RESUMO
Objectives- This report presents 2022 data on U.S. births by selected characteristics. Trends in fertility patterns and maternal and infant characteristics are described. Methods-Descriptive tabulations based on birth certificates of the 3.67 million births registered in 2022 are shown by maternal age, live-birth order, race and Hispanic origin, marital status, tobacco use, prenatal care, source of payment for the delivery, method of delivery, gestational age, birthweight, and plurality. Selected data by mother's state of residence and birth rates also are shown. Trends for 2010 to 2022 are presented for selected items, and by race and Hispanic origin for 2016-2022. Results-A total of 3,667,758 births occurred in the United States in 2022, essentially unchanged from 2021. The general fertility rate declined 1% from 2021 to 56.0 births per 1,000 females ages 15-44 in 2022. The birth rate for females ages 15-19 declined 2% from 2021 to 2022; birth rates fell 7% for women ages 20-24, rose 1% to 5% for women ages 25-29 and 35-44, and rose 12% for women ages 45-49 (the first increase since 2016). The total fertility rate declined less than 1% to 1,656.5 births per 1,000 women in 2022. Birth rates declined for unmarried women but increased for married women from 2021 to 2022. Prenatal care beginning in the first trimester declined to 77.0% in 2022; the percentage of women who smoked during pregnancy declined to 3.7%. The cesarean delivery rate was unchanged in 2022 (32.1%); Medicaid was the source of payment for 41.3% of births. The preterm birth rate declined 1% to 10.38%; the low birthweight rate rose 1% to 8.60%. The twin birth rate was unchanged in 2022 (31.2 per 1,000 births); the 2% decrease in the triplet and higher-order multiple birth rate.
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Gravidez na Adolescência , Nascimento Prematuro , Gravidez , Adolescente , Recém-Nascido , Humanos , Feminino , Estados Unidos/epidemiologia , Peso ao Nascer , Idade Materna , Recém-Nascido de Baixo Peso , Coeficiente de NatalidadeRESUMO
BACKGROUND: Phosphodiesterase 4 (PDE4) inhibition is associated with antiinflammatory and antifibrotic effects that may be beneficial in patients with idiopathic pulmonary fibrosis. METHODS: In this phase 2, double-blind, placebo-controlled trial, we investigated the efficacy and safety of BI 1015550, an oral preferential inhibitor of the PDE4B subtype, in patients with idiopathic pulmonary fibrosis. Patients were randomly assigned in a 2:1 ratio to receive BI 1015550 at a dose of 18 mg twice daily or placebo. The primary end point was the change from baseline in the forced vital capacity (FVC) at 12 weeks, which we analyzed with a Bayesian approach separately according to background nonuse or use of an antifibrotic agent. RESULTS: A total of 147 patients were randomly assigned to receive BI 1015550 or placebo. Among patients without background antifibrotic use, the median change in the FVC was 5.7 ml (95% credible interval, -39.1 to 50.5) in the BI 1015550 group and -81.7 ml (95% credible interval, -133.5 to -44.8) in the placebo group (median difference, 88.4 ml; 95% credible interval, 29.5 to 154.2; probability that BI 1015550 was superior to placebo, 0.998). Among patients with background antifibrotic use, the median change in the FVC was 2.7 ml (95% credible interval, -32.8 to 38.2) in the BI 1015550 group and -59.2 ml (95% credible interval, -111.8 to -17.9) in the placebo group (median difference, 62.4 ml; 95% credible interval, 6.3 to 125.5; probability that BI 1015550 was superior to placebo, 0.986). A mixed model with repeated measures analysis provided results that were consistent with those of the Bayesian analysis. The most frequent adverse event was diarrhea. A total of 13 patients discontinued BI 1015550 treatment owing to adverse events. The percentages of patients with serious adverse events or severe adverse events were similar in the two trial groups. CONCLUSIONS: In this placebo-controlled trial, treatment with BI 1015550, either alone or with background use of an antifibrotic agent, prevented a decrease in lung function in patients with idiopathic pulmonary fibrosis. (Funded by Boehringer Ingelheim; 1305-0013 ClinicalTrials.gov number, NCT04419506.).
