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1.
Medicine (Baltimore) ; 99(45): e23029, 2020 Nov 06.
Artigo em Inglês | MEDLINE | ID: mdl-33157952

RESUMO

RATIONALE: Neuromyelitis optica spectrum disorder (NMOSD) patients, especially those with anti-aquaporin-4 antibody positivity, a water channel expressed on astrocytes, is often accompanied by autoimmune diseases (ADs) including Sjogren syndrome (SS). Here, we report a case of a young Chinese woman with recurrent optic neuritis who was diagnosed with asymptomatic SS and NMOSD. PATIENT CONCERNS: A 22-year-old Chinese woman suffered from optic neuritis for 3 years. The main manifestation was recurrent loss of vision. The anti-aquaporin-4 antibody was positive in the cerebrospinal fluid, and she was diagnosed with NMOSD. Other laboratory tests revealed positive anti-SSA and anti-SSB antibodies, and labial gland biopsy showed lymphocytic infiltration. She also fulfilled the international criteria for SS. DIAGNOSIS: On the basis of recurrent vision loss and laboratory examination, we defined the patient with SS accompanied by NMOSD. INTERVENTIONS: When the patient first experienced vision loss, the corticosteroid treatment in the external hospital was effective, and her visual acuity improved significantly. However, in several later attacks, such treatment was no longer obviously effective. Considering the patient's condition, she was treated with corticosteroids, cyclophosphamide, and immunoglobulin therapy on admission. OUTCOMES: The patient's visual acuity was increased to the right eye 20/800 and left eye finger counting when she was discharged from the hospital. LESSONS: SS accompanied with NMOSD is common in clinical practice, and always with the positive Anti-AQP4 antibody as a potential biomarker. Patients with SS and NMOSD showed significant neurological symptoms and had a worse prognosis than SS patients with negative anti-AQP4 antibody because of cross-immunity between anti-SSA antibody and anti-AQP4 antibody. Rheumatologists and ophthalmologists should pay attention to this and perform appropriate tests.


Assuntos
Aquaporina 4/líquido cefalorraquidiano , Neuromielite Óptica/complicações , Neurite Óptica/etiologia , Síndrome de Sjogren/complicações , Corticosteroides/uso terapêutico , Aquaporina 4/antagonistas & inibidores , Cegueira/diagnóstico , Cegueira/etiologia , Ciclofosfamida/uso terapêutico , Feminino , Humanos , Imunização Passiva/métodos , Imunossupressores/uso terapêutico , Neuromielite Óptica/imunologia , Neurite Óptica/tratamento farmacológico , Recidiva , Resultado do Tratamento , Acuidade Visual/efeitos dos fármacos , Adulto Jovem
2.
Ned Tijdschr Tandheelkd ; 127(10): 533-542, 2020 Oct.
Artigo em Holandês | MEDLINE | ID: mdl-33156294

RESUMO

Three patients who were referred to the saliva of the Center for Special Care Dentistry (Stichting Bijzondere Tandheelkunde, SBT) in Amsterdam clearly demonstrate that in the case of patients suffering from xerostomia, it can be useful to have the saliva secretion rate determined and to take saliva into account in the aetiology and in developing a dental treatment plan. In the first case, a 39-year-old woman presented with dry mouth associated with Sjögren's syndrome. A fair degree of saliva secretion was still possible. The teeth were characterised by wear, caries lesions and sensitivity. The second patient was a 42-year-old man suffering from dry mouth associated with the use of medications. His teeth were weakened to the point of deciding to create conventional full dentures, despite a possible moderate prognosis due to oral dryness. The third patient, a 79-year-old woman, was suffering from severe dry mouth, associated with Sjögren's syndrome. Due to the ruinous condition of her teeth and extreme dry mouth, the decision was made to remove the remaining dentition and insert implant-retained dentures.


