1.Idiopathic interstitial pneumonias: clinical findings, pathogenesis, pathology and radiologic findings.
Journal of Korean Medical Science 1999;14(2):113-127
Idiopathic interstitial pneumonias are currently classified into four categories: usual interstitial pneumonia, nonspecific interstitial pneumonia with fibrosis, acute interstitial pneumonia and desquamative interstitial pneumonia. The fibrotic process in interstitial pneumonias appears to result from a complex interaction between fibroblasts, other lung parenchymal cells and macrophages. The complex relationship between the local release of growth-promoting cytokines by alveolar macrophages and resident fibroblasts represents a necessary step for fibrosis or remodeling after lung injury. Injury to the epithelium and basement membranes is likely necessary for the fibrotic process to occur. Usual interstitial pneumonia, most frequent among interstitial pneumonias and has a poor prognosis, appears on high-resolution CT as patchy subpleural areas of ground-glass attenuation, irregular linear opacity, and honeycombing. Nonspecific interstitial pneumonia with fibrosis, the second most frequent and has a better prognosis than usual interstitial pneumonia, appears as subpleural patchy areas of ground-glass attenuation with associated areas of irregular linear opacity on CT. Acute interstitial pneumonia with high mortality rate presents as extensive bilateral airspace consolidation and patchy or diffuse bilateral areas of ground-glass attenuation. Desquamative interstitial pneumonia with good prognosis presents as patchy subpleural areas of ground-glass attenuation in middle and lower lung zones.
Human
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Lung Diseases, Interstitial/radiography
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Lung Diseases, Interstitial/physiopathology
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Lung Diseases, Interstitial/pathology*
3.A case of interstitial lung and liver disease caused by MARS1 gene mutation.
Wen-Jing PENG ; Yan ZHU ; Lai-Shuan WANG ; Wei LU ; Lin YANG ; Li ZHU
Chinese Journal of Contemporary Pediatrics 2023;25(11):1186-1190
The patient is a female infant, 4 months and 9 days old, who was admitted to the hospital due to recurrent fever, cough, and hepatomegaly for over a month. The patient was a healthy full-term infant with a normal birth history. At 2 months and 22 days after birth, she developed recurrent fever, cough, and respiratory distress. Chest imaging revealed diffuse bilateral lung lesions, and fiberoptic bronchoscopy showed interstitial changes in both lungs. These suggested the presence of interstitial lung disease. The patient also presented with hepatomegaly, anemia, hyperlipidemia, hypothyroidism, and malnutrition. Genetic testing indicated compound heterozygous variations in the MARS1 gene. This mutation can cause interstitial lung and liver disease, which is a severe rare disorder that typically manifests in infancy or early childhood. It is inherited in an autosomal recessive manner and characterized by early-onset respiratory insufficiency and liver disease in infants or young children. Since its first reported case in 2013, as of June 2023, only 38 related cases have been reported worldwide. This article reports the multidisciplinary diagnosis and treatment of interstitial lung and liver disease in an infant caused by MARS1 gene mutation.
Female
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Humans
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Infant
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Cough
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Hepatomegaly/pathology*
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Liver Diseases
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Lung/pathology*
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Lung Diseases, Interstitial/pathology*
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Mutation
6.Idiopathic airway-centered interstitial fibrosis: report of two cases.
Xiang-hua YI ; Hai-qing CHU ; Xiao-ming CHENG ; Ben-fang LUO ; Hui-ping LI
Chinese Medical Journal 2007;120(9):847-850
Adult
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Diagnosis, Differential
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Humans
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Lung
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pathology
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Lung Diseases, Interstitial
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diagnosis
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drug therapy
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pathology
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Male
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Middle Aged
8.Focus on the interstitial lung diseases in infancy.
Chinese Journal of Pediatrics 2014;52(4):241-243
Biopsy
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China
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epidemiology
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Diagnosis, Differential
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Humans
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Infant
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Infant, Newborn
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Lung
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pathology
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Lung Diseases, Interstitial
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diagnosis
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pathology
9.Research progress on the role and mechanism of 5-hydroxytryptamine and M2 macrophages in pulmonary interstitial fibrosis.
Yiming DENG ; Changwen DENG ; Xiaoping ZHU
Chinese Critical Care Medicine 2023;35(9):1004-1008
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
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Serotonin
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Macrophages
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Lung Diseases, Interstitial/pathology*
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Lung/pathology*
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Idiopathic Pulmonary Fibrosis
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Fibrosis
10.Research progress of thyroid hormone in pulmonary fibrosis.
Bao Yan LIU ; Yong WANG ; Yan LIU ; Juan LI ; Ping CUI ; Jin HE
Chinese Journal of Industrial Hygiene and Occupational Diseases 2023;41(1):62-66
Pulmonary fibrosis is end-stage of variety of heterogeneous interstitial lung disease, characterizedby excessive proliferation of fibroblasts and extracellular matrix deposition and destruction of lung parenchyma. Thyroid and lung are derived from the same endodermal cells, thyroid hormone affect the occurrence、development and prognosis of the chronic obstructive pulmonary disease, lung cancer and other lung diseases, This article reviews the role and mechanism of thyroid hormone in pulmonary fibrosis in order to provide new idea for the study of the role and mechanism of thyroid hormone in silicosis.
Humans
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Pulmonary Fibrosis/pathology*
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Lung/pathology*
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Silicosis
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Lung Diseases, Interstitial
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Fibroblasts
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Thyroid Hormones
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Fibrosis