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(C) Innate immune response: 1) antigen recognition with pattern recognition receptors; 2) phagocytosis; 3) antigen processing, MHC I and II pathways; MHC I pathway: after phagocytosis, soluble forms of antigens are formed and escape from phagosomes to cytosol; in cytosol, antigens are connected to TAP and transported to the endosome, where they are connected with MHC I molecules; MHC II pathway: foreign peptides degrade in the proteasome to antigens that are bound to MHC II molecules; and 4) antigen expression: expression of the complex of peptide and MHC I (presented to CD8+ cells) or MHC II (presented to CD4+ T cells) at the surface of antigen-presenting cells (APCs). Reported Genetic Factors in Hypersensitivity Pneumonitis Genes and Genetic Variants Population Race or Nationality First Author (Year) (Reference) MHC II polymorphisms  HLA-DR3 White Rittner (1983) (63)  HLA-DR7 Mexican Selman (1987) (64)  HLA-DQ3 Japanese Ando (1989) (65)  HLA-DRB1*04 Mexican Falfán-Valencia (2014) (66) MHC II haplotypes  Increased DRB1*1305-DQB1*0301; decreased DRB1*0802-DQB1*0402 Mexican Camarena (2001) (67) Proteasome and transporter polymorphisms  PSMB8 KQ Mexican Camarena (2010) (68)  TAP1 637, 661 Mexican Aquino-Galvez (2008) (69) Mucin polymorphisms  MUC5B rs35705950 White Ley (2017) (70) Telomere length and mutations  Telomere length <10th percentile White Ley (2017) (70)  Telomere-related gene mutations White Newton (2016) (71) Antiprotease polymorphisms  TIMP-3-915 TIMP-3-1296 (protective role) White Hill (2004) (72) Definition of abbreviations: MHC = major histocompatibility complex; TAP = transporter for antigen presentation; TIMP = tissue inhibitor of metalloproteinase. To date, there is only one study revealing gene mutations (telomerase components) in patients with HP, and we have very little evidence of common variants of candidate genes increasing the risk of developing the disease (50). [...]the relatives of patients with HP are not screened for genetic background, yet we can anticipate that, in the near future, the individuals at genetic risk of HP will be offered counseling to avoid exposure that might trigger the disease (Figures 1A and 1B). Martina Vasakova 1, Moises Selman 2, Ferran Morell 3, 4, Martina Sterclova 1, Maria Molina-Molina 5, 6, and Ganesh Raghu 7 1Department of Respiratory Medicine, First Faculty of Medicine of Charles University, Thomayer Hospital Prague, Prague, Czech Republic 2Instituto Nacional de Enfermedades Respiratorias “Ismael Cosío Villegas,” Mexico City, Mexico 3Vall d’Hebron Institut de Recerca, Servei de Pneumologiía, Hospital Universitari Vall d’Hebron, Barcelona, Spain 4Department de Medicina UAB, Consorcio Centro de Investigación Biomédica en Red (CIBER) de Enfermedades Respiratoria, Barcelona, Spain 5Hospital Universitario de Bellvitge, Instituto de Investigaciones Biomédicas de Bellvitge, Universidad de Barcelona, Hospitalet de Llobregat, Barcelona, Spain 6CIBER de Enfermedades Respiratorias, Barcelona, Spain; and 7Center for Interstitial Lung Diseases, University of Washington Medical Center, Seattle, Washington Corresponding Author: Ganesh Raghu

Patients with interstitial lung disease associated with systemic sclerosis were treated with usual care plus placebo or nintedanib. The annual rate of change in forced vital capacity assessed over a 52-week period was −52.4 ml per year with nintedanib and −93.3 ml per year with placebo. There were no differences in other measures of systemic sclerosis.

In a trial, patients with moderate to severely advanced idiopathic pulmonary fibrosis were treated with nintedanib plus sildenafil or nintedanib alone, with the goal of decreasing IPF symptoms. There were no between-group differences in any of three symptom measures.

Idiopathic pulmonary fibrosis is a chronic interstitial lung disease of unknown etiology; its epidemiology in the United States has not been well characterized. To estimate the annual incidence and prevalence of idiopathic pulmonary fibrosis in the United States. Retrospective cohort design utilizing a large health care claims database spanning the period January 1996 through December 2000. Persons with idiopathic pulmonary fibrosis were identified based on diagnosis and procedure codes. Using broad case-finding criteria, prevalence was estimated to range from 4.0 per 100,000 persons aged 18 to 34 yr to 227.2 per 100,000 among those 75 yr or older; annual incidence was estimated to range from 1.2 to 76.4 per 100,000. Using narrow case-finding criteria, prevalence ranged from 0.8 to 64.7 per 100,000 persons; comparable figures for incidence were 0.4 to 27.1 per 100,000 persons. Extrapolating these rates to the overall United States' population, prevalence was estimated to be 42.7 per 100,000 (incidence, 16.3 per 100,000) using broad criteria; with narrow criteria, prevalence was estimated to be 14.0 per 100,000 (incidence, 6.8 per 100,000). Our results suggest that idiopathic pulmonary fibrosis is probably more common in the United States than previously reported.