Assuntos
Síndrome de Sjogren , Xerostomia , Adulto , Idoso , Feminino , Humanos , Masculino , Saliva , Síndrome de Sjogren/complicações , Síndrome de Sjogren/diagnóstico , Xerostomia/etiologia
3.
Ned Tijdschr Tandheelkd ; 127(10): 543-550, 2020 Oct.
Artigo em Holandês | MEDLINE | ID: mdl-33156295

RESUMO

Important characteristics of Sjögren syndrome include a sensation of dry mouth, reduced saliva secretion and swelling of the large salivary glands, usually the parotids. Sjögren patients also run a significantly increased risk of developing a non-Hodgkin lymphoma, generally a so-called mucosa associated lymphoid tissue (MALT) lymphoma. Such a MALT lymphoma often develops in the parotid gland. In 2 cases patients presented with recurrent swellings of the parotid glands who developed both Sjögren syndrome and a MALT lymphoma in both of their parotid glands.


Assuntos
Linfoma de Zona Marginal Tipo Células B , Síndrome de Sjogren , Humanos , Linfoma de Zona Marginal Tipo Células B/diagnóstico , Glândulas Salivares , Síndrome de Sjogren/complicações , Síndrome de Sjogren/diagnóstico
6.
Medicine (Baltimore) ; 99(43): e22942, 2020 Oct 23.
Artigo em Inglês | MEDLINE | ID: mdl-33120855

RESUMO

RATIONALE: Tracheobronchial amyloidosis (TBA) associated with Sjögren syndrome is very rare. Here, we describe a case with this phenomenon, in order to better understand the condition. PATIENT CONCERNS: A 52-year-old woman presented after 6 months of coughing, sputum, and dyspnea. Chest computed tomography revealed thickened bronchial walls, which were irregular on the left side the trachea. She had a history of dry eye and dry mouth of at least 3 years' duration. DIAGNOSES: Sjögren syndrome was diagnosed based on her symptoms, ophthalmological and parotid examination, and immunological and autoantibody tests. The diagnosis of TBA was confirmed by Congo red staining of a tracheal biopsy. INTERVENTIONS: The patient was given glucocorticoids without any other immunosuppressants. OUTCOMES: The symptoms improved after 6 months. LESSONS: TBA associated with Sjögren syndrome is a rare condition. TBA is characterized by amyloid deposition to the trachea in the absence of systemic amyloidosis. Diagnosis requires tissue biopsy with demonstration of amyloid deposition.


Assuntos
Amiloidose/etiologia , Broncopatias/patologia , Síndrome de Sjogren/complicações , Doenças da Traqueia/patologia , Amiloidose/diagnóstico , Biópsia , Broncoscopia/métodos , Tosse/diagnóstico , Tosse/etiologia , Dispneia/diagnóstico , Dispneia/etiologia , Feminino , Glucocorticoides/uso terapêutico , Humanos , Pessoa de Meia-Idade , Síndrome de Sjogren/diagnóstico , Síndrome de Sjogren/tratamento farmacológico , Escarro , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento
7.
Clin Exp Rheumatol ; 38 Suppl 126(4): 110-115, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33025900

RESUMO

OBJECTIVES: Digestive involvement (DI) has been reported in 10-30% of primary Sjögren's syndrome (pSS) patients, and few studies have systematically analysed the prevalence of DI in pSS patients. The aim of this study was to describe DI prevalence in pSS patients from the Sjögrenser Study, and to analyse its clinical associations. METHODS: All patients included in the Sjögrenser study, a Spanish multicentre randomised cohort, containing demographic, clinical and histologic data, have been analysed retrospectively. Patients were classified according to the presence of DI (oesophageal, gastric, intestinal, hepatic and pancreatic), and we have performed DI clinical associations, descriptive statistics, Student t or χ2 test, and uni and multivariate logistic regression. RESULTS: From 437 included patients, 95% were women, with a median age of 58 years, 71 (16.2%) presented DI: 21 (29.5%) chronic atrophic gastritis, 12 (16.9%) oesophageal motility dysfunction, 3 (4.2%) lymphocytic colitis, 18 (25.3%) primary biliary cholangitis, 15 (21.1%) autoimmune hepatitis, 7 (9.8%) pancreatic involvement and 5 (7%) coeliac disease. Half of them developed DI at the same time or after pSS diagnosis. Patients with DI were significantly older at pSS diagnosis (p=0.032), more frequently women (p=0.009), presented more autoimmune hypothyroidism and C3 hypocomplementaemia (p=0.040), and were treated more frequently with glucocorticoids, immunosuppressant and biologic therapies. Patients with pancreatic involvement presented more central nervous system and renal involvement, Raynaud's phenomenon, lymphoma and C3/C4 hypocomplementaemia. CONCLUSIONS: DI is frequent in Sjögrenser patients, mainly in the form of autoimmune disorders, and seem to be associated with a more severe phenotype. Our results suggest that DI should be evaluated in pSS patients, especially those with more severe disease.


