ATLANTIC EPIPHYTES: a data set of vascular and non-vascular epiphyte plants and lichens from the Atlantic Forest

Epiphytes are hyper-diverse and one of the frequently undervalued life forms in plant surveys and biodiversity inventories. Epiphytes of the Atlantic Forest, one of the most endangered ecosystems in the world, have high endemism and radiated recently in the Pliocene. We aimed to (1) compile an extensive Atlantic Forest data set on vascular, non-vascular plants (including hemiepiphytes), and lichen epiphyte species occurrence and abundance; (2) describe the epiphyte distribution in the Atlantic Forest, in order to indicate future sampling efforts. Our work presents the first epiphyte data set with information on abundance and occurrence of epiphyte phorophyte species. All data compiled here come from three main sources provided by the authors: published sources (comprising peer-reviewed articles, books, and theses), unpublished data, and herbarium data. We compiled a data set composed of 2,095 species, from 89,270 holo/hemiepiphyte records, in the Atlantic Forest of Brazil, Argentina, Paraguay, and Uruguay, recorded from 1824 to early 2018. Most of the records were from qualitative data (occurrence only, 88%), well distributed throughout the Atlantic Forest. For quantitative records, the most common sampling method was individual trees (71%), followed by plot sampling (19%), and transect sampling (10%). Angiosperms (81%) were the most frequently registered group, and Bromeliaceae and Orchidaceae were the families with the greatest number of records (27,272 and 21,945, respectively). Ferns and Lycophytes presented fewer records than Angiosperms, and Polypodiaceae were the most recorded family, and more concentrated in the Southern and Southeastern regions. Data on non-vascular plants and lichens were scarce, with a few disjunct records concentrated in the Northeastern region of the Atlantic Forest. For all non-vascular plant records, Lejeuneaceae, a family of liverworts, was the most recorded family. We hope that our effort to organize scattered epiphyte data help advance the knowledge of epiphyte ecology, as well as our understanding of macroecological and biogeographical patterns in the Atlantic Forest. No copyright restrictions are associated with the data set. Please cite this Ecology Data Paper if the data are used in publication and teaching events. © 2019 The Authors. Ecology © 2019 The Ecological Society of Americ

Avaliacao de escores prognosticos e de resposta ao tratamento com acido ursodesoxicolico em pacientes com diagnostico de colangite biliar primaria

