Multiparametric magnetic resonance imaging of the liver and spleen in Gaucher disease.

PURPOSE: To assess liver and spleen characteristics of a population with Gaucher disease (GD) using multiparametric MRI and MR elastography (MRE) for evaluation of diffuse liver and spleen disease, which includes liver fat fraction, liver and spleen volume and iron deposition, and liver and spleen stiffness correlated with DS3 Severity Scoring System for Gaucher disease (GD-DS3). METHODS: We prospectively evaluated 41 patients with type 1 Gaucher disease using a 3.0 T MRI and MRE between January 2019 and February 2020. Clinical, laboratory, and imaging data was collected. Mann-Whitney, Kruskal-Wallis, and Spearman's correlation were applied to evaluate liver and spleen MRI and MRE, clinical and laboratory variables, and GD-DS3. ERT and SRT treatment groups were compared. RESULTS: Hepatomegaly was seen in 15% and splenomegaly in 42% of the population. Moderate and strong and correlations were found between liver and spleen iron overload (rho = 0.537; p = 0.002); between liver and spleen volume (rho = 0.692, p < 0.001) and between liver and spleen stiffness (rho = 0.453, p = 0.006). Moderate correlations were found between liver stiffness and GD-DS3 (rho = 0.559; p < 0.001) and between splenic volume and GD-DS3 (rho = 0.524; p = 0.001). CONCLUSION: The prevalence of hepatosplenomegaly, liver fibrosis, and liver iron overload in treated patients with GD is low, which may be related to the beneficial effect of treatment. Liver MRE and splenic volume correlate with severity score and may be biomarkers of disease severity.

A new and simple score to predict adequate and deep response to ursodeoxycholic acid in patients with primary biliary cholangitis: the ALP-A score.

BACKGROUND: Ursodeoxycholic acid (UDCA) is the standard treatment for primary biliary cholangitis (PBC), but a significant proportion of patients do not respond adequately, leading to increased risk of adverse outcomes. This study aims to develop a new and straightforward predictive score to identify PBC patients likely to achieve a complete response to UDCA. METHODS: A logistic regression analysis was conducted using a derivation cohort of PBC patients to identify pre-treatment variables associated with response to UDCA. This analysis led to the development of the ALP-A score, calculated as: Age at diagnosis divided by (alkaline phosphatase at diagnosis/upper limit of normal). ALP-A score accuracy was evaluated using the area under the ROC curve, validated with a large external cohort from Brazil. Additionally, the correlation between the ALP-A score and the previously validated UDCA response score (URS) was assessed. RESULTS: ALP-A score had good predictive power for adequate (AUC 0.794; 95% CI, 0.737-0.852) and deep (0.76; 95% CI, 0.69-0.83) UDCA response at 1 year of treatment. A cutoff score of 17 and 23 points was determined to be the optimal threshold for distinguishing adequate and deep responders, respectively, from non-responders. ALP-A score demonstrated a sensitivity of 73%, specificity of 71%, positive predictive value of 65%, negative predictive value of 78%, and overall accuracy of 72% for biochemical response. The URS displayed similar discriminative ability (AUC 0.798; 95% CI, 0.741-0.855). CONCLUSION: ALP-A score performs comparably to URS but offers the great advantage of simplicity for routine clinical use. It serves as a valuable tool to identify PBC patients less likely to respond to UDCA treatment, facilitating early consideration of alternative therapeutic approaches.

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.

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 (× ULN) among responders versus 5.05 ± 3.08 × 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.

Anti-mitochondrial Antibody-Negative Primary Biliary Cholangitis Is Part of the Same Spectrum of Classical Primary Biliary Cholangitis.