Assuntos
Hepatite Autoimune , Síndrome de Sjogren , Estudos de Coortes , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Ensaios Clínicos Controlados Aleatórios como Assunto , Sistema de Registros , Estudos Retrospectivos , Síndrome de Sjogren/complicações , Síndrome de Sjogren/diagnóstico , Síndrome de Sjogren/epidemiologia
8.
Clin Exp Rheumatol ; 38 Suppl 126(4): 291-300, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33095142

RESUMO

Interstitial lung disease (ILD) is considered the most frequent and serious pulmonary complication in primary Sjögren's syndrome (pSS), with the majority of the studies indicating a prevalence of about 20%, and resulting in significant morbidity and mortality. Although ILD was historically described as a late manifestation of pSS, more recently, a high variability of the time of onset of pSS-ILD has been observed and from 10 to 51% of patients can develop ILD years before the onset of pSS. Lymphocytic interstitial pneumonia is highly typical for SS, but it occurs only in a few cases, while the most common ILD pattern is nonspecific interstitial pneumonia, followed by usual interstitial pneumonia and organising pneumonia. Multidisciplinary discussion can be necessary in pSS cases with ambiguous clinical findings, when differential diagnosis with IIPs might be very difficult. Up to date, available data do not allow to establish an evidence-based treatment strategy in pSS-ILD. Glucocorticoids are empirically used, usually in association to immunosuppressive drugs, such as cyclophosphamide and mycophenolate mofetil. A better understanding of the molecular mechanisms involved in the pathogenesis of pSS should facilitate the development of new therapies. Recently, a trial showed the efficacy of the antifibrotic drug nintedanib in slowing progression of various interstitial lung diseases, including patients with connective tissue diseases. The aims of this review are to describe clinical features, imaging, pathology, together with diagnostic criteria, prognosis and management of pSS-ILD patients.


Assuntos
Doenças do Tecido Conjuntivo , Doenças Pulmonares Intersticiais , Síndrome de Sjogren , Humanos , Imunossupressores/uso terapêutico , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/tratamento farmacológico , Doenças Pulmonares Intersticiais/epidemiologia , Prognóstico , Síndrome de Sjogren/complicações , Síndrome de Sjogren/diagnóstico , Síndrome de Sjogren/tratamento farmacológico
9.
Clin Exp Rheumatol ; 38 Suppl 126(4): 103-109, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33095149

RESUMO

Primary Sjögren's syndrome (pSS) is a common systemic autoimmune disease characterised by exocrinopathy resulting in dryness of the mouth and eyes, unexplained fatigue and diffuse pain. Neurological involvement is uncommon in pSS, involving the central nervous system in 2-5% of cases and more frequently the peripheral nervous system in 5-15% of cases. The diagnosis of pSS is to be considered when confronted with symptoms such as mouth and eye dryness, fatigue and pain, the most frequent of pSS symptoms. Objective measures of oral and eye dryness may help assert the diagnosis of pSS, as well as ACR/EULAR criteria. Differential diagnoses have to be excluded in patients exhibiting neurological symptoms, such as cryoglobulinaemic vasculitis or multiple sclerosis, before considering a neurological involvement specific to pSS. The treatment of these neurological manifestations takes into account different parameters, such as the presence of cryoglobulinaemic vasculitis, the severity of the symptoms, a rapidly progressing evolution and the failure of previous symptomatic treatments.


