Asunto(s)
Síndrome de Sjögren , Humanos , Síndrome de Sjögren/complicaciones , Síndrome de Sjögren/diagnóstico , Femenino , Resultado del Tratamiento , Vasculitis del Sistema Nervioso Central/diagnóstico , Vasculitis del Sistema Nervioso Central/etiología , Persona de Mediana Edad , Imagen por Resonancia MagnéticaRESUMEN
Sjogren's disease (SjD) is an autoimmune disease that is characterized not only by the sicca symptoms it causes but also by its systemic nature, which is capable of several and not yet fully understood extraglandular manifestations. To gain a clearer understanding of these manifestations as well as a better practical approach, a panel of experts from the Brazilian Society of Rheumatology conducted a systematic review and meta-analysis on the identification of epidemiologic and clinical features of the extraglandular manifestations present in ESSDAI (EULAR Sjogren´s syndrome disease activity index), followed by a voting panel with recommendations for clinical practice. This publication is complementary to others already published and covers cutaneous and hematological manifestations, with prevalence data generated by a meta-analysis of 13 clinical or laboratory manifestations and 6 clinical management recommendations.
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Síndrome de Sjögren , Enfermedades de la Piel , Humanos , Brasil/epidemiología , Enfermedades Hematológicas/etiología , Reumatología/normas , Síndrome de Sjögren/complicaciones , Enfermedades de la Piel/etiología , Sociedades MédicasRESUMEN
INTRODUCTION: Sjögren's disease (SD) is an immune-mediated chronic inflammatory disease that affects epithelial tissues, mainly salivary and lacrimal glands. It also presents extraglandular manifestations. The main renal manifestation is tubulointerstitial nephritis (TIN), which can manifest as renal tubular acidosis (RTA). Urinary citrate may be a biomarker of RTA in these patients. The objective of this study was to evaluate whether hypocitraturia is a predictive biomarker of RTA in a sample of patients with SD in a tertiary hospital in southern Brazil. METHODS: All patients with SD who met the inclusion criteria and who participated in the rheumatology outpatient clinic of the Irmandade Santa Casa de Misericórdia de Porto Alegre were included. Demographic, SD, serological and urinary data were obtained. RTA was considered in those patients who persistently presented urinary pH above 5.5 and serum pH below 7.35. Patients who persistently had urinary pH above 5.5 underwent a urinary acidification test with furosemide and fludrocortisone. These patients received 1 mg of fludrocortisone and 40 mg of furosemide and had their urine samples tested 2, 4 and 6 h after taking the medications. The test was stopped at any urine sample with pH 5.5 or less. The variables were expressed as mean and standard deviation or interquartile range. The association between hypocitraturia and RTA was assessed using the chi-square. RESULTS: Forty-two patients were included, 95.2% female with a median age of 61.73 years. The prevalence of complete distal RTA was 4.88%. Twenty-eight patients underwent urine acidification testing. Five patients had hypocitraturia, and two of them had complete distal RTA. The association between hypocitraturia and RTA was statistically significant (p < 0.012), with a sensitivity of 100%, specificity of 91.2% and accuracy of 91.7%. The negative predictive value was 100%. The global renal assessment of the population demonstrated two patients with RTA, one patient with decreased renal function and six patients with proteinuria greater than 0.5 g/24 h. CONCLUSION: The prevalence of RTA in the studied population was 4.88%. Hypocitraturia had high sensitivity and accuracy for the diagnosis of RTA.
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Acidosis Tubular Renal , Biomarcadores , Ácido Cítrico , Furosemida , Síndrome de Sjögren , Humanos , Acidosis Tubular Renal/diagnóstico , Acidosis Tubular Renal/orina , Acidosis Tubular Renal/etiología , Síndrome de Sjögren/complicaciones , Síndrome de Sjögren/orina , Síndrome de Sjögren/diagnóstico , Femenino , Biomarcadores/orina , Persona de Mediana Edad , Masculino , Furosemida/uso terapéutico , Furosemida/administración & dosificación , Ácido Cítrico/orina , Fludrocortisona/uso terapéutico , Adulto , Concentración de Iones de Hidrógeno , Anciano , BrasilRESUMEN
ABSTRACT: A 65-year-old woman presented with unexplained weight loss, recurrent fever, and a dermatosis with painful nodules on the extremities. Biopsies showed focal lobular panniculitis with neutrophilic microgranulomas. Comprehensive investigations ruled out infection and hematologic and solid organ neoplasms. Laboratory results showed anti-Ro/SSA and anti-La/SSB antibody positivity, and elevated inflammatory markers. Dry mouth and eye were confirmed. The diagnosis of Sjögren syndrome with cutaneous panniculitis was established. Prednisone treatment with 30 mg/d resulted in remission of fever and pain improvement. This case emphasizes Sjögren syndrome as an autoimmune disease with multiple cutaneous manifestations and highlights its association with granulomatous panniculitis.
