Idiopathic pulmonary fibrosis

King, Talmadge E.; Pardo, Annie; Selman, Moisés

doi:10.1016/s0140-6736(11)60052-4

Cited by 1,735 publications

(1,551 citation statements)

References 136 publications

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“…Several intracellular trafficking processes might be affected, leading to endoplasmic reticulum stress and apoptosis caused by environmental exposure to injurious agents (e.g., viral infection, recurrent aspiration of gastric contents) or through genetic abnormalities (e.g., surfactant proteins). Injured or dysfunctional epithelial cells are proposed to promote the accumulation of fibroblasts that are responsible for the excessive deposition of extracellular matrices (33).…”

Section: Pulmonary Fibrosis: Pathogenesismentioning

confidence: 99%

“…Regardless of their source of origin, the interplay between dysfunctional epithelial cells and activated fibroblasts (also termed myofibroblasts because of their expression of smooth muscle cell markers) seems to drive unrestrained collagen deposition and tissue scarring. Underlying amplifying factors such as aging and epigenetic changes are thought to further perpetuate the progressive nature of pulmonary fibrosis (33).…”

Section: Pulmonary Fibrosis: Pathogenesismentioning

confidence: 99%

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Pulmonary Fibrosis in Hermansky-Pudlak Syndrome

et al. 2016

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Hermansky-Pudlak syndrome (HPS) is a rare autosomal recessive genetic disorder characterized by oculocutaneous albinism and a bleeding diathesis due to platelet dysfunction. More than 50% of cases worldwide are diagnosed on the Caribbean island of Puerto Rico. Genetic testing plays a growing role in diagnosis; however, not all patients with HPS have identified genetic mutations. In Puerto Rico, patients with HPS are often identified shortly after birth by their albinism, although the degree of hypopigmentation is highly variable. Ten subtypes have been described. Patients with HPS-1, HPS-2, and HPS-4 tend to develop pulmonary fibrosis in Puerto Rico; 100% of patients with HPS-1 develop HPS-PF. HPS-PF and idiopathic pulmonary fibrosis are considered similar entities (albeit with distinct causes) because both can show similar histological disease patterns. However, in contrast to idiopathic pulmonary fibrosis, HPS-PF manifests much earlier, often at 30-40 years of age. The progression of HPS-PF is characterized by the development of dyspnea and increasingly debilitating hypoxemia. No therapeutic interventions are currently approved by the U.S. Food and Drug Administration for the treatment of HPS and HPS-PF. However, the approval of two new antifibrotic drugs, pirfenidone and nintedanib, has prompted new interest in identifying drugs capable of reversing or halting the progression of HPS-PF. Thus, lung transplantation remains the only potentially life-prolonging treatment. At present, two clinical trials are recruiting patients with HPS-PF to identify biomarkers for disease progression. Advances in the diagnosis and management of these patients will require the establishment of multidisciplinary centers of excellence staffed by experts in this disease.

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Section: Pulmonary Fibrosis: Pathogenesismentioning

confidence: 99%

Section: Pulmonary Fibrosis: Pathogenesismentioning

confidence: 99%

Pulmonary Fibrosis in Hermansky-Pudlak Syndrome

et al. 2016

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“…In pulmonary fibrosis, epithelial stress may induce alveolar epithelial cells to secrete TGF-b. Abnormal activation of TGF-b leads to trans-differentiation of quiescent fibroblasts into myofibroblasts, and excessive production of fibrillar collagens [1,22,23] as observed in IPF patients [24]. Additionally, increased elevation of TGF-b1 under hypoxic conditions can lead to rapid and pronounced EMT [25].…”

Section: Discussionmentioning

confidence: 99%

“…Patchy areas of fibrosis associated with exaggerated accumulation of extracellular matrix (ECM) mark the IPF pathology [1,2]. The IPF possibly develops because of recurring injury to the alveolar epithelium with concomitant dysregulation of cellular homeostasis, followed by erroneous repair of the epithelial damage.…”

Section: Introductionmentioning

confidence: 99%

Epithelial–mesenchymal transition of A549 cells is enhanced by co-cultured with THP-1 macrophages under hypoxic conditions