Assuntos
Esclerose Múltipla , Síndrome de Sjogren , Diagnóstico Diferencial , Humanos , Dor , Sistema Nervoso Periférico , Síndrome de Sjogren/complicações , Síndrome de Sjogren/diagnóstico
10.
Clin Exp Rheumatol ; 38 Suppl 126(4): 73-77, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33025891

RESUMO

OBJECTIVES: Rheumatoid arthritis (RA) patients with Sjögren's syndrome (SS) often present with more severe synovitis. We intended to clarify the impact of overlapping SS on ultrasound remission, functional ability and clinical decision-making in RA patients in a real-world cohort from 2009 to 2019. METHODS: The medical records of RA patients in our medical centre from 2009 to 2019 were reviewed. Cox proportional hazards models of ultrasound remission and no disability (by health assessment questionnaire [HAQ]) were conducted in both the 1-to-1 nearest propensity score matched (PSM) and unmatched cohorts between RA patients with SS (RA-SS) and without (RA-noSS) to correct critical confounders. Four kinds of PSM methods were used and the corresponding average treatment effect on the treated (ATT) was calculated to clarify the effect of overlapping SS on distinguishable characteristics or drug prescription in RA patients. RESULTS: A total of 1100 RA patients were included in the study, of which 133 (12.1%) overlapped with SS. Among 256 patients consisting of 128 RA-SS and 128 RA-noSS after 1-to-1 nearest PSM, overlapping SS was associated with a 44%, 32% lower probability of reaching ultrasound remission, no disability in RA patients, respectively. More hydroxychloroquine (HCQ) usage, less biologic disease-modifying anti-rheumatic drugs (bDMARDs) prescription were confirmed to be correlated with overlapping SS by the robust PSM. CONCLUSIONS: Overlapping SS is associated with a lower probability of reaching ultrasound remission and no disability in RA patients. HCQ may still be the mainstream of clinical decision making in RA-SS patients.


Assuntos
Artrite Reumatoide , Síndrome de Sjogren , Atividades Cotidianas , Artrite Reumatoide/diagnóstico por imagem , Artrite Reumatoide/tratamento farmacológico , Humanos , Pontuação de Propensão , Síndrome de Sjogren/complicações , Síndrome de Sjogren/diagnóstico por imagem , Síndrome de Sjogren/tratamento farmacológico , Ultrassonografia
11.
Clin Exp Rheumatol ; 38 Suppl 126(4): 301-309, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33025899

RESUMO

Primary Sjögren's syndrome is a complex systemic autoimmune disorder that primarily affects exocrine glands such as the lacrimal glands. Dry eye disease is one of the most prevalent complications of Sjögren's syndrome, affecting most patients. It significantly impairs quality of life and management is often difficult and unsatisfactory, in part due to weak correlation between symptoms and signs and poor recognition of the three main subtypes aqueous-deficient, evaporative and neuropathic dry eye. This review provides an overview of key aspects of dry eye disease, such as its multifactorial aetiology and recent insights into pathophysiology. The uses and pitfalls of commonly-used diagnostic tests for dry eye are reviewed, as well as the increasing number of new imaging technologies and biomarkers to refine diagnosis. There are many current and emerging treatment options for dry eye in Sjögren's syndrome, but high-level evidence of efficacy is mostly lacking, as are evidence-based treatment algorithms. All these aspects make the management of dry eye in Sjögren's syndrome challenging.


Assuntos
Síndromes do Olho Seco , Aparelho Lacrimal , Síndrome de Sjogren , Síndromes do Olho Seco/diagnóstico , Síndromes do Olho Seco/etiologia , Síndromes do Olho Seco/terapia , Humanos , Aparelho Lacrimal/diagnóstico por imagem , Prevalência , Qualidade de Vida , Síndrome de Sjogren/complicações , Síndrome de Sjogren/diagnóstico , Síndrome de Sjogren/terapia
12.
Beijing Da Xue Xue Bao Yi Xue Ban ; 52(5): 886-891, 2020 Oct 18.
Artigo em Chinês | MEDLINE | ID: mdl-33047724