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Paniculitis , Síndrome de Sjögren , Humanos , Femenino , Síndrome de Sjögren/complicaciones , Síndrome de Sjögren/tratamiento farmacológico , Anciano , Paniculitis/patología , Paniculitis/etiología , Prednisona/uso terapéutico , Granuloma/patología , Resultado del Tratamiento , BiopsiaRESUMEN
This study aimed to perform a systematic review to evaluate the impact of the oral repercussions of Sjogren's Syndrome (SS) on the quality of life (QoL) of patients living with this disease. To conduct this work, we followed the PRISMA guidelines. The included studies evaluated oral repercussions of SS and their correlation with QoL. The risk of bias was assessed with the JBI tools for each type of study design. Our findings resulted in 26 articles met the eligibility criteria. Seventeen articles (65.3%) used the OHIP-14 questionnaire to evaluate oral health-related QoL (OHRQoL) and showed that the oral repercussions of SS had a negative impact. Ten studies applied other QoL instruments, in which 5 found a correlation between oral repercussions of SS and poorer OHRQoL, 3 showed no correlation, and 2 were unclear. Due to significant heterogeneity, the meta-analysis was limited to 8 of the 17 studies that used the OHIP-14. The results showed statistically significant poorer OHRQoL in individuals with SS compared to healthy controls. In conclusion, oral repercussions of SS negatively affect QoL. However, future studies should focus on standardized methodology to provide more homogenous and comparable evidence.
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Calidad de Vida , Síndrome de Sjögren , Síndrome de Sjögren/psicología , Síndrome de Sjögren/complicaciones , Humanos , Encuestas y Cuestionarios , Salud BucalRESUMEN
OBJECTIVES: To describe the taxonomy of the microbiota in crevicular fluid of primary Sjögren's syndrome (pSS) patients, and evaluate its association with clinical/serological variables, and oral quality of life. METHODS: Observational study that included 48 pSS without diabetes mellitus, no active neoplasia, no antibiotic use in the previous two weeks, and no current active infection. We registered demographics, oral/ocular sicca symptoms, parotid enlargement and anti-Ro/La serology. We assessed the non-stimulated whole salivary flow (NSWSF), the EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI), and the Xerostomia-related Quality of Life Scale (XeQoLS). Two periodontists determined the presence of periodontal disease and collected crevicular fluid from 6 teeth using filter paper. Samples were frozen at -86°C until processing. We included 17 sex- and age-matched control subjects. Bacterial DNA was extracted from the crevicular fluid sample using a commercial kit. 16SrRNA V3-V4 region was sequenced using reversible adaptor technology. Sequences were pre-processed and analysed using QIIME2 and phyloseq software programs. Functionality profiles were predicted using the Tax4Fun2 package. RESULTS: PSS patients had more bacteria of the genera Prevotella, Streptococcus, Veillonella, Fusobacterium, and Leptotrichia and fewer bacteria of the genus Selenomonas than controls. The pSS microbiota contained more genes encoding accessory secretory proteins. Microbiota also differed between patients with anti-Ro/La status, parotid gland enlargement, and periodontal disease severity, but did not correlate with NSWSF and XeQoLS. CONCLUSIONS: The crevicular fluid microbiota of pSS patients and controls differed significantly, even in SSP patients depending on their serology, parotid gland enlargement, and periodontal disease status.
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Microbiota , Enfermedades Periodontales , Síndrome de Sjögren , Xerostomía , Humanos , Síndrome de Sjögren/complicaciones , Calidad de VidaRESUMEN
Sjogren's syndrome (SS) is a complex autoimmune disease characterized by lymphocytic infiltration of salivary and lacrimal glands, resulting in sicca symptoms. Additionally, SS presents with neurological manifestations that significantly impact the nervous system. This review aims to provide a comprehensive overview of the neurological aspects of SSj, covering both the peripheral and central nervous system involvement, while emphasizing diagnosis, treatment, and prognosis.