BACKGROUND: Primary biliary cholangitis (PBC) is a chronic cholestatic liver disease in which anti-mitochondrial antibodies (AMA) are the diagnostic hallmark. Whether AMA-negative PBC patients represent a different phenotype of disease is highly debated. AIMS: The purpose of our study was to compare AMA-positive and AMA-negative PBC patients in a large non-white admixed Brazilian cohort. METHODS: The Brazilian Cholestasis Study Group multicentre database was reviewed to assess demographics, clinical features and treatment outcomes of Brazilian PBC patients, stratifying data according to AMA status. RESULTS: A total of 464 subjects (95.4% females, mean age 56 ± 5 years) with PBC were included. Three hundred and eighty-four (83%) subjects were AMA-positive, whereas 80 (17%) had AMA-negative PBC. Subjects with AMA-negative PBC were significantly younger (52.2 ± 14 vs. 59.6 ± 11 years, p = 0.001) and had their first symptom at an earlier age (43.2 ± 13 vs. 49.5 ± 12 years, p = 0.005). Frequency of type 2 diabetes was significantly increased in subjects with AMA-negative PBC (22.5% vs. 12.2%, p = 0.03). Lower IgM (272.2 ± 183 vs. 383.2 ± 378 mg/dL, p = 0.01) and triglycerides (107.6 ± 59.8 vs.129.3 ± 75.7 mg/dL, p = 0.025) and higher bilirubin (3.8 ± 13.5 vs. 1.8 ± 3.4 mg/dL, p = 0.02) levels were also observed in this subgroup. Response to ursodeoxycholic acid varied from 40.5 to 63.3% in AMA-positive and 34 to 62.3% in AMA-negative individuals, according to different response criteria. Outcomes such as development of liver-related complications, death and requirement for liver transplantation were similar in both groups. CONCLUSIONS: AMA-negative PBC patients are similar to their AMA-positive counterparts with subtle differences observed in clinical and laboratory features.

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.

Clinical Features and Outcomes of Primary Sclerosing Cholangitis in the Highly Admixed Brazilian Population.

Background: Primary sclerosing cholangitis (PSC) is associated with a broad phenotypic spectrum in different populations from diverse ethnic and racial backgrounds. This study aimed to describe the clinical characteristics and outcomes of PSC in a multicenter cohort of patients from Brazil. Methods: Data from the Brazilian Cholestasis Study Group were retrospectively reviewed to assess demographic information and clinical characteristics of PSC, as well as the outcomes, such as transplantation-free survival. Results: This cohort included 210 patients. After excluding 33 (15.7%) patients with PSC and overlap syndrome of autoimmune hepatitis, 177 (97 males, median age 33 (21-42) years) with clear-cut PSC were eligible for this study. Most of the patients (n = 139, 78.5%) were symptomatic, and 104 (58.7%) had advanced PSC at the time of diagnosis. Concurrent inflammatory bowel disease was observed in 78 (58.6%) of the investigated patients (n = 133), and most of them had ulcerative colitis (n = 61, 78.2%). The 1- and 5-year survival free of liver transplantation or death were 92.3 ± 2.1% and 66.9 ± 4.2%, respectively, and baseline advanced PSC, pruritus, and elevated bilirubin levels were independent risk factors for the composite adverse outcome. Females were significantly older and had lower bilirubin levels than males at baseline, but survival was not associated with sex. Approximately 12.4% (n = 22) of patients with PSC died, and 32.8% (n = 58) underwent liver transplantation at a median follow-up time of 5.3 and 3.2 years. Conclusion: Multiethnic Brazilian PSC patients exhibited a less pronounced male predominance and a lower frequency of inflammatory bowel disease than Caucasians. Adverse outcomes were more frequent, probably due to advanced disease at baseline.

Fibrates for the Treatment of Primary Biliary Cholangitis Unresponsive to Ursodeoxycholic Acid: An Exploratory Study.

Aim: Up to 40% of patients with primary biliary cholangitis (PBC) will have a suboptimal biochemical response to ursodeoxycholic acid (UDCA), which can be improved by the addition of fibrates. This exploratory study aims to evaluate the long-term real-life biochemical response of different fibrates, including ciprofibrate, in subjects with UDCA-unresponsive PBC. Methods: The Brazilian Cholestasis Study Group multicenter database was reviewed to assess the response rates to UDCA plus fibrates in patients with UDCA-unresponsive PBC 1 and 2 years after treatment initiation by different validated criteria. Results: In total, 27 patients (100% women, mean age 48.9 ± 9.2 years) with PBC were included. Overall response rates to fibrates by each validated criterion varied from 39 to 60% and 39-76% at 12 and 24 months after treatment combination, respectively. Combination therapy resulted in a significant decrease in ALT and ALP only after 2 years, while GGT significantly improved in the first year of treatment. Treatment response rates at 1 and 2 years appear to be comparable between ciprofibrate and bezafibrate using all available criteria. Conclusion: Our findings endorse the efficacy of fibrate add-on treatment in PBC patients with suboptimal response to UDCA. Ciprofibrate appears to be at least as effective as bezafibrate and should be assessed in large clinical trials as a possibly new, cheaper, and promising option for treatment of UDCA-unresponsive PBC patients.

