Understanding Interstitial Lung Diseases Associated with Connective Tissue Disease (CTD-ILD): Genetics, Cellular Pathophysiology, and Biologic Drivers

Article Critical Care Medicine

Long-Term Safety and Efficacy of Tocilizumab in Early Systemic Sclerosis-Interstitial Lung Disease Open-Label Extension of a Phase 3 Randomized Controlled Trial

Dinesh Khanna et al.

Summary: Tocilizumab preserved lung function and had long-term safety in patients with early SSc-associated ILD.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2022)

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Article Immunology

Single-Cell Transcriptomics Reveals Peripheral Immune Responses in Anti-Synthetase Syndrome-Associated Interstitial Lung Disease

Lili Zhu et al.

Summary: By using single-cell RNA sequencing and flow cytometry analysis, we found that patients with ASS-ILD exhibited altered peripheral immune responses, including upregulated interferon responses, altered CD8 T cell homeostasis, and involvement of differentiation signaling pathways of CD4 T cells. These findings may contribute to the identification of novel therapeutic targets for this condition.

FRONTIERS IN IMMUNOLOGY (2022)

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Review Biology

Neutrophil Extracellular Traps in ANCA-Associated Vasculitis and Interstitial Lung Disease: A Scoping Review

Miriana d'Alessandro et al.

Summary: This study reviewed the significance of neutrophil extracellular traps (NETs) in ANCA-associated vasculitis (AAV) associated with interstitial lung disease (ILD). The study discussed the potential of NETs as a theranostic marker and found that inhibition of NET release could reduce disease severity. However, the underlying mechanisms are still unknown and further research is needed.

LIFE-BASEL (2022)

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Article Rheumatology

Integrative analysis of lung molecular signatures reveals key drivers of systemic sclerosis-associated interstitial lung disease

Seung Min Jung et al.

Summary: Integrative transcriptomic analysis of lung tissues from patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD) revealed key signatures shared with other fibrosing pulmonary diseases, but also showed distinct differences in certain pathological processes. SSc-ILD exhibited similar molecular and cellular signatures to idiopathic pulmonary fibrosis (IPF) but had stronger signals for myofibroblasts, which were in a senescent and apoptosis-resistant state. The p53 signalling pathway was found to be the most enriched molecular signature in SSc-ILD lung tissues and fibroblasts, and was significantly correlated with clinical parameters such as carbon monoxide diffusing capacity of lung, cellular senescence, and apoptosis. Key regulators such as EEF2, EFF2K, PHKG2, VCAM1, PRKACB, ITGA4, CDK1, CDK2, FN1, and HDAC1 were identified with high diffusion scores in the disease module. This network-based approach provides valuable insights into potential therapeutic targets for treating SSc-ILD.

ANNALS OF THE RHEUMATIC DISEASES (2022)

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Article Biochemistry & Molecular Biology

Canonical and noncanonical regulatory roles for JAK2 in the pathogenesis of rheumatoid arthritis-associated interstitial lung disease and idiopathic pulmonary fibrosis

Shaohua Wang et al.

Summary: This study uses RNA sequencing of lung biopsies from patients with RA-ILD and IPF to identify shared and distinct disease-causing pathways. The results suggest that JAK2 plays a crucial role in the development of fibrotic lung diseases.

FASEB JOURNAL (2022)

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Article Medical Laboratory Technology

Biomarkers of connective tissue disease-associated interstitial lung disease in bronchoalveolar lavage fluid: A label-free mass spectrometry-based relative quantification study

Jing Ye et al.

Summary: This study identified differentially expressed proteins (DEPs) in connective tissue disease-associated interstitial lung disease (CTD-ILD) using label-free mass spectrometry. The results showed that these DEPs may play important roles in the development of CTD-ILD and can be potential new biomarkers for early diagnosis of CTD-ILD.

JOURNAL OF CLINICAL LABORATORY ANALYSIS (2022)

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Article Biochemistry & Molecular Biology

Are There Differences in Inflammatory and Fibrotic Pathways between IPAF, CTD-ILDs, and IIPs? A Single-Center Pilot Study

Patrycja Rzepka-Wrona et al.