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Fibrose Pulmonar Idiopática , Inibidores da Fosfodiesterase 4 , Teorema de Bayes , Nucleotídeo Cíclico Fosfodiesterase do Tipo 4 , Método Duplo-Cego , Humanos , Fibrose Pulmonar Idiopática/tratamento farmacológico , Fibrose Pulmonar Idiopática/fisiopatologia , Inibidores da Fosfodiesterase 4/uso terapêutico , Inibidores de Fosfodiesterase/uso terapêutico , Resultado do Tratamento , Capacidade Vital/fisiologiaRESUMO
Objectives-This report presents 2021 fetal mortality data by maternal race and Hispanic origin, age, tobacco use during pregnancy, and state of residence, as well as by plurality, sex, gestational age, birthweight, and selected causes of death. Trends in fetal mortality are also examined. Methods-Descriptive tabulations of data are presented and interpreted for all fetal deaths reported for the United States for 2021 with a stated or presumed period of gestation of 20 weeks or more. Cause-of-fetal-death data are restricted to residents of the 41 states and the District of Columbia, where cause of death was based on the 2003 fetal death report revision and less than 50% of deaths were attributed to Fetal death of unspecified cause (P95). Results-A total of 21,105 fetal deaths at 20 weeks of gestation or more were reported in the United States in 2021. The 2021 U.S. fetal mortality rate was 5.73 fetal deaths at 20 weeks of gestation or more per 1,000 live births and fetal deaths, which was essentially unchanged from the rate of 5.74 in 2020. The fetal mortality rate in 2021 for deaths occurring at 20-27 weeks of gestation was 2.95, essentially unchanged from 2020 (2.97). For deaths occurring at 28 weeks of gestation or more, the rate in 2021 (2.80) was not significantly different from 2020 (2.78). In 2021, the fetal mortality rate ranged from 3.94 for non-Hispanic, single-race Asian women to 9.89 for non-Hispanic, single-race Black women. Fetal mortality rates were highest for females under age 15 and aged 40 and over, for women who smoked during pregnancy, and for women with multiple gestation pregnancies. Five selected causes accounted for 89.9% of fetal deaths in the 41-state and District of Columbia reporting area.
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Etnicidade , Mortalidade Fetal , Adolescente , Adulto , Feminino , Humanos , Pessoa de Meia-Idade , Gravidez , District of Columbia/epidemiologia , Morte Fetal , Hispânico ou Latino , Estados Unidos/epidemiologia , Fatores Etários , Asiático , Negro ou Afro-AmericanoRESUMO
Objectives-This report presents 2021 data on U.S. births according to a variety of characteristics. Trends in fertility patterns and maternal and infant characteristics are described and interpreted.
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Gravidez na Adolescência , Gravidez , Feminino , Adolescente , Humanos , Estados Unidos/epidemiologia , Idade Materna , Coeficiente de Natalidade , Declaração de Nascimento , PartoRESUMO
Rationale: Idiopathic pulmonary fibrosis (IPF) is a rare and progressive disease that causes progressive cough, exertional dyspnea, impaired quality of life, and death. Objectives: Bexotegrast (PLN-74809) is an oral, once-daily, investigational drug in development for the treatment of IPF. Methods: This Phase-2a multicenter, clinical trial randomized participants with IPF to receive, orally and once daily, bexotegrast at 40 mg, 80 mg, 160 mg, or 320 mg, or placebo, with or without background IPF therapy (pirfenidone or nintedanib), in an approximately 3:1 ratio in each bexotegrast dose cohort, for at least 12 weeks. The primary endpoint was incidence of treatment-emergent adverse events (TEAEs). Exploratory efficacy endpoints included change from baseline in FVC, quantitative lung fibrosis (QLF) extent (%), and changes from baseline in fibrosis-related biomarkers. Measurements and Main Results: Bexotegrast was well tolerated, with similar rates of TEAEs in the pooled bexotegrast and placebo groups (62/89 [69.7%] and 21/31 [67.7%], respectively). Diarrhea was the most common TEAE; most participants with diarrhea also received nintedanib. Participants who were treated with bexotegrast experienced a reduction in FVC decline over 12 weeks compared with those who received placebo, with or without background therapy. A dose-dependent antifibrotic effect of bexotegrast was observed with QLF imaging, and a decrease in fibrosis-associated biomarkers was observed with bexotegrast versus placebo. Conclusions: Bexotegrast demonstrated a favorable safety and tolerability profile, up to 12 weeks for the doses studied. Exploratory analyses suggest an antifibrotic effect according to FVC, QLF imaging, and circulating levels of fibrosis biomarkers. Clinical trial registered with www.clinicaltrials.gov (NCT04396756).