A síndrome de Sjogren (SS) é uma doença autoimune complexa caracterizada pela infiltração linfocítica das glândulas salivares e lacrimais, resultando em sintomas sicca. Além disso, a SS apresenta manifestações neurológicas que afetam significativamente o sistema nervoso. Esta revisão tem como objetivo fornecer uma visão abrangente dos aspectos neurológicos da SSj, abordando tanto o envolvimento do sistema nervoso periférico quanto do central, com ênfase no diagnóstico, tratamento e prognóstico.
Asunto(s)
Enfermedades del Sistema Nervioso , Síndrome de Sjögren , Humanos , Síndrome de Sjögren/complicaciones , Enfermedades del Sistema Nervioso/etiologíaRESUMEN
BACKGROUND: The aim of this study is to relate all the superficial mucoceles found in a cancer center, described the association with oncological conditions, and discuss its etiology and pathology that we found in the past few years. MATERIAL AND METHODS: Sixteen cases of superficial mucocele were retrieved from the patients' records of the Stomatology Department of the A. C. Camargo Cancer Center, São Paulo, Brazil, and demographic and clinical data were collected from electronic medical records. RESULTS: There were 16 patients, 8 patients were men and 8 women, with ages varying from 26 to 70 years old. Superficial mucoceles were observed in patients submitted to head and neck radiotherapy (n=6), graft versus host disease (n=4), one associated with oral mucositis related to allogenic bone marrow stem cells transplantation (n=1), systemic lupus (n=1), Sjögren's syndrome (n=1), oral lichenoid lesion associated with pembrolizumab (n=1) and no local or systemic inflammatory associated found (n=2). CONCLUSIONS: This study reports a series of superficial mucoceles from a single stomatology unit. Most patients had superficial mucoceles secondary to head and neck radiotherapy and graft versus host diseases. However, two patients (12.5%) had mucoceles related to systemic inflammatory conditions (Sjögren's Syndrome and Systemic Lupus).
Asunto(s)
Enfermedad Injerto contra Huésped , Mucocele , Medicina Oral , Síndrome de Sjögren , Masculino , Humanos , Femenino , Adulto , Persona de Mediana Edad , Anciano , Mucocele/etiología , Mucocele/patología , Síndrome de Sjögren/complicaciones , Estudios Retrospectivos , Brasil/epidemiología , Enfermedad Injerto contra Huésped/complicacionesRESUMEN
BACKGROUND: Several systemic conditions can result in distinct degrees of salivary gland damage and consequent hypofunction. The development of successful management schemes is highly challenging due to the complexity of saliva. This study aimed to systematically map the literature on the physical stimulation of salivary glands for hyposalivation management and the response of individuals according to different systemic conditions causing salivary impairment. METHODS: A systematic search in the literature was performed. Two reviewers independently selected clinical trials, randomized or not, that used physical stimulation to treat hyposalivation caused by systemic conditions. Studies evaluating healthy subjects without hyposalivation were included as controls. Single-arm clinical studies or case series were also included for protocol mapping (PRISMA extension for scoping reviews). RESULTS: Out of 24 included studies, 10 evaluated healthy subjects, from which 9 tested transcutaneous electrical nerve stimulation (TENS) and 1 tested acupuncture and electroacupuncture. Fourteen studies evaluated individuals with hyposalivation: 6 applied TENS, 6 applied low-level laser therapy (LLLT), and 2 applied acupuncture, carried out in post-chemotherapy, medication use, postmenopausal women, hemodialysis patients, smokers, diabetics, Sjögren's syndrome (SS). All showed increased salivation after treatment, except for two LLLT studies in individuals with SS. CONCLUSIONS: Among the different patient groups, individuals with Sjögren's syndrome (SS) exhibited the poorest responses, while those with medication-induced hyposalivation demonstrated the most favorable treatment outcomes, independently of the management strategy for saliva stimulation. It means that physical stimulation of salivary glands holds promise as an alternative for managing hyposalivation in cases of reversible gland damage. However, to make informed decisions in current practice, it is necessary to conduct new well-designed randomized clinical trials with appropriate methodologies.