Resistance Mutations A30K and Y93N Associated with Treatment Failure with Sofosbuvir and Daclatasvir for Hepatitis C Virus Infection Non-Responder Patients: Case Reports.

In Brazil, hepatitis C treatment has been evolving significantly with the licensing of direct-acting antivirals (DAAs). However, viral determinants (amino acid substitutions in hepatitis C virus (HCV) genome and infective genotype) associated with host factors (hepatic condition and prior HCV therapy) might limit the achievement of sustained virologic response (SVR). Here, we described two case reports in which the occurrence of HCV NS5A mutations A30K (subtype 3a) and Y93N (subtype 1a) might have influenced daclatasvir (DCV)/sofosbuvir (SOF) combined therapy non-response. Despite high response rates for DAA combined therapies in Brazil, these case reports stated the importance of an investigation about how to manage a DAA treatment failure since a combination of factors, especially the occurrence of resistance substitutions, could impact a rescue therapy with new available antivirals in clinical routine.

Effects of the open lung concept following ARDSnet ventilation in patients with early ARDS.

BACKGROUND: Ventilation with low tidal volume (VT) is well recognized as a protective approach to patients with acute respiratory distress syndrome (ARDS), but the optimal level of positive end-expiratory pressure (PEEP) remains uncertain. This study aims to evaluate two protective ventilatory strategies sequentially applied in patients with early ARDS. METHODS: In this prospective cohort study, fifteen patients were ventilated during 24 h with positive end-expiratory pressure (PEEP) adjusted according to the ARDSnet low-PEEP table (ARDSnet-24 h). During the next 24 h, nine patients with PaO2/FIO2 ratio below 350 mmHg were ventilated with PEEP titrated according to the Open Lung Concept protocol (ARDSnet + OLC). In the other six patients, regardless of their PaO2/FIO2 ratio, the ARDSnet remained for a further 24 h (ARDSnet-48 h). Ventilatory variables, arterial blood-gas and cytokine were obtained at baseline, 24 and 48 h. Additionally, whole-lung-computed tomography was acquired at 24 and 48 h. RESULTS: A sustained improvement in PaO2/FIO2 ratio (P = 0.008) with a decrease in collapsed regions (P = 0.008) was observed in the ARDSnet + OLC group compared with the ARDSnet-24 h group. A reduction in IL-6 in plasma (P < 0.02) was observed throughout the protocol in the ARDSnet + OLC group. Compared with the ARDSnet-48 h group, the ARDSnet + OLC presented smaller amounts of collapsed areas (P = 0.018) without significant differences in hyperinflated regions and in driving and plateau pressures. CONCLUSIONS: In this set of patients with early ARDS, mechanical ventilation with an individually tailored PEEP sustained improved pulmonary function with better aeration, without significant increase in hyperinflated areas". TRIAL REGISTRATION: Brazilian Clinical Trials Registry (ReBec). RBR-5zm9pr. 04th November 2015.

Avaliação de estratégia ventilatória sequencial em pacientes com lesão pulmonar aguda (LPA) / síndrome do desconforto respiratório agudo (SDRA) / Effects of open lung concept following ARDSNet ventilatory strategy in patients with early acute lung injury (ALI) / acute respiratory distress syndrome (ARDS)