RESUMO

OBJECTIVE: To analyze the clinical features and prognosis in patients with primary Sjögren's syndrome (pSS) and autoimmune liver diseases (ALD). METHODS: A retrospective analysis of clinical manifestation and prognosis was performed in patients with ALD or without ALD during the three years (February 2014 to December 2017). RESULTS: Totally, 203 patients with pSS were included in this study, 68 patients had ALD (31 patients with autoimmune hepatitis, 37 patients with primary biliary cholangitis), while 135 patients did not have ALD. There were no differences between the two groups regarding age, gender, clinical manifestations, such as dry mouth, dry eyes, pain, fatigue, lymphadenopathy, glandular swelling, cutaneous involvement, lung involvement, and renal involvement, and the incidence rate of other autoimmune diseases, such as autoimmune thyroid disease, rheumatoid arthritis, and vasculitis. There were also no differences in the titer of antinuclear antibody (ANA), the positive rates of anti-Sjögren's syndrome A antibody (SSA), SSA52, and anti-Sjögren's syndrome B antibody (SSB), and at the levels of erythrocyte sedimentation rate and C-reactive protein between the two groups. Most importantly, the pSS patients with ALD had a shorter disease course, a higher positive rate of anti-mitochondrial M2 antibody (AMA-M2) and anti-centromere antibody, a higher level of IgG and IgM, a lower level of complement 3, and a decreased number of blood cells. They also had a higher level of liver related serum index, such as alanine aminotransferase, aspartate aminotransferase, gamma-glutamyl transferase, alkaline phosphatase and total bilirubin, direct bilirubin, indirect bilirubin, a higher incidence rate of liver cirrhosis, an increased death incident (the mortality was 13.24% in the pSS patients with ALD, while 2.96% in the controls, P=0.013), and a worse prognosis. Binary Logistic regression analysis revealed that liver cirrhosis, the EULAR Sjögren's syndrome disease activity index (ESSDAI) scores and the level of total bilirubin were the prognostic factors of mortality in the pSS patients with ALD. The survival curve was estimated by the Kaplan-Meier method. It demonstrated that the pSS patients with ALD had a lower survival rate when compared with the controls. CONCLUSION: The patients with both pSS and ALD will suffer from a more severe disease and a higher death incident. We should pay more attention to these patients and provide a better symptomatic treatment for them during clinical practice.


Assuntos
Hepatite Autoimune , Cirrose Hepática Biliar , Síndrome de Sjogren , Hepatite Autoimune/complicações , Hepatite Autoimune/diagnóstico , Hepatite Autoimune/epidemiologia , Humanos , Prognóstico , Estudos Retrospectivos , Síndrome de Sjogren/complicações , Síndrome de Sjogren/epidemiologia
13.
J Fr Ophtalmol ; 43(8): 691-696, 2020 Oct.
Artigo em Francês | MEDLINE | ID: mdl-32896450

RESUMO

PURPOSE: The goal of this study is to determine a link between benign essential blepharospasm and Sjogren's syndrome by analyzing the presence of extractable nuclear antigens in this population. METHODS: Seventy-two patients with benign essential blepharospasm (BEB) were included in this study. We eliminated patients with hemifacial spasm or blepharospasm secondary to corneal pathology. We collected the values of the Schirmer I test and the results of the anti-SSA and anti-SSB antibodies. RESULTS: Our study included 72 patients (144 eyes) whose 62 women (86.1%). Mean age was 74.3 years±10.73. Average Schirmer I test was 3.14mm±4.00mm. Five women (8% of this female population) had positive anti-SSA and SSB antibodies. Their mean age was 65.66 years±13.24 whereas the negative antibody patients had an average age of 75.42±9.27. There was no significant difference between their Schimer I test and the Schirmer I of negative antibody population. CONCLUSION: This study illustrates the possible association between the presence of Sjögren's syndrome and the occurrence of a BEB justifying the search for anti-SSA and anti SSB in blepharospasm patients.