Summary: This pilot study aimed to determine the differences in pathogenetic pathways between interstitial pneumonia with autoimmune features (IPAF), connective-tissue-disease-associated interstitial lung diseases (CTD-ILDs), and idiopathic interstitial pneumonias (IIPs). The study revealed that IL-8 and TGF-beta 1 concentrations were significantly lower in the IPAF group compared to the CTD-ILD group, but similar to the IIP group. IL-8 and CXCL1 were significantly correlated with BAL total cell count (TCC), and IL-8 and CXCL1 were found to be significant predictors of BAL TCC.

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2022)

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Article Pharmacology & Pharmacy

Connective tissue disease-related interstitial lung disease is alleviated by tripterine through inhibition of the PI3K/Akt, apoptosis, and TNF-a signalling pathways

Wen Zhu et al.

Summary: This study investigated the mechanism of tripterine in treating connective tissue disease-related ILD through network pharmacology and in vivo experiments. The results suggest that tripterine may inhibit the PI3K/Akt, apoptosis, and TNF-alpha signaling pathways in the treatment of CTD-ILD.

FRONTIERS IN PHARMACOLOGY (2022)

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Article Medicine, Research & Experimental

Evidence of pyroptosis and ferroptosis extensively involved in autoimmune diseases at the single-cell transcriptome level

Danfeng Zhang et al.

Summary: Using scRNA-seq datasets, this study reveals the abnormal changes of ferroptosis and pyroptosis in autoimmune diseases. The imbalance of these two cell death modalities exists in different diseases, and specific cell types were found to be more sensitive to pyroptosis. The involvement of ferroptosis and pyroptosis in the pathological process of autoimmune diseases is critical.

JOURNAL OF TRANSLATIONAL MEDICINE (2022)

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Review Medicine, General & Internal

Interstitial lung diseases

Marlies Wijsenbeek et al.

Summary: There are over 200 recognized interstitial lung diseases, which vary in rarity. Most of these diseases are characterized by inflammation or fibrosis in the interstitial space, leading to impaired gas exchange and resulting in symptoms such as breathlessness, decreased exercise tolerance, and reduced quality of life. The prognosis for different interstitial lung diseases varies considerably, with some conditions showing reversibility or stabilization, while others lead to respiratory failure and death. Recent advances in the field have led to the approval of drugs for the treatment of specific interstitial lung diseases, including systemic sclerosis-associated interstitial lung disease, interstitial lung disease-associated pulmonary hypertension, and progressive pulmonary fibrosis.

LANCET (2022)

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Review Biology

Potential Role of JAK Inhibitors in the Treatment of Systemic Sclerosis-Associated Interstitial Lung Disease: A Narrative Review from Pathogenesis to Real-Life Data

Elisa Fiorentini et al.

Summary: Systemic sclerosis-associated interstitial lung disease (SSc-ILD) is a serious and fatal complication with limited treatment options. Janus kinase inhibitors (JAKinibs), with their anti-inflammatory and anti-fibrotic properties, may serve as an interesting therapeutic option for SSc-ILD.

LIFE-BASEL (2022)

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Article Rheumatology

Biomarkers in Systemic Sclerosis Associated Interstitial Lung Disease (SSc-ILD)

Alice Cole et al.

Summary: SSc-ILD is the leading cause of mortality in SSc, and finding biomarkers that can provide disease-specific information has been a challenge. This article provides a comprehensive update on the most studied biomarkers and emerging biomarkers.

CURRENT TREATMENT OPTIONS IN RHEUMATOLOGY (2022)

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Letter Respiratory System

The prevalence and burden of interstitial lung diseases in the USA

Niranjan Jeganathan et al.

ERJ OPEN RESEARCH (2022)

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Article Critical Care Medicine

Senescence of Alveolar Type 2 Cells Drives Progressive Pulmonary Fibrosis

Changfu Yao et al.

Summary: The study demonstrates that senescence of AT2 cells is sufficient to drive progressive pulmonary fibrosis, and interventions targeting p53 activation or senescence can block fibrogenesis. Early attenuation of senescence-related pathways and elimination of senescent cells are promising therapeutic approaches to prevent pulmonary fibrosis.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2021)

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Review Rheumatology

Mechanisms of progressive fibrosis in connective tissue disease (CTD)-associated interstitial lung diseases (ILDs)

Paolo Spagnolo et al.