A utilização de baixos volumes correntes na Injuria Pulmonar Aguda (LPA) / Síndrome do Desconforto Respiratório Agudo (SDRA) está comprovadamente associada à redução de mortalidade. No entanto, a aplicação de manobras de recrutamento e a utilização de níveis elevados de PEEP ainda são controversos. O objetivo do presente estudo foi comparar os efeitos da estratégia ARDSnet e de uma estratégia baseada no Open Lung Concept (OLC), aplicadas de forma seqüencial, com relação à função pulmonar, imagem tomográfica e atividade inflamatória, em pacientes com LPA / SDRA. Dez pacientes que preencheram os critérios de LPA / SDRA segundo a Conferência de Consenso de 1994 com tempo de evolução até 48 horas foram incluídos. Para seleção definitiva, gasometria arterial para cálculo da relação PaO2/FIO2 foi coletada após 30 minutos de ventilação com volume corrente (VT) = 10 mL/kg, PEEP=5 cmH2O e FIO2 = 100%. Nas primeiras 24 horas os pacientes foram ventilados segundo o protocolo ARDSnet. Após este período, caso PaO2/FIO2 350, adotava-se a estratégia de OLC, que consistia na realização de manobra de recrutamento e titulação de PEEP. A manobra de recrutamento foi realizada em PCV, com delta de pressão de 20 cmH2O, com incrementos seqüenciais de PEEP em 5 cmH2O, partindo-se de 20 cmH2O até 30 cmH2O. O objetivo durante a titulação de PEEP foi alcançar PaO2/FIO2 > 350, sendo três níveis testados (17, 19 e 21 cmH2O). Ventilação segundo OLC (com PEEP determinado durante a titulação e VT = 6 ml/kg) foi mantida por 24 h adicionais. Após 24 h de cada estratégia, TC de todo o pulmão (1,25 mm de espessura com 15 mm de espassamento) foi realizada após 24 h de cada estratégia. A instituição de OLC foi necessária em 9 dos 10 pacientes estudados. PEEP foi significativamente superior com OLC (17 [17 - 19] vs. 8 cmH2O [7,25 - 11]; p = 0,007) e resultou em melhora significativa de oxigenação, sustentada após 24 h de seguimento, sem diferença na pressão de platô, pressão de distensão...

Low tidal volumes are associated with a reduction in mortality in ALI / ARDS. Nevertheless, the application of recruitment maneuvers and high levels of PEEP are still controversial. The aim of this study was to compare the ARDSnet protocol with a strategy based on Open Lung Concept (OLC), applied in a sequential way, in terms of pulmonary function, computed tomography images and inflammation, in patients with ALI / ARDS. Ten patients fulfilling criteria for ALI /ARDS, based on the American-European Consensus Conference, with less than 48 hours of evolution, were included. For definitive selection, blood gas collected after 30 min application of 5 cmH2O PEEP and VT = 10 mL/kg had to demonstrate a PaO2/FIO2 < 300 mmHg. The patients were initially ventilated for 24 hours according to the ARDSnet protocol. After this period, if the PaO2/FIO2 was 350, an OLC strategy was adopted, with recruitment maneuver and PEEP titration. The recruitment maneuver was applied in PCV, with sequential 5 cmH2O increments in PEEP, starting from 20 cmH2O until PEEP = 30 cmH2O, maintaining a delta pressure of 20 cmH2O. The aim of PEEP titration was to reach PaO2/FIO2 > 350 and three levels were tested: 17, 19 and 21 cmH2O. Ventilation according to OLC (VT = 6 mL/kg and PEEP´s level found during titration) was applied for the next 24 hours. Whole lung computed tomography images (1.25 mm thickness with 15mm gap) were acquired after 24 hours of each strategy. The institution of OLC was necessary in 9 of the 10 studied patients. The PEEP was significantly higher during OLC (17 [17-19] vs 8 cmH2O [8-11]; p=0,007) and resulted in a significant improvement on oxygenation sustained for 24 hours of follow-up, with no significant differences in plateau pressure, static compliance, minute-ventilation, PaCO2 and pH (p > 0.05). OLC resulted in a significant reduction of the fraction of total lung volume that was non-aerated, as compared to ARDSnet protocol...

Ventilação mecânica na lesão pulmonar aguda / síndrome do desconforto respiratório agudo / Mechanical ventilation in the acute lung injury/acute respiratory distress syndrome

