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In coronavirus disease 2019 (COVID-19) patients, dysregulated release of matrix metalloproteinases occurs during the inflammatory phase of acute respiratory distress syndrome (ARDS), resulting in epithelial and endothelial injury with excessive fibroproliferation. COVID-19 resembles idiopathic pulmonary fibrosis (IPF) in several aspects. The fibrotic response in IPF is driven primarily by an abnormally activated alveolar epithelial cells (AECs) which release cytokines to activate fibroblasts. Endoplasmic reticulum (ER) stress is postulated to be one of the early triggers in both diseases. Systemic sclerosis (SSc) is a heterogeneous autoimmune rare connective tissue characterised by fibrosis of the skin and internal organs. Interstitial lung disease (ILD) is a common complication and the leading cause of SSc-related death. Several corollaries have been discussed in this paper for new drug development based on the pathogenic events in these three disorders associated with pulmonary fibrosis. A careful consideration of the similarities and differences in the pathogenic events associated with the development of lung fibrosis in post-COVID patients, IPF patients and patients with SSc-ILD may pave the way for precision medicine. Several questions need to be answered through research, which include the potential role of antifibrotics in managing IPF, SSc-ILD and post-COVID fibrosis. Many trials that are underway will ultimately shed light on their potency and place in therapy.
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http://dx.doi.org/10.4103/lungindia.lungindia_89_22 | DOI Listing |
RMD Open
August 2025
Department of Cell Biology and Immunology, Institute of Parasitology and Biomedicine Lopez-Neyra, CSIC, Granada, Spain
Objective: The common gain-of-function variant rs35705950, located in the promoter of gene, has been strongly associated with interstitial lung diseases (ILDs) of different aetiology, such as idiopathic pulmonary fibrosis (IPF) and rheumatoid arthritis-associated ILD (RA-ILD). In this study, we aimed to investigate the association of this variant and its nearby single nucleotide polymorphisms (SNPs) in the largest cohort of systemic sclerosis-associated ILD (SSc-ILD) to date.
Methods: Samples were collected from blood/saliva, followed by DNA extraction and genotyping using SNP arrays.
Autoimmun Rev
August 2025
Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine, Hokkaido University, Hokkaido, Japan. Electronic address:
Autoimmune connective tissue disease associated with interstitial lung disease (CTD-ILD) includes rheumatoid arthritis-associated ILD, systemic sclerosis-associated-ILD, and several other ILDs. Many patients with CTD-ILD-as well as individuals with other ILDs-develop a progressive pulmonary fibrosis (PPF) similar to idiopathic pulmonary fibrosis (IPF). PPF is characterized by worsening respiratory symptoms, declining lung function despite current pharmacotherapies, and ultimately early death.
View Article and Find Full Text PDFJoint Bone Spine
June 2025
Rheumatology Unit, University of Pisa, Pisa, Italy.
Objective: We assessed the effect of nintedanib (NIN) in terms of quantitative HRCT changes in both idiopathic pulmonary fibrosis (IPF) and systemic sclerosis-associated progressive interstitial lung disease (SSc-ILD), evaluating the relationships between imaging variations and clinical-functional outcomes.
Methods: We prospectively enrolled SSc-ILD and IPF patients treated with NIN and retrospectively selected the same number of subjects from a historical untreated cohort comparable for disease, age, gender and follow-up period. HRCT scans were processed with CALIPER software, obtaining the percentage of normal parenchyma, ILD and vascular-related structures (VRS).
Biomedicines
June 2025
Division of Rheumatology, Department of Internal Medicine, Kyung Hee University College of Medicine, Kyung Hee University Hospital, Seoul 02447, Republic of Korea.
Progressive fibrosing interstitial lung disease (PF-ILD) is a significant complication of connective tissue diseases (CTDs), particularly in systemic sclerosis (SSc), rheumatoid arthritis (RA), and idiopathic inflammatory myopathies (IIM). Despite clinical similarities with idiopathic pulmonary fibrosis (IPF), CTD-associated ILDs exhibit distinct pathogenetic and immunologic features. This review aims to summarize key predictive biomarkers and current treatment strategies associated with the progressive fibrosing phenotype in SSc-ILD, RA-ILD, and IIM-ILD.
View Article and Find Full Text PDFFront Med (Lausanne)
May 2025
Center for Interstitial and Rare Lung Diseases, Ruhrlandklinik, Pneumonology Department, University of Duisburg-Essen, Essen, Germany.
Background: Pulmonary fibrosis is a leading cause of death in patients with Systemic sclerosis (SSc). Single nucleotide polymorphisms (SNPs) within Toll interacting protein () coding gene have been associated with progression and prognosis of Idiopathic Pulmonary Fibrosis (IPF). Aim of the present study was to investigate the association of SNPs with the presence, severity and outcome of interstitial lung disease (ILD) in patients with SSc.
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