline (p<0.05). Salivary flow was significantly increased post-treatment only in the SP's group patients (p=0.039). In addition, periodontal treatment did not influence ESSDAI index (p=0.35) and led to a statistically significant decrease on the ESSPRI index (p=0.03). The present data demonstrated that pSS influences the salivary and GCF levels of inflammatory biomarkers in favour of a proinflammatory profile, however, this profile might doesn't increase the susceptibility of pSS subjects to periodontal breakdown. In addition, the present data demonstrated that non-surgical periodontal treatment has a positive impact on the salivary flow and ESSPRI index of pSS patients. Thus suggesting that periodontal treatment may improve the quality of life of pSS subjects.

Translation and Validation of a Korean Version of the Xerostomia Inventory in Patients with Primary Sjögren's Syndrome

This study was conducted to generate and validate a cross-culturally adapted Korean version of the xerostomia inventory (XI), an 11-item questionnaire designed to measure the severity of xerostomia. The original English version of the XI was translated into Korean according to the guidelines for cross-cultural adaptation of health-related quality-of-life measures. Among a prospective cohort of primary Sjögren's syndrome (pSS) in Korea, 194 patients were analyzed. Internal consistency was evaluated by using Cronbach's alpha, and test-retest reliability was obtained by using an intraclass correlation coefficient (ICC) analysis. Construct validity was investigated by performing a correlation analysis between XI total score and salivary flow rate (SFR). Cronbach's alpha for internal consistency was 0.868, and the ICC for test-retest reliability ranged from 0.48 to 0.827, with a median value of 0.72. Moderate negative correlations between XI score and stimulated SFR, unstimulated SFR, and differential (stimulated minus unstimulated) SFR were observed (Spearman's rho, ρ = -0.515, -0.447, and -0.482, respectively; P < 0.001). The correlation analysis between the visual analogue scale (VAS) score of overall dryness and SFR indicated a smaller ρ value (-0.235 [P = 0.006], -0.243 [P = 0.002], and -0.252 [P = 0.003], respectively), which supports that XI more accurately reflects the degree of xerostomia in the pSS patients. In conclusion, the Korean version of the XI is a reliable tool to estimate the severity of xerostomia in patients with pSS.

An update of neurological manifestations of vasculitides and connective tissue diseases: a literature review / Atualização das manifestações neurológicas das vasculitides e das doenças do tecido conjuntivo: revisão de literatura

Vasculitides comprise a heterogeneous group of autoimmune disorders, occurring as primary or secondary to a broad variety of systemic infectious, malignant or connective tissue diseases. The latter occur more often but their pathogenic mechanisms have not been fully established. Frequent and varied central and peripheral nervous system complications occur in vasculitides and connective tissue diseases. In many cases, the neurological disorders have an atypical clinical course or even an early onset, and the healthcare professionals should be aware of them. The purpose of this brief review was to give an update of the main neurological disorders of common vasculitis and connective tissue diseases, aiming at accurate diagnosis and management, with an emphasis on pathophysiologic mechanisms.

As vasculites são um grupo heterogêneo de doenças autoimunes primárias ou secundárias a uma grande variedade de doenças infecciosas sistêmicas, malignas ou do tecido conjuntivo. Estas últimas são as que ocorrem com mais frequência, porém os mecanismos patogênicos ainda não foram plenamente determinados. Diversas e frequentes complicações do sistema nervoso central e periférico ocorrem nas vasculites e doenças do tecido conjuntivo. Em muitos casos, os distúrbios neurológicos têm evolução clínica atípica ou mesmo início precoce, ao que todos os profissionais de saúde devem estar cientes. O objetivo desta breve revisão foi atualizar os principais distúrbios neurológicos da vasculite comum e das doenças do tecido conjuntivo, visando ao diagnóstico e ao tratamento corretos, com ênfase nos mecanismos fisiopatológicos.

Validação e propriedades psicométricas do Eular Sjögren's Syndrome Patient Reported Index (ESSPRI) para a língua portuguesa / Validation and psychometric properties of the EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI) into Brazilian Portuguese

