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American Journal of Respiratory and... Feb 2000
Review
American Thoracic Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. American Thoracic Society (ATS), and the European Respiratory Society (ERS).
Topics: Adrenal Cortex Hormones; Adult; Aged; Diagnosis, Differential; Female; Humans; Immunosuppressive Agents; Male; Medicine; Middle Aged; Prognosis; Pulmonary Fibrosis; Specialization
PubMed: 10673212
DOI: 10.1164/ajrccm.161.2.ats3-00 -
Annual Review of Medicine 1993Idiopathic pulmonary fibrosis kills half of its victims within five years of diagnosis. Currently available treatment regimens are disappointing, and the incidence of... (Review)
Review
Idiopathic pulmonary fibrosis kills half of its victims within five years of diagnosis. Currently available treatment regimens are disappointing, and the incidence of the disease appears to be increasing. Newer techniques of imaging coupled with laboratory advances in molecular and cellular biology may produce new strategies for modulating the disease process. This article explores new approaches to the diagnosis and management of idiopathic pulmonary fibrosis.
Topics: Diagnostic Imaging; Humans; Pulmonary Fibrosis
PubMed: 8476263
DOI: 10.1146/annurev.me.44.020193.002301 -
Orphanet Journal of Rare Diseases Mar 2008Idiopathic pulmonary fibrosis (IPF) is a non-neoplastic pulmonary disease that is characterized by the formation of scar tissue within the lungs in the absence of any... (Review)
Review
Idiopathic pulmonary fibrosis (IPF) is a non-neoplastic pulmonary disease that is characterized by the formation of scar tissue within the lungs in the absence of any known provocation. IPF is a rare disease which affects approximately 5 million persons worldwide. The prevalence is estimated to be slightly greater in men (20.2/100,000) than in women (13.2/100,000). The mean age at presentation is 66 years. IPF initially manifests with symptoms of exercise-induced breathless and dry coughing. Auscultation of the lungs reveals early inspiratory crackles, predominantly located in the lower posterior lung zones upon physical exam. Clubbing is found in approximately 50% of IPF patients. Cor pulmonale develops in association with end-stage disease. In that case, classic signs of right heart failure may be present. Etiology remains incompletely understood. Some environmental factors may be associated with IPF (cigarette smoking, exposure to silica and livestock). IPF is recognized on high-resolution computed tomography by peripheral, subpleural lower lobe reticular opacities in association with subpleural honeycomb changes. IPF is associated with a pathological lesion known as usual interstitial pneumonia (UIP). The UIP pattern consists of normal lung alternating with patches of dense fibrosis, taking the form of collagen sheets. The diagnosis of IPF requires correlation of the clinical setting with radiographic images and a lung biopsy. In the absence of lung biopsy, the diagnosis of IPF can be made by defined clinical criteria that were published in guidelines endorsed by several professional societies. Differential diagnosis includes other idiopathic interstitial pneumonia, connective tissue diseases (systemic sclerosis, polymyositis, rheumatoid arthritis), forme fruste of autoimmune disorders, chronic hypersensitivity pneumonitis and other environmental (sometimes occupational) exposures. IPF is typically progressive and leads to significant disability. The median survival is 2 to 5 years from the time of diagnosis. Medical therapy is ineffective in the treatment of IPF. New molecular therapeutic targets have been identified and several clinical trials are investigating the efficacy of novel medication. Meanwhile, pulmonary transplantation remains a viable option for patients with IPF. It is expected that, during the next decade, considerable progress will be made toward the understanding and treatment of this devastating illness.
Topics: Aged; Aged, 80 and over; Diagnosis, Differential; Female; Humans; Lung; Lung Diseases; Male; Pulmonary Fibrosis; Tomography, X-Ray Computed
PubMed: 18366757
DOI: 10.1186/1750-1172-3-8 -
Pharmacology & Therapeutics Jul 2023Pulmonary fibrotic diseases are characterized by proliferation of lung fibroblasts and myofibroblasts and excessive deposition of extracellular matrix proteins.... (Review)
Review
Pulmonary fibrotic diseases are characterized by proliferation of lung fibroblasts and myofibroblasts and excessive deposition of extracellular matrix proteins. Depending on the specific form of lung fibrosis, there can be progressive scarring of the lung, leading in some cases to respiratory failure and/or death. Recent and ongoing research has demonstrated that resolution of inflammation is an active process regulated by families of small bioactive lipid mediators termed "specialized pro-resolving mediators." While there are many reports of beneficial effects of SPMs in animal and cell culture models of acute and chronic inflammatory and immune diseases, there have been fewer reports investigating SPMs and fibrosis, especially pulmonary fibrosis. Here, we will review evidence that resolution pathways are impaired in interstitial lung disease, and that SPMs and other similar bioactive lipid mediators can inhibit fibroblast proliferation, myofibroblast differentiation, and accumulation of excess extracellular matrix in cell culture and animal models of pulmonary fibrosis, and we will consider future therapeutic implications of SPMs in fibrosis.