Assuntos
Anticorpos Antinucleares/sangue , Antígenos Nucleares/imunologia , Blefarospasmo/sangue , Blefarospasmo/epidemiologia , Síndromes do Olho Seco/sangue , Síndromes do Olho Seco/epidemiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Blefarospasmo/complicações , Síndromes do Olho Seco/complicações , Feminino , França/epidemiologia , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Síndrome de Sjogren/sangue , Síndrome de Sjogren/complicações , Síndrome de Sjogren/epidemiologia
14.
PLoS One ; 15(9): e0239442, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32946501

RESUMO

BACKGROUND: Although it is known that patients with primary Sjögren's syndrome (pSS) have impaired dental conditions, incidence rates and incidence rate ratios of various dental diseases in these patients are not clear. The aim of this study was to investigate the frequency and prevalence of dental diseases in patients with pSS, and to evaluate the risk of common dental diseases in these patients. METHODS: A population-based retrospective cohort study was conducted using the data from the Taiwan's National Health Insurance Research Database. A total of 709 patients with newly diagnosed pSS between 2000 and 2012 were identified to form the pSS cohort. A comparison cohort of patients without pSS was assembled based on frequency matching for sex, 5-year age interval, and index year at a ratio of 10:1. All participants were followed until the end of the follow-up period or when the outcome of interest occurred. The incidence of dental caries, pulpitis, gingivitis, periodontitis, oral ulceration, and stomatitis were calculated using multiple Poisson regression models. RESULTS: A significantly higher prevalence (74.6% vs. 63.0%, P = 0.001) and frequency (median 5.37 vs. 1.45 per year, P < 0.001) dental visits were observed in patients with pSS compared with patients in the comparison cohort. The risk of dental caries (adjusted incidence rate ratio [aIRR] 1.64, P < 0.001), pulpitis (aIRR 1.42, P < 0.001), gingivitis (aIRR 1.43, P < 0.001), periodontitis (aIRR 1.44, P < 0.001), oral ulceration (aIRR 1.98, P < 0.001), and stomatitis (aIRR 2.06, P < 0.001) were significantly higher in patients with pSS. CONCLUSIONS: In this nationwide, population-based cohort study, a higher prevalence and frequency of dental visits were found in patients with pSS. Patients with PSS had increased risk of six most common dental disorders, including dental caries, pulpitis, gingivitis, periodontitis, oral ulceration, and stomatitis. Rheumatologists should remain vigilant for the dental health of patients with pSS.


Assuntos
Seguro Saúde/estatística & dados numéricos , Síndrome de Sjogren/complicações , Doenças Dentárias/complicações , Adulto , Estudos de Coortes , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Risco , Adulto Jovem
15.
PLoS One ; 15(9): e0239769, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32976549

RESUMO

PURPOSE: To report the significance of extraglandular ocular involvement and long-term systemic morbidity and mortality in primary Sjögren's Syndrome (SS). METHODS: This retrospective, longitudinal cohort study included consecutive patients with primary SS evaluated at a tertiary referral center. An electronic chart review was performed and all available data were extracted from clinic visits between October 1999 and March 2019. The primary outcome measures included occurrence of extraglandular ocular manifestations of SS, serological markers, prevalence of malignancy, and incidence of death. RESULTS: One hundred and twenty-six SS patients with minimum 3 years of follow-up (median 9.6, range 3.0-15.9 years, total of 1,235 patient-years) were included. Of those, 10 patients with inflammatory keratolysis or scleritis had 2.3 times greater likelihood of death compared to the rest of the cohort (OR = 2.3, 95% confidence interval [CI] 0.5 to 4.0, p = 0.01) due to SS related complications. The lifetime prevalence of any malignancy in the entire cohort was 15.5%. The most common hematologic malignancy was non-Hodgkin's lymphoma (4.8%) and the most common solid malignancy was breast cancer (6.0%). Men SS patients were more likely to have a history of or concurrent malignancy compared to women (30.0% versus 13.7%, p = 0.16) and double the mortality (OR = 2.1, 95% CI 0.09 to 1.4, p = 0.04), independent of malignancy. CONCLUSIONS: SS patients with serious ocular manifestations, particularly men, may be at greater risk for mortality due to SS complications. The eye seems to be the barometer of systemic disease activity.