Summary: This study discusses the different types of interstitial lung diseases (ILDs) and their association with autoimmune and connective tissue diseases (CTDs), as well as introduces drug treatment studies for some fibrosing ILDs. It emphasizes the importance of fibrosis in ILDs.

ANNALS OF THE RHEUMATIC DISEASES (2021)

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Article Medicine, General & Internal

Correlation Between Serum Tumor Marker Levels and Connective Tissue Disease-Related Interstitial Lung Disease

Yunqi Bao et al.

Summary: This study aimed to explore the relationship between serum tumor markers and CTD-ILD, and evaluate the clinical value of tumor markers in investigating ILD in CTD patients. Higher levels of CA153 and CYFRA21-1 were associated with an increased risk of developing CTD-ILD, indicating their potential use as biomarkers for detecting CTD-ILD in clinical practice.

INTERNATIONAL JOURNAL OF GENERAL MEDICINE (2021)

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Review Medicine, Research & Experimental

Immune dysregulation as a driver of idiopathic pulmonary fibrosis

Kevin Shenderov et al.

Summary: IPF is a debilitating disease with limited treatment options, with lung transplantation being the only cure. Recent research has highlighted immune dysregulation as a key factor in the pathophysiology of the disease, suggesting that a better understanding of the immune system's role in IPF may lead to the development of targeted therapies.

JOURNAL OF CLINICAL INVESTIGATION (2021)

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Article Genetics & Heredity

Potential clinical utility of MUC5B und TOLLIP single nucleotide polymorphisms (SNPs) in the management of patients with IPF

Francesco Bonella et al.

Summary: This study found the minor allele of MUC5B rs35705950 to be associated with IPF; the minor allele of TOLLIP rs5743890 was linked to worse survival and more rapid disease progression; while the minor allele of TOLLIP rs3750920 showed no association with disease progression or AE.

ORPHANET JOURNAL OF RARE DISEASES (2021)

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Article Rheumatology

Fibroblasts and their responses to chronic injury in pulmonary fibrosis

B. Wu et al.

Summary: The field of pulmonary fibrosis is rapidly expanding, with a focus on understanding how fibroblasts respond to neighboring cells and the injury microenvironment.

SEMINARS IN ARTHRITIS AND RHEUMATISM (2021)

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Article Medicine, Research & Experimental

Molecular mapping of interstitial lung disease reveals a phenotypically distinct senescent basal epithelial cell population

Daryle J. DePianto et al.

Summary: The compromised regenerative capacity of lung epithelial cells can lead to cellular senescence, which may contribute to fibrosis. Senescent cells with the marker p16 are predominantly located in bronchiolized epithelial structures in scarred regions of IPF and SSc-ILD lung tissue, overlapping with basal epithelial markers Keratin 5 and Keratin 17. These senescent basal epithelial cells in ILD show gene expression patterns similar to terminally differentiating cells in stratified epithelia, driven by p53 activation as part of the senescence program.

JCI INSIGHT (2021)

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Article Rheumatology

Serum KL-6, CA19-9, CA125 and CEA are Diagnostic Biomarkers for Rheumatoid Arthritis-Associated Interstitial Lung Disease in the Chinese Population

Muhan Zheng et al.

Summary: KL-6 levels and tumor markers were found to be elevated in rheumatoid arthritis-associated interstitial lung disease (RA-ILD) and strongly correlated with the severity of ILD, indicating their potential as pathogenically relevant biomarkers for noninvasive detection and prediction of disease severity.

RHEUMATOLOGY AND THERAPY (2021)

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Article Immunology

Disparate Interferon Signaling and Shared Aberrant Basaloid Cells in Single-Cell Profiling of Idiopathic Pulmonary Fibrosis and Systemic Sclerosis-Associated Interstitial Lung Disease

Eleanor Valenzi et al.

Summary: IPF and SSc-ILD exhibit differences in cell types and pathways, providing new insights into the diseases.