Introduction: Primary biliary cholangitis (PBC) is a chronic autoimmune liver disease that affects predominantly women in the postmenopausal period. Ursodeoxycholic acid (UDCA) is the election treatment that leads in those who has biochemical response to a reduction in disease progression and increased transplant-­free survival. Objective: To describe epidemiological characteristics and clinical and laboratory presentation of PBC population in a reference service, to apply the response criteria to UDCA treatment and prognostic criteria;; to apply pretreatment response score and analyze clinical and laboratory factors that are correlate with prediction of biochemical response. Material and methods: This is an observational, cross-­sectional and descriptive epidemiological study conducted at the Hepatology Outpatient Clinic of Hospital São Paulo (EPM/ Unifesp), where were included patients with PBC from July 1999 to March 2019. The data were obtained through medical records analysis and entered into a database and analyzed using the IBM SPSS program. Results: 83 individuals were included, with a proportion of 96.4% (80) women with a mean age of 53.46 years. The main presentation-­related symptom was pruritus, present in 51.8% (43) of the patients. We had positive ANA in 88% of cases with disease-­specific pattern in 55.4% (46). The AMA was positive in 86.7% (72) of the individuals. Regarding the biochemical response, 74.7% (62) answered by the Paris II criteria. Characteristics such as ascites (p = 0.01), splenomegaly (p = 0.003), signs of portal hypertension (p = 0.003) and ductopenia (p = 0.003) were related to worse biochemical response. In binary logistic regression analysis we identified age (p = 0.043) and alkaline phosphatase x UNL levels (p = 0.012) as relevant to predict response to treatment with UDCA and that provided us with the construction of a new index for predict biochemical response, the IFA index. The IFA index shows the same accuracy of the UDCA -­ Response Score with an AUROC of 0.883 and a positive correlation with rho=0.956. Conclusion: Those patients with signs of advanced liver disease require close clinical surveillance and rigorous biochemical evaluation of response. The response prediction model has shown diagnostic accuracy comparable to the UDCA -­ Response Index for estimating biochemical response at 1 year and can be used in future studies to assess the impact of combination therapies on patients with low probability of response to UDCA monotherapy.Introdução: A colangite biliar primária (CBP) é uma doença hepática crônica autoimune que acomete de forma mais frequente mulheres no período após a menopausa. O ácido ursodesoxicólico (UDCA) é o tratamento de eleição nessa condição e naqueles que respondem ao tratamento leva a redução na progressão de doença e aumento da sobrevida livre de transplante. Objetivo: Descrever as características epidemiológicas e apresentação clínico-­laboratorial da população com CBP de um serviço de referência, aplicar os critérios de resposta ao tratamento com ácido UDCA e os critério de prognósticos;; aplicar o escore de resposta pré tratamento e analisar fatores clínico laboratoriais que se correlacionem com predição de resposta bioquímica. Material e métodos: Trata-­se de um estudo observacional epidemiológico, transversal e descritivo realizado no ambulatório de Hepatologia do Hospital São Paulo (EPM/Unifesp) onde foram avaliados pacientes portadores de CBP no período de julho de 1999 a março de 2019. Os dados foram retirados através de análise de prontuários e inseridos em um banco de dados e analisados com o programa IBM SPSS. Resultados: Foram incluídos 83 indivíduos, com uma proporção de 96,4% (80) de mulheres com média de idade 53,46 anos. O principal sintoma relatado na apresentação foi prurido, presente em 51,8% (43) dos pacientes. Obtivemos FAN positivo em 88% dos indivíduos com padrão específico para doença em 55,4% (46). O AMA foi positivo em 86,7% (72) dos indivíduos. Em relação a resposta bioquímica, 74,7% (62) apresentaram resposta segundo o critério de Paris II. Características como ascite (p=0,01), esplenomegalia (p=0,003), sinais de hipertensão portal (p=0,003) e ductopenia (p=0,003) foram relacionados a pior resposta bioquímica. Em análise de regressão logística binária identificamos as variáveis idade (p=0,043) e níveis de fosfatase alcalina x LSN (p=0,012) como relevantes para predizer resposta ao tratamento o que nos proporcionou a construção de um novo índice para predizer resposta bioquímica, o índice IFA. O índice IFA apresentou acurácia semelhando ao UDCA -­ Response Score com uma AUROC de 0,883 e uma correlação positiva com rho de 0,956. Conclusão: xiv Aqueles pacientes com sinais de doença hepática avançada necessitam de melhor vigilância e avaliação rigorosa de resposta bioquímica. O modelo de predição de resposta mostrou acurácia diagnóstica comparável ao UDCA – Response Score para estimar resposta bioquímica em 1 ano e poderá ser utilizado em estudos futuros para avaliar o impacto de terapias combinadas em pacientes com baixa probabilidade de resposta a monoterapia com UDCA.Dados abertos - Sucupira - Teses e dissertações (2019

Clinical features and treatment outcomes of primary biliary cholangitis in a highly admixed population

Introduction and objectives: Little is known about primary biliary cholangitis (PBC) in non-whites. The purpose of this study was to evaluate clinical features and outcomes of PBC in a highly admixed population. Material and methods: The Brazilian Cholestasis Study Group multicentre database was reviewed to assess demographics, clinical features and treatment outcomes of Brazilian patients with PBC. Results: 562 patients (95% females, mean age 51 ± 11 years) with PBC were included. Concurrent autoimmune diseases and overlap with autoimmune hepatitis (AIH) occurred, respectively, in 18.9% and 14%. After a mean follow-up was 6.2 ± 5.3 years, 32% had cirrhosis, 7% underwent liver transplantation and 3% died of liver-related causes. 96% were treated with ursodeoxycholic acid (UDCA) and 12% required add-on therapy with fibrates, either bezafibrate, fenofibrate or ciprofibrate. Response to UDCA and to UDCA/fibrates therapy varied from 39%-67% and 42-61%, respectively, according to different validated criteria. Advanced histological stages and non-adherence to treatment were associated with primary non-response to UDCA, while lower baseline alkaline phosphatase (ALP) and aspartate aminotransferase (AST) levels correlated with better responses to both UDCA and UDCA/fibrates. Conclusions: Clinical features of PBC in highly admixed Brazilians were similar to those reported in Caucasians and Asians, but with inferior rates of overlap syndrome with AIH. Response to UDCA was lower than expected and inversely associated with histological stage and baseline AST and ALP levels. Most of patients benefited from add-on fibrates, including ciprofibrate. A huge heterogeneity in response to UDCA therapy according to available international criteria was observed and reinforces the need of global standardization