Topics: Animals; Pulmonary Fibrosis; Lung; Fibrosis; Cell Differentiation; Inflammation; Lipids; Inflammation Mediators
PubMed: 37244406
DOI: 10.1016/j.pharmthera.2023.108460 -
International Journal of Molecular... Mar 2019Idiopathic pulmonary fibrosis (IPF) is defined as a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown cause, occurring primarily in older... (Review)
Review
Idiopathic pulmonary fibrosis (IPF) is defined as a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown cause, occurring primarily in older adults, and limited to the lungs. Despite the increasing research interest in the pathogenesis of IPF, unfavorable survival rates remain associated with this condition. Recently, novel therapeutic agents have been shown to control the progression of IPF. However, these drugs do not improve lung function and have not been tested prospectively in patients with IPF and coexisting lung cancer, which is a common comorbidity of IPF. Optimal management of patients with IPF and lung cancer requires understanding of pathogenic mechanisms and molecular pathways that are common to both diseases. This review article reflects the current state of knowledge regarding the pathogenesis of pulmonary fibrosis and summarizes the pathways that are common to IPF and lung cancer by focusing on the molecular mechanisms.
Topics: Animals; Biomarkers; Cell Communication; Cell Transformation, Neoplastic; Disease Progression; Epithelial-Mesenchymal Transition; Gene Expression Regulation; Humans; Idiopathic Pulmonary Fibrosis; Lung Neoplasms; Pulmonary Fibrosis; Signal Transduction
PubMed: 30909462
DOI: 10.3390/ijms20061461 -
Saudi Medical Journal Jun 2004Pulmonary fibrosis is characterized by the accumulation of excessive connective tissue in the lungs. Its causes include chronic administration of some drugs for example... (Review)
Review
Pulmonary fibrosis is characterized by the accumulation of excessive connective tissue in the lungs. Its causes include chronic administration of some drugs for example bleomycin, cyclophosphamide, amiodarone, procainamide, penicillamine, gold and nitrofurantoin; exposure to certain environmental factors such as gases, asbestos and silica and bacterial or fungal infections. Some systemic diseases also predispose to the disease for example rheumatoid arthritis and systemic lupus erythematosus. The disease is associated with release of oxygen radicals and some mediators such as tumor necrosis factor-alpha TNF-alpha, transforming growth factor-beta TGF-beta, PDGF, IGF-I, ET-I and interleukins 1, 4, 8 and 13. The symptoms of the disease include dyspnea, non-productive cough, fever and damage to the lung cells. It is diagnosed with the aid of chest radiography, high resolution computed tomographic scanning and the result of pulmonary function tests. Drug-induced pulmonary fibrosis may involve release of free oxygen radicals and various cytokines for example IL-Ibeta and TNF-alpha via activation of nuclear transcription factor NF-beta as in the case of bleomycin and mitomycin or via release of TGF-beta as in case of tamoxifen or via inhibition of macrophages' and lymphocytes' phospholipases as in the case of amiodarone with the resultant accumulation of phospholipids and reduction of the immune system.
Topics: Antioxidants; Apoptosis; Cytokines; Humans; Pulmonary Fibrosis
PubMed: 15195196
DOI: No ID Found -
Oxidative Medicine and Cellular... 2020Pulmonary fibrosis is a progressively aggravating lethal disease that is a serious public health concern. Although the incidence of this disease is increasing, there is... (Review)
Review
Pulmonary fibrosis is a progressively aggravating lethal disease that is a serious public health concern. Although the incidence of this disease is increasing, there is a lack of effective therapies. In recent years, the pathogenesis of pulmonary fibrosis has become a research hotspot. p53 is a tumor suppressor gene with crucial roles in cell cycle, apoptosis, tumorigenesis, and malignant transformation. Previous studies on p53 have predominantly focused on its role in neoplastic disease. Following in-depth investigation, several studies have linked it to pulmonary fibrosis. This review covers the association between p53 and pulmonary fibrosis, with the aim of providing novel ideas to improve the clinical diagnosis, treatment, and prognosis of pulmonary fibrosis.
