Humans ; Diagnosis, Differential ; Idiopathic Pulmonary Fibrosis/pathology* ; Lung/pathology*

Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal interstitial lung disease, the cause is not yet clear. Pathological manifestations are abnormal repair changes resulting from sustained lung injury. Macrophages have been identified as playing a key role in IPF pathogenesis. In different local microenvironments, macrophages can exhibit either classically activated (M1) or alternately activated (M2) phenotypes. M1 plays a key role in promoting inflammatory response and is involved in the process of causing alveolar tissue injury. M2 is involved in wound healing and stopping lung inflammation. Previous studies have shown that activation of 5-hydroxytryptamine (5-HT) signaling is enhanced in pulmonary fibrosis and that 5-HT receptors play an important role in the observed pro-fibrotic effects. As a multifunctional signaling molecule, 5-HT is closely related to lung macrophage polarization, early lung tissue injury, abnormal proliferation and repair, and late extracellular matrix (ECM) deposition. This article reviewed the role of 5-HT and M2 macrophages in the pathogenesis of IPF and the possible regulatory mechanism of 5-HT, in order to provide a reference for further research.
Humans ; Serotonin ; Macrophages ; Lung Diseases, Interstitial/pathology* ; Lung/pathology* ; Idiopathic Pulmonary Fibrosis ; Fibrosis

Idiopathic pulmonary fibrosis (IPF) is a progressive and often fatal form of interstitial lung disease. Despite extensive efforts in research during recent years, the mechanisms of the disease remain poorly understood. Evidence of an inflammatory mechanism, both supportive and contrary, is briefly reviewed in this paper. However, growing evidence has indicated that the apoptosis of alveolar epithelial cells (AECs) may be the early driving force of progression, with subsequent disrupted integrity of the alveolar-capillary basement membrane leading to an abnormal wound healing pathway. Thus, this paper will focus on outlining a process of pathogenesis of IPF from initial apoptosis of AECs to end lung remodeling.
Apoptosis ; Epithelial Cells ; pathology ; Humans ; Idiopathic Pulmonary Fibrosis ; etiology ; pathology ; Lung ; pathology

Lysophosphatidic acid (LPA) is a signaling lipid that binds to six known lysophosphatidic acid receptors (LPARs), named LPA1-LPA6. These receptors initiate signaling cascades relevant to development, maintenance, and healing processes throughout the body. The diversity and specificity of LPA signaling, especially in relation to cancer and autoimmune disorders, makes LPA receptor modulation an attractive target for drug development. Several LPAR-specific analogues and small molecules have been synthesized and are efficacious in attenuating pathology in disease models. To date, at least three compounds have passed phase I and phase II clinical trials for idiopathic pulmonary fibrosis and systemic sclerosis. This review focuses on the promising therapeutic directions emerging in LPA signaling toward ameliorating several diseases, including cancer, fibrosis, arthritis, hydrocephalus, and traumatic injury.
Arthritis ; Autoimmune Diseases ; Fibrosis ; Hydrocephalus ; Idiopathic Pulmonary Fibrosis ; Pathology ; Pharmacology ; Receptors, Lysophosphatidic Acid ; Scleroderma, Systemic ; Sensitivity and Specificity

Idiopathic nonspecific interstitial pneumonia (NSIP) is one of the varieties of idiopathic interstitial pneumonias. Diagnosis of idiopathic NSIP can be done via multidisciplinary approach in which the clinical, radiologic, and pathologic findings were discussed together and exclude other causes. Clinical manifestations include subacute or chronic dyspnea and cough that last an average of 6 months, most of which occur in non-smoking, middle-aged women. The common findings in thoracic high-resolution computed tomography in NSIP are bilateral reticular opacities, traction bronchiectasis, reduced volume of the lobes, and ground-glass opacity in the lower lungs. These lesions can involve diffuse bilateral lungs or subpleural area. Unlike usual interstitial pneumonia, honeycombing is sparse or absent. Pathology shows diffuse interstitial inflammation and fibrosis which are temporally homogeneous, namely NSIP pattern. Idiopathic NSIP is usually treated with steroid only or combination with immunosuppressive agents such as azathioprine, cyclophosphamide, cyclosporine, and mycophenolate mofetil. Prognosis of idiopathic NSIP is better than idiopathic pulmonary fibrosis. Many studies have reported a 5-year survival rate of more than 70%.
Azathioprine ; Bronchiectasis ; Cough ; Cyclophosphamide ; Cyclosporine ; Diagnosis ; Dyspnea ; Female ; Fibrosis ; Humans ; Idiopathic Interstitial Pneumonias ; Idiopathic Pulmonary Fibrosis ; Immunosuppressive Agents ; Inflammation ; Lung ; Lung Diseases, Interstitial ; Pathology ; Prognosis ; Survival Rate ; Traction

