• Journal of Yeungnam Medical Science
• /
• 제37권4호
• /
• pp.269-276
• /
• 2020

Fibrosis is characterized by excessive accumulation of extracellular matrix components. The fibrotic process ultimately leads to organ dysfunction and failure in chronic inflammatory and metabolic diseases such as pulmonary fibrosis, advanced kidney disease, and liver cirrhosis. Idiopathic pulmonary fibrosis (IPF) is a common form of progressive and chronic interstitial lung disease of unknown etiology. Pathophysiologically, the parenchyma of the lung alveoli, interstitium, and capillary endothelium becomes scarred and stiff, which makes breathing difficult because the lungs have to work harder to transfer oxygen and carbon dioxide between the alveolar space and bloodstream. The transforming growth factor beta (TGF-β) signaling pathway plays an important role in the pathogenesis of pulmonary fibrosis and scarring of the lung tissue. Recent clinical trials focused on the development of pharmacological agents that either directly or indirectly target kinases for the treatment of IPF. Therefore, to develop therapeutic targets for pulmonary fibrosis, it is essential to understand the key factors involved in the pathogenesis of pulmonary fibrosis and the underlying signaling pathway. The objective of this review is to discuss the role of kinase signaling cascades in the regulation of either TGF-β-dependent or other signaling pathways, including Rho-associated coiled-coil kinase, c-jun N-terminal kinase, extracellular signal-regulated kinase 5, and p90 ribosomal S6 kinase pathways, and potential therapeutic targets in IPF.

• Tuberculosis and Respiratory Diseases
• /
• 제67권4호
• /
• pp.275-280
• /
• 2009

Interstitial lung disease (ILD) is a group of diseases characterized by pulmonary interstitial inflammation. Finally the inflammation results in pulmonary fibrosis and impairment of oxygen transportation. The causes of idiopathic interstitial pneumonia (IIP) are unknown. Diagnosis of IIP is not easy, especially distinguising between nonspecific interstitial pneumonia and usual interstitial pneumonia (UIP). First line treatments of IIP include corticosteroids and immune modulators, which have limited effect. Currently, several drugs are being researched to prevent and treat fibrosis. Newer drugs that may useful to treat pulmonary fibrosis include endothelin receptor antagonist, recombinant soluble TNF receptor antagonist, and cotrimoxazole. The causes of IIP are largely unknown, treatment is not specific, and prognosis is poor. Recent studies are underway to investigate the pathogenesis and treatment of IIP and pulmonary fibrosis. As the pathogenesis of IIP is elucidated, better treatments will emerge.

• 대한세포병리학회지
• /
• 제1권2호
• /
• pp.129-138
• /
• 1990

Bronchoalveolar lavage (BAL) has emerged as a useful technique for the study of pulmonary interstitial disorders. Several types of Information are provided by the evaluation of lavage fluid identification of cellular constituents helps to separate inflammatory process. Recently we have studied cellular constituents of BAL from three cases with histologically confirmed pulmonary sarcoidosis, idiopathic pulmonary fibrosis and hypereosinophilic syndrome. Pulmonary sarcoidosis showed a marked increase in lymphocytes, idiopathic pulmonary fibrosis revealed a predominance of neutrophils, and hypereosinophilic syndrome presented a marked increase in eosinophils in the lavage fluids.

• 대한한의학회지
• /
• 제25권2호
• /
• pp.41-50
• /
• 2004

Backgrounds & Objectives: Many acute and chronic lung disorders with variable degrees of pulmonary inflammation and fibrosis are collectively referred to as interstitial lung diseases. Idiopathic pulmonary fibrosis (IPF) is one of several idiopathic interstitial pneumonias with the pathogenesis unclear. Astragali Radix is known to inhibit the Th2 immune response. The effects of Astragali Radix on bleomycin-induced lung fibrosis were evaluated. Materials and Methods: Astragali Radix extract was daily given to the normal rats, control (bleomycin) and treated (bleomycin and Astragali Radix extract, 24.0 mg/10g body weight) rats for 14 days. After 14 days, we observed the change of total leukocyte count and percentage, IFN-gamma and IL-4 in BALF (Bronchoalveolar lavage fluid), and of semiquantitative histological index (SHI). Results: Compared to the control group, Astragali Radix decreased total leukocyte count (p<0.05), lymphocyte (p<0.05), neutrophil (no significance) percentage, SHI (p<0.05), IFN-gamma and IL-4 (p<0.05). Otherwise, macrophage percentage was increased (p<0.01). Conclusion: This study showed that Astragali Radix reduced the incidence of inflammatory cells and cytokines and prevented the fibrosis of tissue in bleomycin-induced lung fibrosis rats.

• Molecules and Cells
• /
• 제37권2호
• /
• pp.133-139
• /
• 2014

Idiopathic pulmonary fibrosis (IPF) is the most common and severe type of idiopathic interstitial pneumonias (IIP), and which is currently no method was developed to restore normal structure and function. There are several reports on therapeutic effects of adult stem cell transplantations in animal models of pulmonary fibrosis. However, little is known about how mesenchymal stem cell (MSC) can repair the IPF. In this study, we try to provide the evidence to show that transplanted mesenchymal stem cells directly replace fibrosis with normal lung cells using IPF model mice. As results, transplanted MSC successfully integrated and differentiated into type II lung cell which express surfactant protein. In the other hand, we examine the therapeutic effects of microvesicle treatment, which were released from mesenchymal stem cells. Though the therapeutic effects of MV treatment is less than that of MSC treatment, MV treat-ment meaningfully reduced the symptom of IPF, such as collagen deposition and inflammation. These data suggest that stem cell transplantation may be an effective strategy for the treatment of pulmonary fibrosis via replacement and cytoprotective effect of microvesicle released from MSCs.