JUSTIFICATIVA E OBJETIVOS: Em 2000, foi publicado o II Consenso Brasileiro de Ventilação Mecânica. Desde então, o conhecimento na área da ventilação mecânica avançou rapidamente, com a publicação de inúmeros estudos clínicos que acrescentaram informações importantes para o manuseio de pacientes críticos em ventilação artificial. Além disso, a expansão do conceito de Medicina Baseada em Evidências determinou a hierarquização das recomendações clínicas, segundo o rigor metodológico dos estudos que as embasaram. Essa abordagem explícita vem ampliando a compreensão e a aplicação das recomendações clínicas. Por esses motivos, a AMIB - Associação de Medicina Intensiva Brasileira - e a SBPT - Sociedade Brasileira de Pneumologia e Tisiologia - julgaram conveniente a atualização das recomendações descritas no Consenso anterior. Dentre os tópicos selecionados a Ventilação Mecânica na Síndrome do Desconforto Respiratório Agudo (SDRA) foi um dos temas propostos. O objetivo foi descrever os pontos mais importantes relacionados à ventilação mecânica na Síndrome do Desconforto Respiratório Agudo e discutir o papel das estratégias protetoras aplicada a esses pacientes. MÉTODO: Objetivou-se chegar a um documento suficientemente sintético, que refletisse a melhor evidência disponível na literatura. A revisão bibliográfica baseou-se na busca de estudos através de palavras-chave e em sua gradação conforme níveis de evidência. As palavras-chave utilizadas para a busca foram: mechanical ventilation e acute respiratory distress syndrome. RESULTADOS: São apresentadas recomendações quanto à utilização das estratégias protetoras (uso de baixos volumes-correntes e limitação da pressão de platô inspiratório), assim como, o estado atual da aplicação da PEEP e o papel das manobras de recrutamento. CONCLUSÕES: A ventilação mecânica na SDRA apresentou muitas mudanças nesses últimos anos e o uso de estratégias ventilatórias que preservem a micro-arquitetura pulmonar...

BACKGROUND AND OBJECTIVES: The II Brazilian Consensus Conference on Mechanical Ventilation was published in 2000. Knowledge on the field of mechanical ventilation evolved rapidly since then, with the publication of numerous clinical studies with potential impact on the ventilatory management of critically ill patients. Moreover, the evolving concept of evidence - based medicine determined the grading of clinical recommendations according to the methodological value of the studies on which they are based. This explicit approach has broadened the understanding and adoption of clinical recommendations. For these reasons, AMIB - Associação de Medicina Intensiva Brasileira and SBPT - Sociedade Brasileira de Pneumologia e Tisiologia - decided to update the recommendations of the II Brazilian Consensus. Acute Respiratory Distress Syndrome (ARDS) has been one of the updated topics. This objective was described the most important topics related to mechanical ventilation in patients with acute respiratory distress syndrome. METHODS: Systematic review of the published literature and gradation of the studies in levels of evidence, using the key words mechanical ventilation and acute respiratory distress syndrome. RESULTS: Recommendations on the use of lung protective strategies during mechanical ventilation based on reduced tidal volumes and limitation of plateau pressure. The state of the art of recruitment maneuvers and PEEP titration is also discussed. CONCLUSIONS: The mechanical ventilation of patients with ADRS changed in the last few years. We presented the role of lung protective strategies that could be applied to these patients.

[Mechanical ventilation in the acute lung injury/acute respiratory distress syndrome]. / Ventilação mecânica na lesão pulmonar aguda / síndrome do desconforto respiratório agudo.

BACKGROUND AND OBJECTIVES: The II Brazilian Consensus Conference on Mechanical Ventilation was published in 2000. Knowledge on the field of mechanical ventilation evolved rapidly since then, with the publication of numerous clinical studies with potential impact on the ventilatory management of critically ill patients. Moreover, the evolving concept of evidence - based medicine determined the grading of clinical recommendations according to the methodological value of the studies on which they are based. This explicit approach has broadened the understanding and adoption of clinical recommendations. For these reasons, AMIB - Associação de Medicina Intensiva Brasileira and SBPT - Sociedade Brasileira de Pneumologia e Tisiologia - decided to update the recommendations of the II Brazilian Consensus. Acute Respiratory Distress Syndrome (ARDS) has been one of the updated topics. This objective was described the most important topics related to mechanical ventilation in patients with acute respiratory distress syndrome. METHODS: Systematic review of the published literature and gradation of the studies in levels of evidence, using the key words mechanical ventilation and acute respiratory distress syndrome. RESULTS: Recommendations on the use of lung protective strategies during mechanical ventilation based on reduced tidal volumes and limitation of plateau pressure. The state of the art of recruitment maneuvers and PEEP titration is also discussed. CONCLUSIONS: The mechanical ventilation of patients with ADRS changed in the last few years. We presented the role of lung protective strategies that could be applied to these patients.