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Fibrose Pulmonar Idiopática , Indóis , Piridonas , Humanos , Fibrose Pulmonar Idiopática/tratamento farmacológico , Fibrose Pulmonar Idiopática/fisiopatologia , Masculino , Feminino , Idoso , Pessoa de Meia-Idade , Indóis/uso terapêutico , Piridonas/uso terapêutico , Piridonas/efeitos adversos , Resultado do Tratamento , Método Duplo-Cego , Relação Dose-Resposta a DrogaRESUMO
Objectives-This report presents 2020 fetal mortality data by maternal race and Hispanic origin, age, tobacco use during pregnancy, and state of residence, as well as by plurality, sex, gestational age, birthweight, and selected causes of death. Trends in fetal mortality are also examined.
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Mortalidade Fetal , Hispânico ou Latino , Peso ao Nascer , Feminino , Idade Gestacional , Humanos , Gravidez , Gravidez Múltipla , Estados Unidos/epidemiologiaRESUMO
Objectives-This report describes changes between 2020 and 2021 in the percentage of home births by month, race and Hispanic origin, and state of residence of the mother, and makes comparisons with changes occurring between 2019 and 2020.
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Parto Domiciliar , Gravidez , Feminino , Estados Unidos/epidemiologia , Humanos , Mães , Hispânico ou LatinoRESUMO
PURPOSE OF REVIEW: Idiopathic pulmonary fibrosis (IPF) is the prototype of fibrosing interstitial lung diseases. It is mirrored by progressive pulmonary fibrosis (PPF), an umbrella term which characterizes disease behavior of various fibrotic interstitial lung diseases with irreversible progression, accounting for loss of lung function, exercise intolerance and respiratory failure leading to early mortality. Pirfenidone and nintedanib halve the decline in lung function but do not halt disease progression. RECENT FINDINGS: Since the publication in 2014 of pivotal pirfenidone and nintedanib studies, a number of clinical trials were conducted, many of them did not reach their primary endpoints. In IPF, promising phase 2 trials were followed by large phase 3 trials that did not confirm a favorable efficacy to tolerability favorable profile, including those with ziritaxestat, an autotaxin-1 inhibitor, zinpentraxin-alpha (human recombinant pentraxin-2), and the monoclonal antibody pamrevlumab targeting connective tissue growth factor. Nevertheless, newer compounds that hold promise are currently being evaluated in phase 3 or phase 2b randomized controlled trials, including: nerandomilast, a preferential phosphodiesterase 4B inhibitor; admilparant, a lysophosphatidic acid receptor antagonist; inhaled treprostinil, a prostacyclin agonist; and bexotegrast, a dual-selective inhibitor of αvß6 and αvß1 integrins. Nerandomilast, admilparant, inhaled treprostinil, and inhaled AP01 (pirfenidone), are currently studied in patients with PPF. SUMMARY: Despite recent frustrating negative results, there is a growing portfolio of candidate drugs developed in both IPF and PPF.