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Síndrome de Sjögren , Xerostomía , Humanos , Femenino , Síndrome de Sjögren/complicaciones , Síndrome de Sjögren/terapia , Xerostomía/etiología , Xerostomía/terapia , Saliva , Voluntarios Sanos , Estimulación FísicaRESUMEN
Neonatal lupus erythematosus (NLE) is a very rare autoimmune disease, occurring in neonates born to mothers who present auto-antibodies to cytoplasmic antigens of Sjögren's syndrome. In most cases, the clinical course is benign toward spontaneous resolution, but there is a group of patients who develop severe involvement of the cardiac conduction system, therefore, early detection is critical. OBJECTIVE: To describe a clinical case of neonatal lupus erythematosus emphasizing the importance of timely diagnosis in the patient and the mother. CLINICAL CASE: A 33-year-old woman, with a history of hypertension, came to the dermatology department for her 15-day-old male neonate who presented a recent onset of round, erythematous, raised-edged, and non-scaling plaques consistent with NLE. Cardiac conduction involvement was ruled out. Newborn's laboratory tests showed moderate neutropenia, mild increase of transaminases, and positive anti-Ro and anti-La antibodies. On directed anamnesis, the mother reported a personal history of symptoms consistent with connective tissue disease, such as fatigue, alopecia, and xerophthalmia. Antinuclear antibodies from the mother showed titers of 1/1280 in a speckled pattern, positive anti-double-stranded DNA antibodies, and anti-Ro and Anti-La antibodies. Schirmer Test was consistent with dry eye, therefore, Systemic Lupus Erythematosus associated with Sjögren's Syndrome was diagnosed. The infant was followed up for 5 months with remission of cutaneous manifestations and normalization of laboratory tests. CONCLUSION: Although cutaneous manifestations of NLE are benign and transient in the newborn, these can be associated with other life-threatening manifestations that require an active search and prompt management by the medical team. A 25% of mothers of newborns with NLE are asymptomatic or unaware of their SLE diagnosis before delivery, so timely diagnosis of NLE leads to the diagnosis of asymptomatic mothers, improving their follow-up and treatment.
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Lupus Eritematoso Cutáneo , Lupus Eritematoso Sistémico , Síndrome de Sjögren , Lactante , Femenino , Humanos , Recién Nacido , Masculino , Adulto , Síndrome de Sjögren/complicaciones , Lupus Eritematoso Cutáneo/diagnóstico , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/diagnóstico , MadresRESUMEN
Neurological manifestations can occur in up to 67% of patients with primary Sjögren's Syndrome, also known as Neuro-Sjogren's syndrome (NSS), and a 5% can present central nervous system involvement, with severe and possibly lethal consequences. We present the radiological follow-up of a patient with NSS who consulted for limb weakness and visual loss, and fourteen years later developed sicca symptoms. She was diagnosed with a saliva gland biopsy, and started treatment with steroids, cyclophosphamide, and then rituximab, achieving a favourable clinical response and stabilization of lesions. We discuss key aspects regarding the clinical presentation, diagnosis, imaging, and treatment of this elusive disease.
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Enfermedades del Sistema Nervioso Central , Síndrome de Sjögren , Femenino , Humanos , Síndrome de Sjögren/complicaciones , Síndrome de Sjögren/diagnóstico por imagen , Enfermedades del Sistema Nervioso Central/diagnóstico por imagen , Enfermedades del Sistema Nervioso Central/tratamiento farmacológico , Rituximab/uso terapéutico , Ciclofosfamida/uso terapéutico , Sistema Nervioso Central/diagnóstico por imagenRESUMEN
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.
Asunto(s)
Humanos , Masculino , Femenino , Adulto , Persona de Mediana Edad , Anciano , Adulto Joven , Ansiedad/epidemiología , Calidad de Vida/psicología , Síndrome de Sjögren/epidemiología , Depresión/epidemiología , Síndrome de Sjögren/complicaciones , Estudios de Casos y ControlesRESUMEN
Neurological manifestations such as polyneuropathy are reported in 8-49% of cases with Sjögren's Syndrome (SjS), but central nervous system involvement is seldom described. We report a 46-year-old woman with a history of SjS with distal renal tubular acidosis and autoimmune thyroiditis. She consulted in the emergency room for a five-days history of holocranial headache and explosive vomiting. Fundoscopy showed bilateral papilledema. Brain computed tomography (CT) without contrast showed diffuse encephalic edema, with effacement ofsulci and restriction ofperitruncal cisterns. Brain AngioCT ruled out thrombosis, and brain magnetic resonance (MRI) was without structural alterations or hydrocephalus. Lumbar puncture had increased cerebrospinal fluid output pressure but without cytochemical alterations, and negative gram, cultures and filmarray. The diagnosis of Intracranial Hypertension Syndrome (ICHTS) ofprobable autoimmune etiology in the context of SjS was proposed, and management with high-dose corticosteroids was initiated with favorable clinical and imaging response.
