Recommendations for evaluation and diagnosis of extra-glandular manifestations of primary Sjögren syndrome: results of an epidemiologic systematic review/meta-analysis and a consensus guideline from the Brazilian society of rheumatology (hepatic, gastrointestinal and pancreatic).

Sjogren's syndrome (SS) is an autoimmune disease characterized by lymphocytic infiltration of the exocrine glands and other organs, associated with sicca syndrome but also with systemic involvement with varying degrees of severity. Despite their importance, some systemic manifestations, mainly liver, gastrointestinal, and pancreatic are not routinely evaluated. To address these manifestations, the Sjögren's Syndrome Committee of the Brazilian Society of Rheumatology conducted a broad systematic review of the literature on studies investigating prevalence and diagnosis of these symptoms in Sjogren´s patients and made recommendations based on the findings. Agreement between the experts was achieved using the Delphi method. This is the second part of this guideline, providing 6 recommendations for liver, gastrointestinal, and pancreatic care of SS patients.

Recommendations for evaluation and diagnosis of extra-glandular manifestations of primary Sjögren syndrome: results of an epidemiologic systematic review/ meta-analysis and a consensus guideline from the Brazilian society of rheumatology (hepatic, gastrointestinal and pancreatic)

Abstract Sjogren's syndrome (SS) is an autoimmune disease characterized by lymphocytic infiltration of the exocrine glands and other organs, associated with sicca syndrome but also with systemic involvement with varying degrees of severity. Despite their importance, some systemic manifestations, mainly liver, gastrointestinal, and pancreatic are not routinely evaluated. To address these manifestations, the Sjögren's Syndrome Committee of the Brazilian Society of Rheumatology conducted a broad systematic review of the literature on studies investigating prevalence and diagnosis of these symptoms in Sjogren´s patients and made recommendations based on the findings. Agreement between the experts was achieved using the Delphi method. This is the second part of this guideline, providing 6 recommendations for liver, gastrointestinal, and pancreatic care of SS patients.

Capillary loss on nailfold capillary microscopy is associated with mortality in systemic sclerosis.

The objective of this study is to test the association of the severity of nailfold capillaroscopy (NFC) abnormalities with mortality in systemic sclerosis (SSc). One hundred and seventy SSc patients underwent an extensive evaluation (including high-resolution computed tomography, pulmonary function tests, and Doppler echocardiography) at baseline following a standard protocol. Capillary loss on NFC was evaluated using the avascular score (AS, ranging from 0 to 3), and the mean number of ectasias, megacapillaries, and hemorrhages per finger was also recorded. After a mean period of 10.1 ± 4.9 years, the life status of the patients was ascertained. Univariate and multivariate Cox proportional hazards models were used for statistical analysis. Overall, 73 patients died. By univariate Cox analysis, the AS was significantly associated with mortality (hazard ratio [HR] = 1.64, 95% CI 1.22 to 2.19, p = 0.001). In our study, this association was stronger than that of race, gender, anticentromere antibodies, anti-topoisomerase I antibodies, and form of disease and had similar strength to that of skin score in univariate analyses. However, after controlling for a combination of variables (age, skin score, gender, race, signs of peripheral ischemia, and extent of interstitial lung disease, all independently associated with mortality), the association of AS with mortality was blunted (HR = 1.15, 95% CI 0.80 to 1.65, p = 0.445). Other NFC variables were not related to mortality. AS was associated with higher risk of death and, despite not having an independent association with mortality after controlling for a set of demographic and clinical variables, may be a useful tool in prognostic evaluation of SSc.

TCRBV20S1 and TCRBV3S1 gene segment polymorphisms in systemic sclerosis.

OBJECTIVE: To compare the frequencies of variants of TCRBV20S1 and TCRBV3S1 gene segments in patients with systemic sclerosis (SSc) and in controls. The null allele (allele 2) of TCRBV20S1 is associated with reduced levels of Vbeta20+ T-cells in the peripheral blood, while allele 1 of TCRBV3S1 is related to a low frequency of Vbeta3.1+ T-cells. METHODS: One hundred thirty patients with SSc and 118 healthy volunteer controls were genotyped for TCRBV20S1, and 117 patients and 85 controls were genotyped for TCRBV3S1 variants by PCR-RFLP. Patients underwent clinical evaluation, serology, pulmonary function tests, high resolution computed tomography, and Doppler echocardiography. RESULTS: The genotypic frequencies of TCRBV20S1 were 0.46 (allele 1/allele 1), 0.43 (allele 1/allele 2), and 0.11 (allele 2/allele 2) in SSc patients; in controls the frequencies were 0.70, 0.26, and 0.04, respectively (p < 0.001). The Mantel-Haenszel odds ratio (stratified by race and sex) of the allele 2 carrier state was 3.88 (95% CI 1.94 to 7.75). The allelic and genotypic frequencies of the TCRBV3S1 gene segment did not differ significantly in patients and controls. However, among patients, allele 1 (TCRBV3S1) carriers had a higher prevalence of interstitial lung disease (adjusted p = 0.032). CONCLUSION: The null allele of the TCRBV20S1 and the allele 1 of TCRBV3S1 gene segments may be considered risk factors for the development of SSc and interstitial lung disease, respectively, suggesting a protective role of Vbeta20+ and Vbeta3.1+ cells in the pathogenic immune responses in SSc.