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Fibrose Pulmonar Idiopática , Doenças Pulmonares Intersticiais , Piridonas , Humanos , Fibrose Pulmonar Idiopática/tratamento farmacológico , Fibrose Pulmonar Idiopática/fisiopatologia , Piridonas/uso terapêutico , Doenças Pulmonares Intersticiais/tratamento farmacológico , Indóis/uso terapêutico , Progressão da Doença , Ensaios Clínicos Controlados Aleatórios como Assunto , Ensaios Clínicos como AssuntoRESUMO
Dengue, caused by the dengue virus (DENV), is a global health threat transmitted by Aedes mosquitoes, resulting in 400 million cases annually. The disease ranges from mild to severe, with potential progression to hemorrhagic dengue. Current research is focused on natural antivirals due to challenges in vector control. This study evaluates the antiviral potential of peptides derived from the microalgae Phaeodactylum tricornutum, known for its bioactive compounds. Microalgae were cultivated under controlled conditions, followed by protein extraction and hydrolysis to produce four peptide fractions. These fractions were assessed for cytotoxicity via the MTT assay and antiviral activity against DENV serotype 2 using flow cytometry and plaque formation assays. The 10-30 kDa peptide fraction, at 150 and 300 µg/mL concentrations, demonstrated no cytotoxicity and significantly reduced the percentage of infected cells and viral titers. These findings suggest that peptides derived from Phaeodactylum tricornutum exhibit promising antiviral activity against dengue virus serotype 2, potentially contributing to developing new therapeutic approaches for dengue.
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Antivirais , Vírus da Dengue , Microalgas , Vírus da Dengue/efeitos dos fármacos , Antivirais/farmacologia , Antivirais/química , Animais , Hidrolisados de Proteína/farmacologia , Hidrolisados de Proteína/química , Dengue/tratamento farmacológico , Dengue/virologia , Peptídeos/farmacologia , Peptídeos/química , Sorogrupo , Chlorocebus aethiops , Humanos , Aedes/efeitos dos fármacos , Células VeroRESUMO
The relationship between sleep, glial cells, and the endocannabinoid system represents a multifaceted regulatory network with profound implications for neuroinflammation and cognitive function. The molecular underpinnings of sleep modulation by the endocannabinoid system and its influence on glial cell activity are discussed, shedding light on the reciprocal relationships that govern these processes. Emphasis is placed on understanding the role of glial cells in mediating neuroinflammatory responses and their modulation by sleep patterns. Additionally, this review examines how the endocannabinoid system interfaces with glia-immune signaling to regulate inflammatory cascades within the central nervous system. Notably, the cognitive consequences of disrupted sleep, neuroinflammation, and glial dysfunction are addressed, encompassing implications for neurodegenerative disorders, mood disturbances, and cognitive decline. Insights into the bidirectional modulation of cognitive function by the endocannabinoid system in the context of sleep and glial activity are explored, providing a comprehensive perspective on the potential mechanisms underlying cognitive impairments associated with sleep disturbances. Furthermore, this review examines potential therapeutic avenues targeting the endocannabinoid system to mitigate neuroinflammation, restore glial homeostasis, and normalize sleep patterns. The identification of novel therapeutic targets within this intricate regulatory network holds promise for addressing conditions characterized by disrupted sleep, neuroinflammation, and cognitive dysfunction. This work aims to examine the complexities of neural regulation and identify potential avenues for therapeutic intervention.
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Endocanabinoides , Transtornos do Sono-Vigília , Humanos , Doenças Neuroinflamatórias , Sistema Nervoso Central , Sono , NeurogliaRESUMO
Objectives-This report presents 2020 data on U.S. births according to a wide variety of characteristics. Trends in fertility patterns and maternal and infant characteristics are described and interpreted.
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Coeficiente de Natalidade , Gravidez na Adolescência , Adolescente , Declaração de Nascimento , Feminino , Humanos , Idade Materna , Parto , Gravidez , Estados Unidos/epidemiologiaRESUMO
Objectives-This report presents 2019 fetal mortality data by maternal race and Hispanic origin, age, tobacco use during pregnancy, and state of residence, as well as by plurality, sex, gestational age, birthweight, and selected causes of death. Trends in fetal mortality are also examined.