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Humanos , Femenino , Persona de Mediana Edad , Síndrome de Sjögren/complicaciones , Síndrome de Sjögren/diagnóstico , Hipertensión Intracraneal/etiología , Encéfalo , Imagen por Resonancia Magnética , CefaleaRESUMEN
OBJECTIVES: We aimed to determinate the frequency of this association and compare the features of neuromyelitis optica spectrum disorder (NMOSD) with and without associated autoimmune diseases (AD) in a Latin American (LATAM) population in clinical practice. METHODS: We retrospectively reviewed the medical records of patients with NMOSD according to the 2015 diagnostic criteria. Patients from Argentina (n=77), Brazil (n=46), and Venezuela (n=17) were enrolled and classified into two groups as follows: with AD or without AD. Clinical, paraclinical (including aquaporin-4 antibodies (AQP4-ab) status), magnetic resonance imaging (MRI), and prognosis data were analyzed and compared. Kaplan-Meier (KM) and the Nelson-Aalen estimator analyses were performed to estimate both time and the cumulative hazard risk of disability reaching an EDSS≥4; and time for the first recurrence. RESULTS: Out of 140 patients, 33 (23.5%) patients had associated an AD at presentation. The most frequent associated AD was Hashimoto disease (n=10) followed by lupus (n=7) and Sjogren's syndrome (n=6). However, rituximab use (42.4% vs. 21.5%, p=0.02), female gender (82.2% vs. 100%, p=0.006), corticospinal lesions on MRI (0% vs. 12.5%, p=0.01) at onset, and positivity for antinuclear antibodies (21.2% vs. 48.4%, p=0.03) were significantly associated with NMOSD patients with AD in comparison to NMOSD patients without AD. No differences were found in other clinical and paraclinical aspects between groups. KM and Nelson-Aalen estimator analyses did not show differences between groups. CONCLUSION: NMOSD patients associated with AD were observed in 23.5%. In addition, NMOSD patients with and without associated AD were similar in most evaluated features.
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Neuromielitis Óptica , Síndrome de Sjögren , Humanos , Femenino , Neuromielitis Óptica/complicaciones , Neuromielitis Óptica/diagnóstico por imagen , Neuromielitis Óptica/epidemiología , Acuaporina 4 , Estudios Retrospectivos , Autoanticuerpos , Síndrome de Sjögren/complicaciones , Síndrome de Sjögren/epidemiologíaRESUMEN
BACKGROUND: Few studies have described lymphocytic interstitial pneumonia (LIP) in Sjögren's syndrome (SS). We aimed to analyze the clinical, therapeutic, and outcome of patients with SS and LIP. METHODS: We searched for articles in PubMed/MEDLINE, LILACS, SciELO, and Cochrane from 1966 to 2020, in English, Spanish, French, Chinese, and Japanese literature had an English summary about LIP and SS. The keywords were "Sjögren syndrome" and "Lymphocytic interstitial pneumonia." Additionally, we report a patient with SS and LIP. RESULTS: 27 patients with SS and LIP were found. The age range ranged from 14 to 73 years old, with only 3 male patients, with a predominance of LIP cases in patients with primary SS (22/27). In the following case, the LIP preceded SS by 2 years; in the other 26 patients, SS preceded it. The majority presented dyspnea, mainly on exertion, followed by a dry cough. Lung biopsy was performed in 10 studies. Therapy varied from the use of clinical observation, corticosteroids alone, or associated with immunosuppressants. Most studies have shown improvement or stabilization of the pulmonary condition after therapy (13/16 studies). CONCLUSION: This article reviews cases of lymphocytic interstitial pneumonia associated with Sjögren syndrome and shows a good outcome with adequate treatment. It emphasizes that early LIP diagnosis in patients with Sjogren Syndrome may be determined using lung computed tomography.