Assuntos
Neoplasias da Mama/epidemiologia , Oftalmopatias/epidemiologia , Linfoma não Hodgkin/epidemiologia , Síndrome de Sjogren/complicações , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Síndrome de Sjogren/epidemiologia , Síndrome de Sjogren/mortalidade
17.
Medicine (Baltimore) ; 99(32): e21644, 2020 Aug 07.
Artigo em Inglês | MEDLINE | ID: mdl-32769933

RESUMO

INTRODUCTION: The kidney is one of the common extraglandular sites involved in primary Sjögren syndrome (pSS), with chronic tubulointerstitial nephritis (TIN) the most common pathology type. Renal involvement in pSS often presents as chronic TIN accompanied by type 1 or 2 renal tubular acidosis (RTA). Description of renal involvement as acute TIN with type III RTA in pSS has been rarely reported. PATIENT CONCERNS: A 37-year-old woman was admitted with complaints of dry mouth, dry eyes, and progressive muscle weakness for 17 months. Two months before admission, the patient had a blood potassium level of 1.7 mmol/L. DIAGNOSIS: Further tests confirmed pSS and type III RTA. Renal biopsy demonstrated acute TIN and thin basement membrane nephropathy (TBMN). INTERVENTIONS: Full-dose corticosteroid (1 mg/kg/day) and cyclophosphamide (100 mg/day) were applied. OUTCOMES: The creatinine levels of the patient decreased 0.28 mg/dL (1.18-0.90 mg/dL) during 3-month follow-up. CONCLUSIONS: We reported a patient with pSS-associated kidney injury, presenting as acute TIN with type 3 RTA and TBMN. This case increases the awareness of a rare manifestation of pSS-associated kidney injury. In pSS-associated acute TIN, cyclophosphamide combined with full-dose corticosteroids may achieve good outcomes.


Assuntos
Acidose Tubular Renal/etiologia , Nefrite Intersticial/etiologia , Síndrome de Sjogren/complicações , Acidose Tubular Renal/fisiopatologia , Corticosteroides/uso terapêutico , Adulto , Creatinina/análise , Creatinina/sangue , Ciclofosfamida/uso terapêutico , Síndromes do Olho Seco/etiologia , Feminino , Humanos , Debilidade Muscular/etiologia , Nefrite Intersticial/fisiopatologia , Nefrose/etiologia , Nefrose/fisiopatologia , Potássio/sangue , Síndrome de Sjogren/fisiopatologia
18.
BMC Oral Health ; 20(1): 187, 2020 07 03.
Artigo em Inglês | MEDLINE | ID: mdl-32620111

RESUMO

BACKGROUND: The aim of this study was to evaluate chemosensory function and oral disorders in patients with primary Sjögren's syndrome (pSS) and to compare these findings with those of age- and gender-matched healthy controls. METHODS: This comparative cross-sectional study included 58 patients with primary Sjögren's syndrome (pSS) and 55 age- and gender-matched healthy controls. Olfactory and gustatory function, burning sensations in the tongue (BST) and halitosis were assessed. Oral health-related quality of life (OHRQoL) was evaluated using the short-form Oral Health Impact Profile (OHIP-14). RESULTS: Patients with pSS had significantly lower self-reported visual analogue scale (VAS) smell score (8.6 ± 2.2 vs. 9.6 ± 0.7, p = 0.016) and VAS taste score (8.5 ± 2.1 vs. 9.5 ± 0.7, p = 0.014) than healthy controls. A greater proportion of patients with pSS had anosmia (3.8% vs. 0.0%) or hyposmia (36.5% vs. 13.2%) and ageusia for basic tastes: sweetness (34.0% vs. 7.5%), sourness (10.6% vs. 0.0), saltiness (10.0% vs. 5.7%) or bitterness (19.1% vs. 1.9%) as evaluated using Sniffin Sticks test and taste stripts, respectively. A higher proportion of pSS patients complained of dysgeusia (52.6% vs. 9.4%, p < 0.0001) and BST (45.6% vs. 0.0%, p < 0.0001), while similar number of patients with pSS and controls reported halitosis (31.6% vs. 28.3%, p = 0.434). The mean OHIP-14 score was significantly higher in patients with pSS (6.8 ± 7.0 vs. 2.3 ± 8.5, p < 0.001) indicating patients' poorer OHRQoL compared with controls. CONCLUSIONS: The majority of patients with pSS had impaired chemosensory function and indicators of oral health in comparison with the age- and gender-matched healthy controls. Further studies of oral hygiene habits and dietary intake of these patients are needed to ensure better management of oral health problems in patients with pSS.