Topics: Animals; Humans; Prognosis; Pulmonary Fibrosis; Tumor Suppressor Protein p53
PubMed: 33312337
DOI: 10.1155/2020/6635794 -
Allergy Apr 2005
Review
Topics: Humans; Incidence; Prevalence; Prognosis; Pulmonary Fibrosis
PubMed: 15727572
DOI: 10.1111/j.1398-9995.2005.00719.x -
European Journal of Pharmacology Aug 2017Pulmonary fibrosis (PF) constitutes the end stage of a broad range of heterogeneous interstitial lung diseases, characterized by the destruction of the pulmonary... (Review)
Review
Pulmonary fibrosis (PF) constitutes the end stage of a broad range of heterogeneous interstitial lung diseases, characterized by the destruction of the pulmonary parenchyma, deposition of extracellular matrix and dramatic changes in the phenotype of both fibroblasts and alveolar epithelial cells. More than 200 causes of pulmonary fibrosis have been identified so far, yet the most common form is idiopathic pulmonary fibrosis (IPF). IPF is a lethal lung disorder of unknown etiology with a gradually increasing worldwide incidence and a median survival of 3-5 years from the time of diagnosis. Despite intense research efforts, the pathogenesis remains elusive and no effective treatment is available. Accumulating body of evidence suggests an abnormal wound healing response followed by extracellular matrix deposition, destruction of lung architecture, ultimately leading to respiratory failure. The contribution of immune system in lung fibrogenesis had been largely underscored due to the absence of response to immunosuppressive agents; however, the premise that lung fibrosis has an immunologic background has been recently revived. Toll-like receptors (TLRs) are pattern recognition receptors (PRRs), which link innate and adaptive immune response and regulate wound healing. TLRs promote tissue repair or fibrosis in many disease settings including lung fibrosis, albeit with profound differences depending on the cellular microenvironment. This review summarizes the current state of knowledge regarding the mechanistic implications between TLRs and lung fibrosis and highlights the therapeutic potential of targeting TLR signaling at the ligand or receptor level.
Topics: Animals; Humans; Pulmonary Fibrosis; Signal Transduction; Toll-Like Receptors; Wound Healing
PubMed: 27364757
DOI: 10.1016/j.ejphar.2016.06.045 -
Current Opinion in Pulmonary Medicine Sep 2006Idiopathic pulmonary fibrosis is a progressively fatal interstitial lung disease associated with pathological findings of usual interstitial pneumonia. Its pathogenesis... (Review)
Review
PURPOSE OF REVIEW
Idiopathic pulmonary fibrosis is a progressively fatal interstitial lung disease associated with pathological findings of usual interstitial pneumonia. Its pathogenesis is unknown, and there are no proven effective therapies. Familial cases account for 0.5-2% of idiopathic pulmonary fibrosis. Familial idiopathic pulmonary fibrosis occurs as an autosomal dominant disorder with variable penetrance. The clinical characteristics of familial idiopathic pulmonary fibrosis are indistinguishable from sporadic idiopathic pulmonary fibrosis. These marked similarities support the hypothesis there may, in part, be a genetic basis for idiopathic pulmonary fibrosis.
RECENT FINDINGS
Many investigations have evaluated genetic polymorphisms and idiopathic pulmonary fibrosis. Candidate genes include cytokines and surfactant protein genes. To date, no gene has been consistently identified to be associated with idiopathic pulmonary fibrosis. Recent studies in familial idiopathic pulmonary fibrosis have demonstrated a strong association with surfactant protein C gene mutations in one large kindred.
SUMMARY
The pathogenesis of both idiopathic pulmonary fibrosis and familial idiopathic pulmonary fibrosis remains unclear. A consistent genetic basis has not yet been demonstrated in all cases. Other factors, including variable gene expression, co-carriage of other modifying genes and environmental stimuli, particularly cigarette smoke, significantly contribute to disease expression.
Topics: Humans; Pedigree; Pulmonary Fibrosis
PubMed: 16926644
DOI: 10.1097/01.mcp.0000239546.24831.61