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Enfermedades Pulmonares Intersticiales , Síndrome de Sjögren , Adolescente , Adulto , Anciano , Humanos , Masculino , Persona de Mediana Edad , Adulto Joven , Pulmón/patología , Enfermedades Pulmonares Intersticiales/complicaciones , Enfermedades Pulmonares Intersticiales/diagnóstico por imagen , Síndrome de Sjögren/complicaciones , Síndrome de Sjögren/diagnóstico , Tomografía Computarizada por Rayos X , FemeninoRESUMEN
Neurological manifestations such as polyneuropathy are reported in 8-49% of cases with Sjögren's Syndrome (SjS), but central nervous system involvement is seldom described. We report a 46-year-old woman with a history of SjS with distal renal tubular acidosis and autoimmune thyroiditis. She consulted in the emergency room for a five-days history of holocranial headache and explosive vomiting. Fundoscopy showed bilateral papilledema. Brain computed tomography (CT) without contrast showed diffuse encephalic edema, with effacement ofsulci and restriction ofperitruncal cisterns. Brain AngioCT ruled out thrombosis, and brain magnetic resonance (MRI) was without structural alterations or hydrocephalus. Lumbar puncture had increased cerebrospinal fluid output pressure but without cytochemical alterations, and negative gram, cultures and filmarray. The diagnosis of Intracranial Hypertension Syndrome (ICHTS) ofprobable autoimmune etiology in the context of SjS was proposed, and management with high-dose corticosteroids was initiated with favorable clinical and imaging response.
Asunto(s)
Hipertensión Intracraneal , Síndrome de Sjögren , Femenino , Humanos , Persona de Mediana Edad , Síndrome de Sjögren/complicaciones , Síndrome de Sjögren/diagnóstico , Encéfalo , Cefalea , Imagen por Resonancia Magnética , Hipertensión Intracraneal/etiologíaRESUMEN
OBJECTIVE: This study aimed to assess the correlation between Sjögren's Syndrome (SS) and adverse pregnancy outcomes, with the aim of providing a basis for preconception and pregnancy interventions in women with SS. METHODS: A search of electronic databases in English and Chinese databases from January 2005 to December 2021, was conducted to collect the literature of case-control studies or cohort studies on the association between SS and pregnancy outcome studies. Literature inclusion and data extraction were performed according to established criteria, and the Newcastle-Ottawa scale was used to evaluate the quality of the literature. Stata 15 software was used for meta-analysis. RESULTS: A total of nine papers were included in this study. Meta-analysis results showed that SS was associated with spontaneous abortion (RR = 8.85, 95% CI 3.10â25.26), preterm birth (RR = 2.27, 95% CI 1.46â3.52), low birth mass (RR = 1.99, 95% CI 1.34â2.97), and birth defects (RR = 4.28, 95% CI 3.08â5.96). CONCLUSION: SS can increase the incidence of adverse pregnancy outcomes.
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Aborto Espontáneo , Nacimiento Prematuro , Síndrome de Sjögren , Embarazo , Recién Nacido , Femenino , Humanos , Resultado del Embarazo/epidemiología , Nacimiento Prematuro/epidemiología , Nacimiento Prematuro/etiología , Síndrome de Sjögren/complicaciones , Aborto Espontáneo/epidemiología , Estudios de CohortesRESUMEN
The aim of this retrospective cross-sectional study was to verify the association between salivary flow rates (SFR) and the histopathologic aspects of labial salivary glands (LSG) in patients with rheumatoid arthritis (RA). Patients presenting rheumatologic diseases referred for oral evaluation were included in the study if they had RA and had SFR measured and LSG biopsy performed. Patients were excluded if they had systemic conditions that affect SFR or if they were being treated for hyposalivation. Cases without enough material for histopathologic analysis were also excluded. Data were collected through questionnaires, oral examination, resting and stimulated SFR, and LSG biopsies. A histopathologic reevaluation was carried out in order to seek for additional histopathologic aspects. Fifty-one patients met the inclusion criteria. The mean age was 53.5 years (25-77), and 94.1% were women. The median resting and stimulated SFRs were 0.24 mL/min and 1.02 mL/min, respectively. The presence of lymphocytic focus and fibrosis were significantly associated with stimulated SFR, but not with resting SFR. The odds ratio of patients who had hyposalivation for presenting a positive lymphocytic focus was 7.33 (confidence interval CI: 1.53-35.23) by the stimulated technique, and 2.56 (CI: 0.57-11.40) in resting SFR. In the medical records, 14 (31.80%) patients had been diagnosed with secondary Sjögren's syndrome. In conclusion, stimulated SFR represent a good screening test to predict lymphocytic focus in LSG in patients with RA, which represents the most specific test to diagnose Sjögren's syndrome.