FRONTIERS IN IMMUNOLOGY (2021)

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Article Multidisciplinary Sciences

Categorization of lung mesenchymal cells in development and fibrosis

Xue Liu et al.

Summary: Pulmonary mesenchymal cells play critical roles in lung development and disease states in both mice and humans, showing high heterogeneity and subtype-specific transcriptomic differences which contribute to matrix gene expression in fibrosis. Comparative analysis reveals conserved transcriptomic signatures in orthologous subpopulations of murine and human lung mesenchymal cells, enhancing our understanding of mesenchymal cell diversity in lung development and disease conditions.

ISCIENCE (2021)

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Article Respiratory System

Peripheral blood leucocyte telomere length is associated with progression of interstitial lung disease in systemic sclerosis

Shuo Liu et al.

Summary: Peripheral blood leucocyte telomere length (PBL-TL) is associated with progression of systemic sclerosis-associated interstitial lung disease (SSc-ILD), with shorter PBL-TL potentially indicating higher risk of SSc-ILD progression. These findings suggest that telomere dysfunction may be associated with SSc-ILD progression, and PBL-TL measurement may be useful for stratifying risk for SSc-ILD progression.

THORAX (2021)

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Article Multidisciplinary Sciences

Association of rs3750920 polymorphism in TOLLIP with clinical characteristics of fibrosing interstitial lung diseases in Japanese

Takuma Isshiki et al.

Summary: The presence of the T allele of rs3750920 was associated with better survival in Japanese patients with fibrosing interstitial lung diseases, particularly in IPF patients. This suggests that the T allele may serve as an indicator of improved outcomes for fibrosing ILD.

SCIENTIFIC REPORTS (2021)

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Review Immunology

Interstitial Lung Disease in Connective Tissue Disease: A Common Lesion With Heterogeneous Mechanisms and Treatment Considerations

Tihong Shao et al.

Summary: CTD-related interstitial lung disease (CTD-ILD) is a major cause of morbidity and mortality, characterized by heterogeneity and complexity in clinical manifestations. The disease involves chronic inflammation of blood vessels and connective tissues, affecting multiple organ systems. Diagnosis and management of CTD-ILD require comprehensive assessment and precision medicine strategies due to its varied clinical course and etiology.

FRONTIERS IN IMMUNOLOGY (2021)

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Review Immunology

The cGAS-STING pathway as a therapeutic target in inflammatory diseases

Alexiane Decout et al.

Summary: The cGAS-STING signaling pathway plays a crucial role in inflammation in infection, cellular stress, and tissue damage settings. It has the ability to sense and regulate cellular responses to DNA and has the potential to be targeted in the treatment of various inflammatory diseases.

NATURE REVIEWS IMMUNOLOGY (2021)

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Review Critical Care Medicine

Etiology, Risk Factors, and Biomarkers in Systemic Sclerosis with Interstitial Lung Disease

Dinesh Khanna et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2020)

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Review Respiratory System

Predictors of progression in systemic sclerosis patients with interstitial lung disease

Oliver Distler et al.

EUROPEAN RESPIRATORY JOURNAL (2020)

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Review Immunology

Transforming growth factor-beta in tissue fibrosis

Nikolaos G. Frangogiannis

JOURNAL OF EXPERIMENTAL MEDICINE (2020)

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Review Rheumatology

Evasion of apoptosis by myofibroblasts: a hallmark of fibrotic diseases

Boris Hinz et al.

NATURE REVIEWS RHEUMATOLOGY (2020)

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Review Rheumatology

Circulating lung biomarkers in idiopathic lung fibrosis and interstitial lung diseases associated with connective tissue diseases: Where do we stand?

Muriel Elhai et al.

SEMINARS IN ARTHRITIS AND RHEUMATISM (2020)

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Article Cell Biology

Vitamin D levels are prognostic factors for connective tissue disease associated interstitial lung disease (CTD-ILD)

Yujuan Gao et al.

AGING-US (2020)

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Article Respiratory System

The value of serum Krebs von den lungen-6 as a diagnostic marker in connective tissue disease associated with interstitial lung disease

Hua Ma et al.