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Mortalidade Fetal , Hispânico ou Latino , Peso ao Nascer , Feminino , Idade Gestacional , Humanos , Gravidez , Gravidez Múltipla , Estados Unidos/epidemiologiaRESUMO
Objectives-This report describes changes between 2019 and 2020 in the percentage of U.S. home births by month, race and Hispanic origin, and state of residence of the mother and makes comparisons with changes occurring between 2018 and 2019.
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Parto Domiciliar , Feminino , Hispânico ou Latino , Humanos , Mães , Gravidez , Estados Unidos/epidemiologiaRESUMO
PURPOSE OF REVIEW: Pulmonary hypertension associated with interstitial lung disease (ILD-PH) is associated with significant alteration of quality of life, exercise capacity, and survival. Over the past 2 years, there were changes in the guideline definition and classification of ILD-PH, and positive randomized controlled trials were published. RECENT FINDINGS: Pulmonary hypertension associated with chronic lung disease is now hemodynamically defined as a mean pulmonary artery pressure more than 20âmmHg, with pulmonary artery wedge pressure 15âmmHg or less, and pulmonary vascular resistance (PVR) at least 2 Wood units. Severe ILD-PH is defined by PVR more than 5 Wood units. In the INCREASE trial, patients receiving inhaled treprostinil had favorable significant changes in 6-min walk distance, NT-proBNP level, clinical worsening events, and forced vital capacity, which were maintained in the open label extension study. Promising results were obtained in a placebo-controlled pilot trial using escalated doses of inhaled nitric oxide. According to European guidelines, patients with ILD-PH should be referred to pulmonary hypertension centers, where inhaled treprostinil may be considered; phosphodiesterase type-5 inhibitors may also be considered in patients with severe ILD-PH. SUMMARY: Recent changes in the definitions and a new therapeutic option have an impact on the diagnosis and management of ILD-PH.
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Hipertensão Pulmonar , Doenças Pulmonares Intersticiais , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/tratamento farmacológico , Hipertensão Pulmonar/etiologia , Artéria Pulmonar , Qualidade de Vida , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/tratamento farmacológico , Resistência VascularRESUMO
Background: The presence of emphysema is relatively common in patients with fibrotic interstitial lung disease. This has been designated combined pulmonary fibrosis and emphysema (CPFE). The lack of consensus over definitions and diagnostic criteria has limited CPFE research. Goals: The objectives of this task force were to review the terminology, definition, characteristics, pathophysiology, and research priorities of CPFE and to explore whether CPFE is a syndrome. Methods: This research statement was developed by a committee including 19 pulmonologists, 5 radiologists, 3 pathologists, 2 methodologists, and 2 patient representatives. The final document was supported by a focused systematic review that identified and summarized all recent publications related to CPFE. Results: This task force identified that patients with CPFE are predominantly male, with a history of smoking, severe dyspnea, relatively preserved airflow rates and lung volumes on spirometry, severely impaired DlCO, exertional hypoxemia, frequent pulmonary hypertension, and a dismal prognosis. The committee proposes to identify CPFE as a syndrome, given the clustering of pulmonary fibrosis and emphysema, shared pathogenetic pathways, unique considerations related to disease progression, increased risk of complications (pulmonary hypertension, lung cancer, and/or mortality), and implications for clinical trial design. There are varying features of interstitial lung disease and emphysema in CPFE. The committee offers a research definition and classification criteria and proposes that studies on CPFE include a comprehensive description of radiologic and, when available, pathological patterns, including some recently described patterns such as smoking-related interstitial fibrosis. Conclusions: This statement delineates the syndrome of CPFE and highlights research priorities.