Idiopathic pulmonary fibrosis is characterized by rapid loss of vital capacity, disability, and death. There are no effective treatments. Although this study failed to meets its primary end point, the data show therapeutic efficacy at a cost of substantial GI toxicity. Idiopathic pulmonary fibrosis is a debilitating disease characterized by destruction of the gas-exchanging regions of the lung. 1 Its pathogenesis is thought to involve aberrant wound healing mediated by multiple signaling pathways, resulting in progressive lung injury and scarring. 1 Symptoms, including cough and dyspnea, limit physical activity and reduce the patient's quality of life and independence. 2 The course of the disease is difficult to predict, but it generally involves progressive deterioration, with a median survival time of 2.5 to 3.5 years after diagnosis. 3 Unpredictable acute exacerbations occur in some patients and are often fatal. 3 , 4 BIBF 1120 is a potent intracellular . . .

The COVID-19 crisis has accelerated the adoption of telemedicine, presenting challenges and opportunities for clinicians trying to manage diverse, and not only pandemic-related, health conditions. Here, we consider some limitations of telemedicine and offer a perspective on how clinicians can adapt to working in different health-care delivery systems. Here, the authors consider some limitations of telemedicine and offer a perspective on how clinicians can adapt to working in different health-care delivery systems.

The persistent challenge of idiopathic pulmonary fibrosis (IPF), characterized by disease progression and high mortality, underscores the urgent need for innovative therapeutic strategies. We have developed a novel small molecule—catechin derivative ABI-171—selectively targeting dual-specificity tyrosine-phosphorylation-regulated kinase 1A (DYRK1A) and proviral integration site for Moloney murine leukemia virus 1 (PIM1) kinases, crucial in the pathogenesis of fibrotic processes. We employed the Bleomycin-induced (intratracheal) mouse model of pulmonary fibrosis (PF) to evaluate the therapeutic efficacy of ABI-171. Mice with induced PF were treated QD with ABI-171, either prophylactically or therapeutically, using oral and intranasal routes. Pirfenidone (100 mg/kg, TID) and Epigallocatechin gallate (EGCG, 100 mg/kg, QD), a natural catechin currently in a Phase 1 clinical trial, were used as reference compounds. ABI-171, administered prophylactically, led to a significant reduction in hydroxyproline levels and fibrotic tissue formation compared to the control group. Treatment with ABI-171 improved body weight, indicating mitigation of disease-related weight loss. Additionally, ABI-171 demonstrated anti-inflammatory activity, reducing lymphocyte and neutrophil infiltration. In the therapeutic setting, ABI-171, administered 7 days post-induction, reduced mortality rates (p = 0.04) compared with the bleomycin and EGCG control groups. ABI-171 also ameliorated the severity of lung injuries assessed by improved Masson’s trichrome scores when administered both orally and intranasally. ABI-171 significantly decreases bleomycin-induced PF and improves survival in mice, showcasing promising therapeutic potential beyond current medications like pirfenidone and EGCG for patients with IPF. Based on these results, further studies with ABI-171 are ongoing in preclinical studies.

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive lung disease characterized by progressive lung scarring and the histological picture of usual interstitial pneumonia (UIP). It is associated with increasing cough and dyspnoea and impaired quality of life. IPF affects ∼3 million people worldwide, with incidence increasing dramatically with age. The diagnostic approach includes the exclusion of other interstitial lung diseases or overlapping conditions and depends on the identification of the UIP pattern, usually with high-resolution CT; lung biopsy might be required in some patients. The UIP pattern is predominantly bilateral, peripheral and with a basal distribution of reticular changes associated with traction bronchiectasis and clusters of subpleural cystic airspaces. The biological processes underlying IPF are thought to reflect an aberrant reparative response to repetitive alveolar epithelial injury in a genetically susceptible ageing individual, although many questions remain on how to define susceptibility. Substantial progress has been made in the understanding of the clinical management of IPF, with the availability of two pharmacotherapeutic agents, pirfenidone and nintedanib, that decrease physiological progression and likely improve progression-free survival. Current efforts are directed at identifying IPF early, potentially relying on combinations of biomarkers that include circulating factors, demographics and imaging data. Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease characterized by progressive lung scarring that is thought to occur in a genetically susceptible, ageing individual through aberrant reparative responses to repeated injury to the alveolar epithelium. This Primer summarizes the processes thought to underlie the development of IPF and clinical challenges faced in treating patients with this irreversible condition.

This document updates the American Thoracic Society/European Respiratory Society/Japanese Respiratory Society/Latin American Thoracic Association guideline on idiopathic pulmonary fibrosis treatment. Systematic reviews and, when appropriate, meta-analyses were performed to summarize all available evidence pertinent to our questions. The evidence was assessed using the GRADE (Grading of Recommendations, Assessment, Development and Evaluation) approach and then discussed by a multidisciplinary panel. Predetermined conflict-of-interest management strategies were applied, and recommendations were formulated, written, and graded exclusively by the nonconflicted panelists. After considering the confidence in effect estimates, the importance of outcomes studied, desirable and undesirable consequences of treatment, cost, feasibility, acceptability of the intervention, and implications to health equity, recommendations were made for or against specific treatment interventions. The panel formulated and provided the rationale for recommendations in favor of or against treatment interventions for idiopathic pulmonary fibrosis.