Nailfold capillary microscopy can suggest pulmonary disease activity in systemic sclerosis.

OBJECTIVE: To evaluate the association of capillaroscopic alterations with pulmonary disease activity in systemic sclerosis (SSc). METHODS: Ninety-one patients with SSc were studied by means of interview, physical examination, nailfold capillary microscopy (NCM), serology, pulmonary function tests, esophageal transit scintigraphy, Doppler echocardiography, and pulmonary high resolution computed tomography (HRCT). Pulmonary disease activity was diagnosed by the observation of ground-glass opacities on pulmonary HRCT. Capillary loss on NCM was evaluated using the avascular score: patients with mean score > or = 1 or mean number of megacapillaries per finger > or = 1 were considered to have severe capillaroscopic alterations. RESULTS: Patients with higher skin scores, longer disease duration, signs of peripheral ischemia, esophageal dysfunction, antitopoisomerase I antibodies, and ground-glass opacities had higher mean avascular scores (p < or = 0.05 in all tests). The association between ground-glass opacities and higher avascular scores was particularly strong in patients with disease duration < or = 5 years. Among these patients, ground-glass opacities were present in 14 of 19 patients with severe NCM alterations, but were absent in all patients (n = 8) with mild or no NCM alterations (p < 0.001). ROC curves confirmed the ability of NCM to discriminate between patients with and without ground-glass opacities among those with disease duration < or = 5 years. However, NCM could not predict the presence of reduced pulmonary diffusing capacity. CONCLUSION: The severity of NCM abnormalities is associated with lung disease activity in SSc, particularly when the disease duration is relatively short.

Capilaroscopia periungueal pode sugerir atividade de doença pulmonar na esclerose sistêmica / Nailfold capillaroscopy can predict active pulmonary involvement in systemic sclerosis

Objetivo: Estudar a associação entre alterações capilaroscópicas e lesões em órgãos-alvo na esclerose sistêmica (ES). Métodos: Noventa e um pacientes com ES foram avaliados através de entrevista, exame físico, capilaroscopia periungueal (CPU), sorologia, provas de função pulmonar, cintilografia de trânsito esofágico, ecocardiografia com Doppler e tomografia computadorizada de alta resolução pulmonar (TCAR). A deleção capilar na CPU foi avaliada usando o escore de deleção vascular de Lee; consideram-se alterações severas na CPU um escore médio de deleção maior e igual 1 ou número médio de megacapilares por dedo maior e igual 1 para fins de análise. Hipertensão arterial pulmonar (HAP) foi definida como pressão sistólica na artéria pulmonar maior e igual 40 mmHg. Resultados: Pacientes com alterações capilaroscópicas severas apresentaram maior prevalência de áreas de opacidade em vidro-fosco (OVF) (P=0,016), redução da capacidade difusional pulmonar (P=0,026) e disfunção esofágica (P=0,001). HAP ocorreu somente em pacientes com alterações severas na CPU (P=0,114). Naqueles com duração de doença menor e igual 5 anos, OVF estavam presentes em 14 de 19 pacientes com alterações severas na CPU, mas não estavam presentes nos 8 pacientes com alterações capilaroscópicas leves ou ausentes (<0,001). Nesse subgrupo, nenhuma outra variável clínica ou laboratorial associou-se à presença de OVF. O uso de curvas ROC mostrou uma boa capacidade de CPU em discriminar pacientes com e sem o desfecho combinado de HAP ou doença intersticial pulmonar em pacientes com duração de doença menor e igual 5 anos. Conclusões: A severidade das anormalidade da CPU está associada com dano em órgãos-alvo na ES. A CPU pode indicar a presença de doença pulmonar ativa (representada por áreas de opacidade em vidro-fosco na TCAR) na ES de duração relativamente curta.

Using cyclosporin A in the rheumatology practice

The treatment of connective tissue diseases with cyclosporin-A is discussed with emphasis in nowadays literature and discuss some aspects like mechanism of

Neurite óptica associada a síndrome de Sjögren primária: relato de um caso e revisao da literatura / Optic reuritis associated to primary Sjögren's syndrome: Case report and review of literature

O envolvimento do sistema nervoso central (SNC) parece ser uma das complicaçoes importantes na síndrome de Sjögren primária. Muitas dessas manifestaçoes neurológicas assemelham-se ao quadro clínico da esclerose múltipla. Os autores relatam o caso de uma paciente com síndrome de Sjögren primária, cujo comprometimento do SNC, manifesto por neurite óptica isolada, procedeu em quatro anos o diagnóstico da doença básica

O laboratório em reumatologia / Laboratory in rheumatology

Os autores fazem uma revisäo das provas laboratoriais freqüentemente utilizadas na investigaçäo das doenças reumáticas, visando auxiliar o clínico näo especialista no diagnóstico destas doenças

Diagnóstico diferencial: febre reumática aguda versus endocardite infecciosa / Differential diagnosis: acute rheumatic fever versus infective endocarditis

Os autores fazem uma revisäo prática sobre o diagnóstico diferencial entre endocardite infecciosa e febre reumática aguda, considerando especialmente as manifestaçöes clínicas e exames complementares mais freqüentes