RESUMOObjetivo:Fazer a adaptação transcultural do Eular Sjögren's Syndrome Patient Reported Index (ESSPRI) para a língua portuguesa e avaliar as suas propriedades psicométricas.Método:Estudo transversal de pacientes com síndrome de Sjögren primária (SS). Foram estudadas as propriedades psicométricas (reprodutibilidade intraobservador e a validade de construto). Na validade de construto, o ESSPRI foi comparado com o Patient's Global Assessment (PaGA), Profile of Fatigue and Discomfort (Profad), Sicca Symptoms Inventory (SSI) e Functional Assessment of Chronic Illness Therapy (Facit-fatigue). Os testes estatísticos usados foram o α-Cronbach, coeficiente de correlação intraclasse (CCI), método de Bland-Altman e coeficiente de Spearman. Foi considerado significativo o p ≤ 0,05.Resultados:Não houve diferença entre as versões nas duas línguas e obteve-se, assim, a versão consensual brasileira. Todos os indivíduos foram mulheres de 49,4 ± 11,6 anos, com início dos sintomas de 7,2 ± 5,4 anos e tempo de diagnóstico de 3 ± 3,3 anos. A média do ESSPRI foi de 6,87 ± 1,97. A reprodutibilidade intraobservador foi alta e significativa (0,911) e, no método de Bland-Altman, não houve viés sistemático na concordância das medidas entre as avaliações. Houve correlação moderada do ESSPRI com todos os instrumentos testados.Conclusão:A versão do ESSPRI em português é válida e reprodutível.

ABSTRACTObjective:To carry out the cross-cultural adaptation of EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI) for Portuguese language and evaluate its psychometric properties.Method:Cross-secal study of patients with primary Sjögren's syndrome (SS). The psychometric properties (intraobserver reproducibility and construct validity) were studied. In construct validity, ESSPRI was compared with the Patient's Global Assessment (PGA), Profile of Fatigue and Discomfort (Profad), Sicca Symptoms Inventory (SSI) and Functional Assessment of Chronic Illness Therapy (Facit-F). Statistical tests used were: Cronbach's alpha, intraclass correlation coefficient (ICC), Bland-Altman method and Spearman coefficient. A value of p ≤ 0.05 was considered significant.Results:There was no difference between versions in both languages; thus, a Brazilian consensual version was obtained. All subjects were women aged 49.4 ± 11.6 years, with onset of symptoms of 7.2 ± 5.4 years, and time of diagnosis of 3.0 ± 3.3 years. The mean ESSPRI was 6.87 ± 1.97. The intraobserver reproducibility was high and significant (0.911) and, with Bland-Altman method, there was no systematic bias in the agreement of measures among evaluations. A moderate correlation of ESSPRI with all tested instruments was observed.Conclusion:The Brazilian Portuguese version of ESSPRI is a valid and reproducible version.

Dental perspective on Sjõgren’s syndrome: literature review / Perspectiva odontológica del síndrome de Sjõgren: revisión de literatura

The human body releases around 500-600mL of saliva daily, however when values of unstimulated whole saliva range from 0.1 to 0.2mL/ min, there is a condition called Hyposalivation or hyposialia. Hyposalia is characterized by a large number of systemic conditions, including Sjõgren’s syndrome, a chronic autoimmune disease that affects between 0.1 and 3 percent of the world population and is characterized by exocrinopathy of the salivary glands leading to glandular hypofunction and thus decreasing the normal salivary flow. Saliva is part of innate immunity, when there is a decrease in protein secretion, numerous oral manifestations occur such as dental caries, candidiasis, gingival disease, angular cheilitis, lymphomas of the salivary glands, dysphagia, erythematous and fissured tongue, among others. Currently there is no defined dental treatment, however there are alternative treatments by sialogogues and salivary substitutes, plus non-pharmacological therapies, which seek to maintain the ecology and oral conditions stable, in addition to preventive and restorative dental treatment for lesions already established as a consequence of the disease. The aim of this study is to conduct a literature review on the characteristics, classification, oral manifestations and dental management of Sjõgren’s syndrome...

El ser humano secreta alrededor de 500 a 600 mL de saliva diariamente, sin embargo, al encontrarse valoresde 0.1 - 0.2 mL/min de saliva en reposo se presenta una condición llamada hiposalivación o hiposialia, la cual puedeser manifestada por una numerosa cantidad de condiciones sistémicas, entre ellas el síndrome de Sjõgren, la cual es una enfermedad autoinmune crónica presente en entre el 0.1 y3% de la población mundial, y es caracterizada por exocrinopatíade las glándulas salivales conllevando a la hipofunción glandular y disminuyendo así el flujo salival normal.Debido a que la saliva forma parte de la inmunidad innata, al presentarse una disminución en su secreción proteica sedesencadenan numerosas manifestaciones orales, tales comocaries dental, candidiasis, enfermedad gingival, queilitis angular, linfomas de las glándulas salivales, disfagia, lenguaeritematosa y fisurada, entre otras. Actualmente no existe un tratamiento odontológico definido, sin embargo se tienen alternativas de tratamiento mediante fármacos sialogogos ysustitutos salivales, además de terapias no farmacológicas, las cuales intentan mantener la ecología y las condiciones orales estables, además de los tratamientos odontológicos preventivosy restaurativos para lesiones ya establecidas por consecuenciade la enfermedad. El objetivo del presente estudio es realizar una revisión de literatura sobre las características, criterios de clasificación, manifestaciones orales y el manejoodontológico del Síndrome de Sjõgren...