Sueki

Matsuda

Iwashita

et al. 2014

Biochemical and Biophysical Research Communications

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a b s t r a c tEpithelial-mesenchymal transition (EMT) is associated with pulmonary fibrosis, including idiopathic pulmonary fibrosis (IPF). In this study, we investigated EMT of human pulmonary epithelial-derived cells (A549). A549 cells was either cultured by itself or co-cultured with THP-1 macrophages under normoxic (21% O 2 ) and hypoxic (2% O 2 ) conditions. We evaluated the presence of EMT by determining the expression of EMT markers, E-cadherin, vimentin, and fibronectin. To determine the role of TGF-b1 and IL-1b in EMT of the A549 cells, we analyzed the effects of blocking their activity with TGF-b1 inhibitor or IL-1b neutralizing antibody respectively. The A549 cells presented EMT when they were co-cultured with THP-1 macrophages. The EMT of the A549 cells co-cultured with THP-1 macrophages was exacerbated under hypoxia. In addition, the EMT were prevented by the addition of TGF-b1 type I receptor kinase inhibitor. The hypoxic condition increased the mRNA levels of TGF-b1 in A549 cells and THP-1 macrophages and that of IL-1b in THP-1 macrophages when each cells were co-cultured. Anti-IL-1b neutralizing antibody attenuated TGF-b1 secretion in co-culture media under hypoxic conditions. Thus, the IL-1b from THP-1 macrophages up-regulated the TGF-b1 from A549 cells and THP-1 macrophages, and then the TGF-b1 from both cells induced and promoted the EMT of A549 cells when they were co-cultured under hypoxia. Together, these results demonstrate that the interaction between type II pneumocytes and macrophages under hypoxia is necessary for the development of pulmonary fibrosis.

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“…Similarly Raghu and Meyer [24] concluded from their results of study that older patients were detected in IPF. Many studies declared that changes in gene expression or genetic polymorphisms which occur in older age were the commonest cause for injury and fibrosis of aged lung [25]. Poh et al [26] concluded that elder patients are always associated with decrease gastroesophageal motion with decreasing in the pressure of the upper sphincter of the esophagus, and increasing exposure to acids in esophagus.…”

mentioning

confidence: 99%

Assessment of Bronchoalveolar Lavage Pepsin Level in Idiopathic Pulmonary Fibrosis

Said¹,

Alsowey²,

Ibraheem³

2017

Zagazig University Medical Journal

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Background: Idiopathic pulmonary fibrosis (IPF) is defined as a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown cause.Recently, there is increasing evidence supporting a relationship between gastroesophageal reflux, microaspiration, and IPF, since the diagnostic gold-standard for microaspiration remains unknown. So, the aim of this study is to assess the presence of chronic microaspirates by bronchoalveolar lavage pepsin level in these patients. Patients and method: A case -control study was conducted at Chest Department and outpatient clinics Faculty of Medicine, Zagazig University hospitals in the period from (February 2013 till January 2016) on thirty patients with stable interstitial lung diseases (ILD).they were divided into two groups: 15 patients (group I) with stable idiopathic pulmonary fibrosis (IPF), another 15 patients (group II) stable ILD other than IPF. Both groups underwent pulmonary function tests (PFTs), Pulmonary HRCT and Bronchoscopy. Measurement of bronchoalveolar lavage pepsin level was done for all patients. Results: 80% of IPF patients complained from gastroesophageal reflux with a statistically significant decrease in PaO 2 , FEV 1 and FVC than in non IPF patients.Bronchoalveolar lavage pepsin level was (43.711.4ng/ml) in IPF group, which was significantly higher than non IPF group (32.710.5 ng/ml).Also total cell count in IPF patients were increased significantly including neutrophils, alveolar macrophages and red blood. Increased reticulation on HRCT for IPF patients group were associated with higher BAL pepsin levels (p <0.01). Regression coefficient revealed the most powerful predictor of IPF severity were; HRCT findings: reticulation score,honeycombing score[(Odds ratio(95%CI)=5.3 (1.9 to14.9), 7.8 (2.7 to 22.3)respectively],pepsin level[(Odds ratio(95%CI)=4.6 (1.9-11.4)],FEV1 and FVC[(Odds ratio(95%CI)=3.7 (1.6 to 8.9), 3.2 (1.4 to 7.5)respectively].while gastroesophageal reflux symptom [(Odds ratio(95%CI)=1.04(0.94-1.14)] was the least powerful predictor. Conclusion: Gastroesophageal reflux or microaspiration is common in patients with IPF, associated with elevated levels of pepsin in BAL fluid.

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Idiopathic pulmonary fibrosis

Cited by 1,735 publications

References 136 publications

Pulmonary Fibrosis in Hermansky-Pudlak Syndrome

Pulmonary Fibrosis in Hermansky-Pudlak Syndrome

Epithelial–mesenchymal transition of A549 cells is enhanced by co-cultured with THP-1 macrophages under hypoxic conditions

Assessment of Bronchoalveolar Lavage Pepsin Level in Idiopathic Pulmonary Fibrosis

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