BMC PULMONARY MEDICINE (2020)

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Review Biochemistry & Molecular Biology

Alveolar Epithelial Type II Cells as Drivers of Lung Fibrosis in Idiopathic Pulmonary Fibrosis

Tanyalak Parimon et al.

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2020)

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Article Rheumatology

Efficacy and safety of pirfenidone in systemic sclerosis-related interstitial lung disease-a randomised controlled trial

Nupoor Acharya et al.

RHEUMATOLOGY INTERNATIONAL (2020)

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Review Medicine, General & Internal

Comparative diagnostic efficacy of serum Krebs von den Lungen-6 and surfactant D for connective tissue disease-associated interstitial lung diseases A meta-analysis

Danli Zhong et al.

MEDICINE (2020)

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Article Immunology

Prominence of IL6, IGF, TLR, and Bioenergetics Pathway Perturbation in Lung Tissues of Scleroderma Patients With Pulmonary Fibrosis

Ludivine Renaud et al.

FRONTIERS IN IMMUNOLOGY (2020)

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Review Medicine, General & Internal

Treatment of Rheumatoid Arthritis-Associated Interstitial Lung Disease: Lights and Shadows

Giulia Cassone et al.

JOURNAL OF CLINICAL MEDICINE (2020)

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Article Critical Care Medicine

Single-Cell Reconstruction of Human Basal Cell Diversity in Normal and Idiopathic Pulmonary Fibrosis Lungs

Gianni Carraro et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2020)

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Article Pharmacology & Pharmacy

Combined anti-fibrotic and anti-inflammatory properties of JAK-inhibitors on macrophages in vitro and in vivo: Perspectives for scleroderma-associated interstitial lung disease

Alain Lescoat et al.

BIOCHEMICAL PHARMACOLOGY (2020)

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Article Multidisciplinary Sciences

Single-cell RNA-seq reveals ectopic and aberrant lung-resident cell populations in idiopathic pulmonary fibrosis

Taylor S. Adams et al.

SCIENCE ADVANCES (2020)

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Review Respiratory System

Connective Tissue Disease-Related Interstitial Lung Disease: Prevalence, Patterns, Predictors, Prognosis, and Treatment

Niranjan Jeganathan et al.

LUNG (2020)

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Article Medicine, General & Internal

Neutrophil-to-Lymphocyte Ratio and Systemic Immune-Inflammation Index-Biomarkers in Interstitial Lung Disease

Victoria Maria Ruta et al.

MEDICINA-LITHUANIA (2020)

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Article Rheumatology

The characteristics of lymphocytes in patients positive for anti-MDA5 antibodies in interstitial lung disease

Wenhan Huang et al.

RHEUMATOLOGY (2020)

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Review Biochemistry & Molecular Biology

The cGAS-STING pathway: The role of self-DNA sensing in inflammatory lung disease

Ruihua Ma et al.

FASEB JOURNAL (2020)

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Review Medicine, Research & Experimental

Contributions of alveolar epithelial cell quality control to pulmonary fibrosis

Jeremy Katzen et al.

JOURNAL OF CLINICAL INVESTIGATION (2020)

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Review Medicine, General & Internal

Spectrum of Fibrotic Lung Diseases

Marlies Wijsenbeek et al.

NEW ENGLAND JOURNAL OF MEDICINE (2020)

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Article Critical Care Medicine

Tocilizumab in systemic sclerosis: a randomised, double-blind, placebo-controlled, phase 3 trial

Dinesh Khanna et al.

LANCET RESPIRATORY MEDICINE (2020)

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Article Rheumatology

Expression of the Autoantigen Topoisomerase-1 is Enriched in the Lung Tissues of Patients With Autoimmune Interstitial Lung Disease: A Case Control Study

Tricia R. Cottrell et al.

ACR OPEN RHEUMATOLOGY (2020)

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Article Rheumatology

The JAK/STAT pathway is activated in systemic sclerosis and is effectively targeted by tofacitinib

Wenxia Wang et al.

JOURNAL OF SCLERODERMA AND RELATED DISORDERS (2020)

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Article Rheumatology

Pathogenesis of systemic sclerosis associated interstitial lung disease

Svetlana Nihtyanova et al.