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Enfisema , Hipertensão Pulmonar , Doenças Pulmonares Intersticiais , Enfisema Pulmonar , Fibrose Pulmonar , Feminino , Humanos , Pulmão , Masculino , Enfisema Pulmonar/complicações , Enfisema Pulmonar/diagnóstico por imagem , Fibrose Pulmonar/complicações , Fibrose Pulmonar/diagnóstico por imagem , Estudos Retrospectivos , Síndrome , Revisões Sistemáticas como AssuntoRESUMO
Background: When considering the diagnosis of idiopathic pulmonary fibrosis (IPF), experienced clinicians integrate clinical features that help to differentiate IPF from other fibrosing interstitial lung diseases, thus generating a "pre-test" probability of IPF. The aim of this international working group perspective was to summarize these features using a tabulated approach similar to chest HRCT and histopathologic patterns reported in the international guidelines for the diagnosis of IPF, and to help formally incorporate these clinical likelihoods into diagnostic reasoning to facilitate the diagnosis of IPF. Methods: The committee group identified factors that influence the clinical likelihood of a diagnosis of IPF, which was categorized as a pre-test clinical probability of IPF into "high" (70-100%), "intermediate" (30-70%), or "low" (0-30%). After integration of radiological and histopathological features, the post-test probability of diagnosis was categorized into "definite" (90-100%), "high confidence" (70-89%), "low confidence" (51-69%), or "low" (0-50%) probability of IPF. Findings: A conceptual Bayesian framework was created, integrating the clinical likelihood of IPF ("pre-test probability of IPF") with the HRCT pattern, the histopathology pattern when available, and/or the pattern of observed disease behavior, into a "post-test probability of IPF." The diagnostic probability of IPF was expressed using an adapted diagnostic ontology for fibrotic interstitial lung diseases. Interpretation: The present approach will help incorporate the clinical judgment into the diagnosis of IPF, thus facilitating the application of IPF diagnostic guidelines and, ultimately improving diagnostic confidence and reducing the need for invasive diagnostic techniques.
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Fibrose Pulmonar Idiopática , Doenças Pulmonares Intersticiais , Teorema de Bayes , Humanos , Fibrose Pulmonar Idiopática/diagnóstico , Fibrose Pulmonar Idiopática/patologia , Pulmão/patologia , Doenças Pulmonares Intersticiais/diagnóstico , ProbabilidadeRESUMO
The intricate mechanisms governing brain health and function have long been subjects of extensive investigation. Recent research has shed light on two pivotal systems, the glymphatic system and the endocannabinoid system, and their profound role within the central nervous system. The glymphatic system is a recently discovered waste clearance system within the brain that facilitates the efficient removal of toxic waste products and metabolites from the central nervous system. It relies on the unique properties of the brain's extracellular space and is primarily driven by cerebrospinal fluid and glial cells. Conversely, the endocannabinoid system, a multifaceted signaling network, is intricately involved in diverse physiological processes and has been associated with modulating synaptic plasticity, nociception, affective states, appetite regulation, and immune responses. This scientific review delves into the intricate interconnections between these two systems, exploring their combined influence on brain health and disease. By elucidating the synergistic effects of glymphatic function and endocannabinoid signaling, this review aims to deepen our understanding of their implications for neurological disorders, immune responses, and cognitive well-being.
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Sistema Glinfático , Doenças do Sistema Nervoso , Humanos , Sistema Glinfático/metabolismo , Endocanabinoides/metabolismo , Encéfalo/metabolismo , Sistema Nervoso Central , Doenças do Sistema Nervoso/metabolismoRESUMO
PURPOSE OF REVIEW: Idiopathic pulmonary fibrosis (IPF), characterized by relentless disease progression from the time of diagnosis, is part of a larger group of chronic fibrosing interstitial lung diseases (ILDs). A proportion of patients with non-IPF ILDs may develop, despite conventional treatment, a progressive pulmonary fibrosis (PPF), also referred to as ILD with a progressive fibrosing phenotype (PF-ILD). These patients experience worsening of respiratory symptoms, decline in lung function, and early mortality. The goal of this review is to describe the epidemiology and recent real-life cohorts of PF-ILD, with implications for management. RECENT FINDINGS: The relatively new concept of PF-ILD has aroused active clinical research over the past years. To understand risk factors for progression and the real burden of the disease is crucial to improve management. In the last 2 years, different cohort studies have addressed these questions. They showed that almost one-third of the non-IPF fibrotic ILD patients develop PF-ILD or PPF. SUMMARY: Emerging data show similarities in prognosis between patients with IPF or with non-IPF PF-ILD patients. Early detection and appropriate treatment of this group of patients is a priority. Further research is needed to identify risk factors of progression, to clarify the assessment of progression in clinical practice, for a better management of patients with PF-ILD in a real-world setting.
