Interstitial lung disease (ILD) is the most common lung manifestation of systemic connective tissue disease (CTD) including rheumatoid arthritis, scleroderma, systemic lupus erythematosus, polymyositis or dermatomyositis, Sjögren's syndrome, and mixed connective tissue disease. Declining lung function, worsening respiratory symptoms, and higher mortality are common clinical manifestations of CTD-ILD, which result from progressive fibrosis in the lungs. Although several clinical trials showed that nintedanib and pirfenidone were safe and effective in treating SSc-ILD and other CTDs with fibrosing ILDs, little is known about the pathophysiology of CTD-ILD and the similarities and differences in their molecular underpinnings. Some mechanisms have been proposed such as cellular and/or humoral autoimmunity, endothelial cell dysfunction, and fibroblasts dysfunction. At the same time, both the innate and adaptive immune systems contribute to the development of fibrosis, including profibrotic (Th2, Th17), antifibrotic (Th1, Th22, and -T), pleiotropic (Tregs and Th9), and classical M1 and M2 macrophages that secrete proinflammatory and/or profibrotic cytokines. Furthermore, Neutrophils also played a crucial role in CTD-ILD through secreting elastase and matrix metalloproteinase.
This research topic intends to better investigate the mechanistic similarities and differences of diverse CTD-ILD, as well as the malfunction of immunity and fibrosis in the molecular pathophysiology of CTD-ILD. Understanding the fibrosis and immune system dysfunction in CTD-ILD has the potential to identify therapeutic targets and result in major pharmacological options for treatment.
We invite authors to contribute original research articles as well as review articles that focus on the topic of pathogenesis of CTD-ILD and clinical management in CTD-ILD. Potential topics include but are not limited to the following:
• Pathogenesis of CTD-ILD advances, such as new molecular and cellular pathways
• Dysfunction of innate and adaptive immune cells and humoral autoimmunity in CTD-ILD
• Advances in pathogenesis of fibrosis in CTD-ILD
• Probing the new mechanistic commonalities and differences of various CTD-ILD
• Novel agents and technologies that may be useful in the diagnosis and treatment of CTD-ILD
Interstitial lung disease (ILD) is the most common lung manifestation of systemic connective tissue disease (CTD) including rheumatoid arthritis, scleroderma, systemic lupus erythematosus, polymyositis or dermatomyositis, Sjögren's syndrome, and mixed connective tissue disease. Declining lung function, worsening respiratory symptoms, and higher mortality are common clinical manifestations of CTD-ILD, which result from progressive fibrosis in the lungs. Although several clinical trials showed that nintedanib and pirfenidone were safe and effective in treating SSc-ILD and other CTDs with fibrosing ILDs, little is known about the pathophysiology of CTD-ILD and the similarities and differences in their molecular underpinnings. Some mechanisms have been proposed such as cellular and/or humoral autoimmunity, endothelial cell dysfunction, and fibroblasts dysfunction. At the same time, both the innate and adaptive immune systems contribute to the development of fibrosis, including profibrotic (Th2, Th17), antifibrotic (Th1, Th22, and -T), pleiotropic (Tregs and Th9), and classical M1 and M2 macrophages that secrete proinflammatory and/or profibrotic cytokines. Furthermore, Neutrophils also played a crucial role in CTD-ILD through secreting elastase and matrix metalloproteinase.
This research topic intends to better investigate the mechanistic similarities and differences of diverse CTD-ILD, as well as the malfunction of immunity and fibrosis in the molecular pathophysiology of CTD-ILD. Understanding the fibrosis and immune system dysfunction in CTD-ILD has the potential to identify therapeutic targets and result in major pharmacological options for treatment.
We invite authors to contribute original research articles as well as review articles that focus on the topic of pathogenesis of CTD-ILD and clinical management in CTD-ILD. Potential topics include but are not limited to the following:
• Pathogenesis of CTD-ILD advances, such as new molecular and cellular pathways
• Dysfunction of innate and adaptive immune cells and humoral autoimmunity in CTD-ILD
• Advances in pathogenesis of fibrosis in CTD-ILD
• Probing the new mechanistic commonalities and differences of various CTD-ILD
• Novel agents and technologies that may be useful in the diagnosis and treatment of CTD-ILD