Assuntos
Ageusia/etiologia , Transtornos do Olfato/etiologia , Saúde Bucal , Qualidade de Vida/psicologia , Saliva/metabolismo , Síndrome de Sjogren/complicações , Adulto , Ageusia/diagnóstico , Estudos de Casos e Controles , Estudos Transversais , Disgeusia/diagnóstico , Disgeusia/etiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Transtornos do Olfato/diagnóstico , Higiene Bucal , Síndrome de Sjogren/psicologia
19.
J Fr Ophtalmol ; 43(7): e211-e215, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32532571

RESUMO

PURPOSE: To study whether there is an association between benign essential blepharospasm and Sjögren's syndrome by analyzing the presence of antibodies to extractable nuclear antigens in this population. METHODS: Seventy-two patients with benign essential blepharospasm (BEB) were included in this study. We excluded patients with hemifacial spasm or blepharospasm secondary to known corneal pathology. We recorded results of Schirmer I testing as well as levels of anti-SSA/Ro and anti-SSB/La antibodies. RESULTS: Our study included 72 patients (144 eyes), of which 62 (86.1%) were women. The mean age was 74.3±10.73 years. The mean Schirmer I test result was 3.14±4.00mm. Five women (8% of this female population) were found to have positive anti-SSA/Ro and anti-SSB/La antibodies. Their mean age was 65.66±13.24 years, while the mean age of the antibody-negative patients was 75.42±9.27 years. There was no statistically significant difference between the Schirmer I tests of the antibody positive and negative patients. CONCLUSION: This study demonstrates a possible association between Sjögren's syndrome and benign essential blepharospasm, justifying anti-SSA/Ro and anti-SSB/La testing in these patients.


Assuntos
Anticorpos Antinucleares/sangue , Blefarospasmo/sangue , Blefarospasmo/epidemiologia , Síndromes do Olho Seco/sangue , Síndromes do Olho Seco/epidemiologia , Síndrome de Sjogren/sangue , Síndrome de Sjogren/epidemiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Anticorpos Antinucleares/análise , Antígenos Nucleares/imunologia , Blefarospasmo/complicações , Blefarospasmo/diagnóstico , Comorbidade , Síndromes do Olho Seco/complicações , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Síndrome de Sjogren/complicações , Síndrome de Sjogren/diagnóstico
20.
Artigo em Inglês | MEDLINE | ID: mdl-32455849

RESUMO

This study aimed to investigate the relationships among sicca symptoms, oral health conditions, salivary profiles and oral Candida in Sjögren's syndrome (SS) patients. Eighty-five SS patients (mean age = 50.5) and 40 healthy non-SS individuals (mean age = 51.4) were recruited. They self-completed the Sicca Symptoms Inventory (SSI). Decayed, missing and filled surface (DMFS) scores, salivary flow rates, pH and oral Candida colonization were determined. Mean SSI summary scores of SS patients and non-SS individuals were 11.1 and 5.4 respectively (p < 0.001). The most prevalent sicca symptoms in SS patients were eye irritation (93%), dry throat or nose (88%) and need of fluid for mouth wetting (88%). SS patients had significantly lower whole salivary flow rates than the non-SS individuals. Candida strains were isolated from over 60% of SS patients but not in non-SS patients. C. albicans was the predominant species. SSI summary score was negatively correlated to salivary flow rates while SSI summary and domain scores were positively correlated to the number of filled surfaces (FS) and DMFS scores and oral Candida counts. In conclusion, SS patients had more severe sicca symptoms than non-SS individuals. SSI scores were negatively correlated to the salivary flow rates but positively correlated to caries experience and oral Candida colonization.


Assuntos
Saúde Bucal , Síndrome de Sjogren , Candida , Estudos de Casos e Controles , Feminino , Nível de Saúde , Humanos , Masculino , Pessoa de Meia-Idade , Síndrome de Sjogren/complicações
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