Immune-mediated lung diseases: A narrative review

Abstract

The role of immunity in the pathogenesis of various pulmonary diseases, particularly interstitial lung diseases (ILDs), is being increasingly appreciated as mechanistic discoveries advance our knowledge in the field. Immune-mediated lung diseases demonstrate clinical and immunological heterogeneity and can be etiologically categorized into connective tissue disease (CTD)-associated, exposure-related, idiopathic, and other miscellaneous lung diseases including sarcoidosis, and post-lung transplant ILD. The immunopathogenesis of many of these diseases remains poorly defined and possibly involves either immune dysregulation, abnormal healing, chronic inflammation, or a combination of these, often in a background of genetic susceptibility. The heterogeneity and complex immunopathogenesis of ILDs complicate management, and thus a collaborative treatment team should work toward an individualized approach to address the unique needs of each patient. Current management of immune-mediated lung diseases is challenging; the choice of therapy is etiology-driven and includes corticosteroids, immunomodulatory drugs such as methotrexate, cyclophosphamide and mycophenolate mofetil, rituximab, or other measures such as discontinuation or avoidance of the inciting agent in exposure-related ILDs. Antifibrotic therapy is approved for some of the ILDs (e.g., idiopathic pulmonary fibrosis) and is being investigated for many others and has shown promising preliminary results. A dire need for advances in the management of immune-mediated lung disease persists in the absence of standardized management guidelines.

The Physical and Psychosocial Impact of Fatigue among Patients with Sjogren’s Syndrome: A Systematic Review

“…Also, both conditions can affect the exchange of oxygen and carbon dioxide in the lungs, and determine the activation of various neural, humoral, thrombotic, metabolic, and inflammatory pathways. If oxygen levels drop during the night due to respiratory issues, it can lead to a state of hypoxemia, which may contribute to feelings of fatigue and exhaustion [ 65 , 66 ]. Poor sleep quality due to respiratory issues can result in daytime sleepiness and a greater need for napping.…”

Molecular Mechanisms Responsible for the Therapeutic Potential of Mesenchymal Stem Cell-Derived Exosomes in the Treatment of Lung Fibrosis

“…Lung or pulmonary fibrosis is a chronic and progressive lung disease characterized by the formation of excessive scar tissue, fibrosis, in the lungs [1]. This scarring occurs due to the abnormal accumulation of collagen and other extracellular matrix components, which leads to the thickening and stiffening of lung parenchymal [1,2].…”

“…Lung or pulmonary fibrosis is a chronic and progressive lung disease characterized by the formation of excessive scar tissue, fibrosis, in the lungs [1]. This scarring occurs due to the abnormal accumulation of collagen and other extracellular matrix components, which leads to the thickening and stiffening of lung parenchymal [1,2]. The pathological process begins with an initial insult or injury to the lung, which can be caused by various factors such as environmental toxins, infections, autoimmune reaction or radiation exposure [2,3].…”

“…Upon DAMPs-dependent activation, alveolar macrophages produce TNF-α and IL-1β (which induce enhanced expression of E and P selectins on lung endothelial cells (ECs)) and neutrophil-, monocyte-and T cellattracting chemokines (CCL2, CCL3 and CXCL12) which bind to their respective receptors on circulating leukocytes, enabling their massive influx in the fibrotic areas [4,5]. As a result, the lungs become infiltrated with immune cells, less elastic and unable to properly expand [1,2].…”

Interferon-λ3 rs12979860 can regulate inflammatory cytokines production in pulmonary fibrosis

“…Numerous ILDs have an immunopathogenesis that is still poorly understood and may be caused by one or more than one of several factors, e.g., immunological dysregulation, inappropriate regeneration, prolonged inflammation, and frequently in the context of genetic predisposition ( Sweis et al, 2023 ).…”