JOURNAL OF SCLERODERMA AND RELATED DISORDERS (2020)

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Article Pharmacology & Pharmacy

Increases in tumor markers are associated with primary Sjogren's syndrome-associated interstitial lung disease

Lei Shi et al.

THERAPEUTIC ADVANCES IN CHRONIC DISEASE (2020)

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Article Critical Care Medicine

BAL Cell Gene Expression Is Indicative of Outcome and Airway Basal Cell Involvement in Idiopathic Pulmonary Fibrosis

Antje Prasse et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2019)

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Article Pharmacology & Pharmacy

Systemic treatment with resveratrol alleviates adjuvant arthritis-interstitial lung disease in rats via modulation of JAK/STAT/RANKL signaling pathway

Guangxia Yang et al.

PULMONARY PHARMACOLOGY & THERAPEUTICS (2019)

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Review Biochemistry & Molecular Biology

Cellular Senescence: The Trojan Horse in Chronic Lung Diseases

Shruthi Hamsanathan et al.

AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY (2019)

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Article Medicine, General & Internal

Nintedanib for Systemic Sclerosis-Associated Interstitial Lung Disease

Oliver Distler et al.

NEW ENGLAND JOURNAL OF MEDICINE (2019)

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Article Critical Care Medicine

Rare Protein-Altering Telomere-related Gene Variants in Patients with Chronic Hypersensitivity Pneumonitis

Brett Ley et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2019)

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Article Respiratory System

Proliferating SPP1/MERTK-expressing macrophages in idiopathic pulmonary fibrosis

Christina Morse et al.

EUROPEAN RESPIRATORY JOURNAL (2019)

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Review Rheumatology

Biomarkers in systemic sclerosis-associated interstitial lung disease: review of the literature

Olivier Bonhomme et al.

RHEUMATOLOGY (2019)

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Review Biotechnology & Applied Microbiology

The Possible Pathogenesis of Idiopathic Pulmonary Fibrosis considering MUC5B

Qinghua Zhang et al.

BIOMED RESEARCH INTERNATIONAL (2019)

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Article Rheumatology

Single-cell analysis reveals fibroblast heterogeneity and myofibroblasts in systemic sclerosis-associated interstitial lung disease

Eleanor Valenzi et al.

ANNALS OF THE RHEUMATIC DISEASES (2019)

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Article Medicine, General & Internal

Progressive fibrosing interstitial lung diseases: current practice in diagnosis and management

Marlies Wijsenbeek et al.

CURRENT MEDICAL RESEARCH AND OPINION (2019)

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Article Critical Care Medicine

Single-Cell Transcriptomic Analysis of Human Lung Provides Insights into the Pathobiology of Pulmonary Fibrosis

Paul A. Reyfman et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2019)

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Review Physiology

Cell Death in the Lung: The ApoptosisNecroptosis Axis

Maor Sauler et al.

ANNUAL REVIEW OF PHYSIOLOGY, VOL 81 (2019)

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Article Respiratory System

Telomere length and genetic variant associations with interstitial lung disease progression and survival

Chad A. Newton et al.

EUROPEAN RESPIRATORY JOURNAL (2019)

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Article Medicine, General & Internal

Proceedings of the American College of Rheumatology/Association of Physicians of Great Britain and Ireland Connective Tissue Disease-Associated Interstitial Lung Disease Summit: A Multidisciplinary Approach to Address Challenges and Opportunities

Aryeh Fischer et al.

QJM-AN INTERNATIONAL JOURNAL OF MEDICINE (2019)

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Article Rheumatology

Serum KL-6 levels reflect the severity of interstitial lung disease associated with connective tissue disease

Jeong Seok Lee et al.

ARTHRITIS RESEARCH & THERAPY (2019)

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Review Physiology

Fibroblast senescence in the pathology of idiopathic pulmonary fibrosis

David W. Waters et al.

AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY (2018)

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Article Biochemistry & Molecular Biology

Deletion of c-FLIP from CD11b(hi) Macrophages Prevents Development of Bleomycin-induced Lung Fibrosis

Alexandra L. McCubbrey et al.

AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY (2018)

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Review Respiratory System

Pulmonary phenotypes associated with genetic variation in telomere-related genes

Thijs W. Hoffman et al.

CURRENT OPINION IN PULMONARY MEDICINE (2018)

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Review Biochemistry & Molecular Biology

Matrix remodeling in chronic lung diseases

Bon-Hee Gu et al.

MATRIX BIOLOGY (2018)

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Article Dermatology

Rapid alteration of serum interleukin-6 levels may predict the reactivity of i.v. cyclophosphamide pulse therapy in systemic sclerosis-associated interstitial lung disease

Hiroko Numajiri et al.

JOURNAL OF DERMATOLOGY (2018)

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Review Multidisciplinary Sciences

Diverse mechanisms for endogenous regeneration and repair in mammalian organs

James M. Wells et al.

NATURE (2018)

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Article Rheumatology

Value of Serum Carbohydrate Antigen 19-9 and Carcinoembryonic Antigen in Evaluating Severity and Prognosis of Connective Tissue Disease-Associated Interstitial Lung Disease

Qian Jin et al.

ARCHIVES OF RHEUMATOLOGY (2018)

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Article Medicine, General & Internal

MUC5B Promoter Variant and Rheumatoid Arthritis with Interstitial Lung Disease

P. -A. Juge et al.

NEW ENGLAND JOURNAL OF MEDICINE (2018)

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Article Rheumatology

Neutrophil extracellular traps may contribute to interstitial lung disease associated with anti-MDA5 autoantibody positive dermatomyositis

Yun Peng et al.

CLINICAL RHEUMATOLOGY (2018)

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Article Multidisciplinary Sciences

Cellular senescence mediates fibrotic pulmonary disease

Marissa J. Schafer et al.

NATURE COMMUNICATIONS (2017)

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Article Rheumatology

Expression of Cyclic GMP-AMP Synthase in Patients With Systemic Lupus Erythematosus

Jie An et al.

ARTHRITIS & RHEUMATOLOGY (2017)

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Article Cell Biology

Serpine 1 induces alveolar type II cell senescence through activating p53-p21-Rb pathway in fibrotic lung disease

Chunsun Jiang et al.

AGING CELL (2017)

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Review Medicine, General & Internal

Genetics in idiopathic Pulmonary Fibrosis Pathogenesis, Prognosis, and Treatment

Amarpreet Kaur et al.

FRONTIERS IN MEDICINE (2017)

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Meeting Abstract Rheumatology

SHARED GENETIC PREDISPOSITION IN RHEUMATOID ARTHRITIS-INTERSTITIAL LUNG DISEASE AND FAMILIAL PULMONARY FIBROSIS

P-A. Juge et al.

ANNALS OF THE RHEUMATIC DISEASES (2017)

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Article Rheumatology

An Open-label, Phase II Study of the Safety and Tolerability of Pirfenidone in Patients with Scleroderma-associated Interstitial Lung Disease: the LOTUSS Trial

Dinesh Khanna et al.

JOURNAL OF RHEUMATOLOGY (2016)

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Review Immunology

Regulation and function of the cGAS-STING pathway of cytosolic DNA sensing

Qi Chen et al.

NATURE IMMUNOLOGY (2016)

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Article Cell Biology

Loss-of-function mutations in the RNA biogenesis factor NAF1 predispose to pulmonary fibrosis-emphysema

Susan E. Stanley et al.

SCIENCE TRANSLATIONAL MEDICINE (2016)

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Review Biochemistry & Molecular Biology

Pulmonary Macrophages: A New Therapeutic Pathway in Fibrosing Lung Disease?

Adam J. Byrne et al.

TRENDS IN MOLECULAR MEDICINE (2016)

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Article Rheumatology

Frequency of circulating topoisomerase-I-specific CD4 T cells predicts presence and progression of interstitial lung disease in scleroderma

Andrea Fava et al.

ARTHRITIS RESEARCH & THERAPY (2016)

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Article Critical Care Medicine

TOLLIP, MUC5B, and the Response to N-Acetylcysteine among Individuals with Idiopathic Pulmonary Fibrosis

Justin M. Oldham et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2015)

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