Alveolitis, Extrinsic Allergic ; diagnosis ; pathology ; Biopsy ; Chronic Disease ; Cryptogenic Organizing Pneumonia ; diagnosis ; Diagnosis, Differential ; Granuloma, Respiratory Tract ; diagnosis ; Humans ; Idiopathic Interstitial Pneumonias ; diagnosis ; Idiopathic Pulmonary Fibrosis ; diagnosis

Idiopathic pulmonary fibrosis (IPF) is a chronic progressive fibrous interstitial lung disease of unknown etiology. IPF is also considered to be among the independent risk factors for lung cancer, increasing the risk of lung cancer by 7% and 20%. The incidence of IPF complicated with lung cancer, especially non-small cell lung cancer (NSCLC), is increasing gradually, but there is no consensus on unified management and treatment. IPF and NSCLC have similar pathological features. Both appear in the surrounding area of the lung. In pathients with IPF complicated with NSCLC, NSCLC often develops from the honeycomb region of IPF, but the mechanism of NSCLC induced by IPF remains unclear. In addition, IPF and NSCLC have similar genetic, molecular and cellular processes and common signal transduction pathways. The universal signal pathways targeting IPF and NSCLC will become potential therapeutic drugs for IPF complicated with NSCLC. This article examines the main molecular mechanisms involved in IPF and NSCLC and the research progress of drugs under development targeting these signal pathways.
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Humans ; Carcinoma, Non-Small-Cell Lung/genetics* ; Idiopathic Pulmonary Fibrosis/drug therapy* ; Lung Neoplasms/genetics* ; Lung/pathology* ; Signal Transduction

The objective of this study was to elucidata the correlation of 16-detector row CT features with the complications and anatomic-pathology of the disease in patients with idiopathic pulmonary fibrosis (IPF). The 16-detector row CT appearances and predominant anatomic distribution were analyzed in 38 cases of IPF. Of the 14 patients (36.8%) with honeycomb lung, 10 (71.4%) had honeycomb appearances predominantly distributed in the peripheral zone of the middle and lower lobes, particularly in the basal segments of the lower lobes. 11 patients (78.6%) had the clinical duration of honeycomb lung for more than 3 years. The interlobular interstitial thickening was seen in 16 cases (42.1%), the interlobular septa thickening in 20 cases (52.6%); and the thickened bronchus-vascular bundle in 9 cases (23.7%). The above-mentioned three kinds of CT features observably distributed in the peripheral zone of the middle and lower lobes, in the basal segments of the lower lobes, and in the lower lobes and middle lobe of right lung in the middle of the zone, respectively. In 15 patients (39.5%), ground-glass opacity was predominantly distributed in the peripheral zone of lung and in the basal segments of the lower lobes; and the clinical duration lasted less than 1 year was seen in 10 cases (66.7%). These results indicated that some characteristic manifestations with pathologic variation and predominant anatomic distribution of IDF can be shown on 16-detector row CT.
Adult ; Aged ; Female ; Humans ; Idiopathic Pulmonary Fibrosis ; diagnostic imaging ; pathology ; Male ; Middle Aged ; Tomography, Spiral Computed ; methods

Animals ; Diagnosis, Differential ; Humans ; Idiopathic Interstitial Pneumonias ; pathology ; Lung ; diagnostic imaging ; pathology ; Lung Diseases, Interstitial ; classification ; pathology ; Pulmonary Fibrosis ; pathology ; Tomography, X-Ray Computed

Interstitial pneumonia is a heterogenous group of inflammatory and fibrosing lesions that manifest themselves as infiltrative lung disease. Of these, nonspecific interstitial pneumonia is characterized as a variable degree of interstitial inflammation with or without fibrosis and is distinguished from usual interstitial pneumonia and desquamative interstitial pneumonia, histologically. The influx of inflammatory cells and the responses of immune effector cells injury to the alveolar wall and these initial injuries results in alveolitis and fibrosis. Consequently, the gas exchange throughout the alveolar wall is impaired and the patients suffer from lung diseases of a restrictive pattern. The chief complaints represented are dyspnea and dry cough. We experienced a case of nonspecific interstitial pneumonia in a 10-year old girl. The patient had been healthy and had not been exposed to organic dusts or other toxic materials. The pathology of lung biopsy tissue showed that the alveoli were thickened by a mixture of chronic inflammatory cells and collagen type fibrosis. High resolution computed tomography(HRCT) found the patchy areas of ground-glass opacity with patchy consolidation and irregular reticular opacity, and diffuse distribution without zonal predominance. The forced vital capicity(FVC) was 31%, forced expiratory volume in one second (FEV1) 29% and FEV1/FVC 90%, so a restrictive pulmonary insufficiency was found.
Biopsy ; Child* ; Collagen ; Cough ; Dust ; Dyspnea ; Female ; Fibrosis ; Fluconazole ; Forced Expiratory Volume ; Humans ; Idiopathic Pulmonary Fibrosis ; Inflammation ; Lung ; Lung Diseases ; Lung Diseases, Interstitial* ; Pathology