Response to Ursodeoxycholic Acid May Be Assessed Earlier to Allow Second-Line Therapy in Patients with Unresponsive Primary Biliary Cholangitis

Background Response to ursodeoxycholic acid (UDCA) in primary biliary cholangitis (PBC) has been traditionally assessed 1 to 2 years after treatment initiation. With the development of new drugs, some patients may benefit from an earlier introduction of second-line therapies. Aims This study aims to identify whether well-validated response criteria could correctly identify individuals likely to benefit from add-on second-line therapy at 6 months. Methods Analysis of a multicenter retrospective cohort which included only patients with clear-cut PBC. Results 206 patients with PBC (96.6% women; mean age 54 +/- 12 years) were included. Kappa concordance was substantial for Toronto (0.67), Rotterdam (0.65), Paris 1 (0.63) and 2 (0.63) criteria at 6 and 12 months, whereas Barcelona (0.47) and POISE trial (0.59) criteria exhibited moderate agreement. Non-response rates to UDCA was not statistically different when assessed either at 6 or 12 months using Toronto, Rotterdam or Paris 2 criteria. Those differences were even smaller or absent in those subjects with advanced PBC. Mean baseline alkaline phosphatase was 2.73 +/- 1.95 times the upper limit of normal (x ULN) among responders versus 5.05 +/- 3.08 x ULN in non-responders (p < 0.001). Conclusions After 6 months of treatment with UDCA, the absence of response by different criteria could properly identify patients who could benefit from early addition of second-line therapies, especially in patients with advanced disease or high baseline liver enzymes levels

Risk factors for cancer in patients with primary biliary cholangitis and autoimmune hepatitis and primary biliary cholangitis overlap syndrome

Introduction and objectives: Primary biliary cholangitis (PBC) and autoimmune hepatitis (AIH) and PBC overlap syndrome (AIH/PBC) have been associated with a higher risk of hepatocellular carcinoma (HCC) and extra-hepatic malignancy (EHM). This study aims to assess potential risk factors associated with cancer development in PBC and AIH/PBC. Materials and methods: The Brazilian Cholestasis Study Group database was reviewed to compare clinical and laboratory features of PBC patients with HCC and EHM with those without cancer. Results: Among the 752 PBC patients enrolled, 64 of them with AIH/PBC, 87 cancers were identified in 72 patients, including 20 cases of HCC and 67 of EHM. Patients with HCC had a higher prevalence of cirrhosis (95% vs. 32.5% of those subjects without cancer, p≤0.001), smoking (55% vs. 12.3%, p≤0.001), CREST syndrome (30% vs 7.6%, p=0.003) and prior azathioprine (30% vs 8%, p= 0.005) and prednisone (35% vs 14%, p= 0.018) use, whereas patients with EHM had a higher prevalence of smoking (42.3% vs 12.4% of those subjects without cancer, p= <0.001), AMA positivity (96.6% vs 80.1%, p≤0.001), azathioprine therapy (21% vs 7.9%, p= 0.01) and concurrent other autoimmune diseases. In multivariate analysis, cirrhosis, obesity and prior azathioprine therapy were independent risk factors for HCC, while Sjogren syndrome and psoriasis were associated with EHM. Fibrates reduced EHM risk. Conclusions: The prevalence of EHM is higher when compared to HCC in PBC patients. Cirrhosis, obesity, prior azathioprine use, and concurrent autoimmune diseases were significantly associated with cancer in PBC