Assuntos
Fibrose Pulmonar Idiopática , Doenças Pulmonares Intersticiais , Progressão da Doença , Fibrose , Humanos , Fibrose Pulmonar Idiopática/tratamento farmacológico , Doenças Pulmonares Intersticiais/diagnóstico , Fatores de RiscoRESUMO
PURPOSE OF REVIEW: Pleuroparenchymal fibroelastosis (PPFE) is a clinico-radiologic-pathologic interstitial lung disease (ILD) characterized by fibrosis that has upper lobe and subpleural predominance, involving both the visceral pleura and the subjacent subpleural lung parenchyma, and comprises dense fibroelastic changes with prominent elastosis of the alveolar walls together with fibrous thickening of the visceral pleura. The goal of this review is to summarize the state-of-the-art understanding in PPFE. RECENT FINDINGS: PPFE was described in an increasing number of conditions. The course of disease is heterogeneous. Idiopathic PPFE, cases associated with telomerase-related gene mutations, cases related to a history of chemotherapy, and cases combining PPFE with a pattern of usual interstitial pneumonia, may have a particularly poor prognosis. Well-conducted retrospective studies identified marked PPFE features in approximately 10% of patients with idiopathic pulmonary fibrosis, 11% of patients with systemic sclerosis-associated ILD, 6.5% of patients with rheumatoid arthritis-associated ILD, and 23% of patients with hypersensitivity pneumonitis. Drug therapy has not been evaluated prospectively. A small retrospective study suggests that nintedanib may slow disease progression. However, whether the efficacy of antifibrotics is comparable in PPFE and in other forms of progressive pulmonary fibrosis warrants further evaluation. SUMMARY: Accumulating data indicate that PPFE features are associated with poor prognosis in fibrosing ILDs. Further research on the management of PPFE is warranted.
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Fibrose Pulmonar Idiopática , Doenças Pulmonares Intersticiais , Fibrose , Humanos , Fibrose Pulmonar Idiopática/complicações , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Fibrose Pulmonar Idiopática/tratamento farmacológico , Pulmão/diagnóstico por imagem , Pulmão/patologia , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/tratamento farmacológico , Pleura/patologia , Estudos Retrospectivos , Tomografia Computadorizada por Raios XRESUMO
Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is an often deadly complication of IPF. No focussed international guidelines for the management of AE-IPF exist. The aim of this international survey was to assess the global variability in prevention, diagnostic and treatment strategies for AE-IPF.Pulmonologists with ILD expertise were invited to participate in a survey designed by an international expert panel.509 pulmonologists from 66 countries responded. Significant geographical variability in approaches to manage AE-IPF was found. Common preventive measures included antifibrotic drugs and vaccination. Diagnostic differences were most pronounced regarding use of Krebs von den Lungen-6 and viral testing, while high-resolution computed tomography, brain natriuretic peptide and D-dimer are generally applied. High-dose steroids are widely administered (94%); the use of other immunosuppressant and treatment strategies is highly variable. Very few (4%) responders never use immunosuppression. Antifibrotic treatments are initiated during AE-IPF by 67%. Invasive ventilation or extracorporeal membrane oxygenation are mainly used as a bridge to transplantation. Most physicians educate patients comprehensively on the severity of AE-IPF (82%) and consider palliative care (64%).Approaches to the prevention, diagnosis and treatment of AE-IPF vary worldwide. Global trials and guidelines to improve the prognosis of AE-IPF are needed.