• Tuberculosis and Respiratory Diseases
• /
• 제75권2호
• /
• pp.47-51
• /
• 2013

Interstitial lung diseases are heterogeneous entities with diverse clinical presentations. Among them, idiopathic pulmonary fibrosis and connective tissue disease-associated interstitial lung disease are specific categories that pulmonologists are most likely to encounter in the clinical field. Despite the accumulated data from extensive clinical trial and observations, we continue to have many issues which need to be resolved in this field. In this update, we present the review of several articles regarding the clinical presentation, prognosis and treatment of patients with idiopathic pulmonary fibrosis or connective tissue disease-associated interstitial lung disease.

• Tuberculosis and Respiratory Diseases
• /
• 제74권1호
• /
• pp.1-6
• /
• 2013

The concept on idiopathic pulmonary fibrosis (IPF) pathogenesis has progressed from chronic inflammation to aberrant wounding healing and even more to the current paradigms of a multifactorial and heterogeneous disease process. Despite the growth of clinical trials for IPF, most of the results, including N-acetylcysteine combination, warfarin, and bosentan, were disappointing. On the other hand, there have been a number of important developments; the foremost is the licensing of pirfenidone in Europe and Asia. In this article, we briefly review the recent knowledge of pathogenesis of IPF. We also summarize the recent clinical trials regarding the management of IPF.

• 대한한방내과학회지
• /
• 제42권3호
• /
• pp.375-384
• /
• 2021

Objective: This study was designed to analyze the clinical features of idiopathic pulmonary fibrosis patients who attended a Korean medicine hospital and the treatment effects through retrospective chart reviews. Methods: The medical records of 82 outpatients who had been diagnosed with idiopathic pulmonary fibrosis and visited the Allergy, Immune, and Respiratory System Department of Kyung Hee Korean Medicine Hospital from 8 January 2015 to 8 January 2021 were retrospectively reviewed. To assess the treatment outcomes, we used the FVC (Forced Vital Capacity), DLCO (Diffusing capacity of the Lung for CO), 6-minute walk test, and HRCT (High Resolution Computed Tomography). Results: The study group consisted of 28 females and 54 males. The median age of the patients was 67.98±11.44 years. The chief complaints were cough (n=51) and dyspnea (n=49). Other frequent symptoms were general weakness (n=8), weight loss (n=4), and a fever (n=2). A total of 77 (93.90%) patients were prescribed Korean herbal medicine, and 52 (63.41%) patients were treated with acupuncture, moxibustion, cupping therapy, ICT, or pharmacopuncture. After treatments, FVC, DLCO, the 6-minute walk test, and HRCT were maintained or worsened slightly. Conclusions: This study presented the characteristics of idiopathic pulmonary fibrosis patients treated by Korean medical therapies, and further studies of Korean medical treatments for idiopathic pulmonary fibrosis patients would be valuable.

• Tuberculosis and Respiratory Diseases
• /
• 제40권2호
• /
• pp.185-191
• /
• 1993

연구배경 : ICAM-1은 90 kD의 당단백으로서 ${\beta}_2$ integrin과 관계를 가지며, 특발성 폐섬유증의 병인론에 있어서 ICAM-1의 발현과 밀접한 관계가 있다고 보고되고 있다. 특발성 폐섬유종에 있어서 ICAM-1의 발현 정도는 상향조절된다. 특발성 폐섬유증에 있어서 ICAM-1의 발현 양상에 대한 보고는 드물다. 방법 : 본 연구는 특발성 폐섬유증에 있어서 ICAM-1의 발현 양상을 연구하고자 개흉폐생검으로 채취된 특발성 간질성 폐섬유증 3예의 6절편과 폐절제시 채취한 3예의 정상조직을 연구재료로 하여 ICAM-1의 단세포군항체를 이용하여 연역조직화학적 검색을 실시하였다. 결과 : ICAM-1은 3예의 정상조직의 기관지 상피세포나 폐포 세포에서는 발현되지 않았다. 3예의 특발성 폐섬유증 6절편 중 5절편에서 폐포벽의 간질성 섬유아 세포들에서 발현되었고, 1절편에서는 증식된 폐포내 폐포세포에서 발현되었다. 그 정도는 비균질적인 양상을 보였다. 3예의 6절편 중 5절편에서 강한 발현을 1절편에서는 약한 발현정도를 나타냈다. 결론 : 위의 연구 결과를 종합하여 보면, ICAM-1의 발현 증가는 특발성 폐섬유증의 병인과 상당한 관련이 있음을 암시해 주는 것으로 추정된다.

• Tuberculosis and Respiratory Diseases
• /
• 제71권3호
• /
• pp.163-171
• /
• 2011

Recently published articles on interstitial lung disease (ILD) have focused on the accurate diagnosis of idiopathic pulmonary fibrosis (IPF), serum biomarkers, acute exacerbation of IPF, the prognostic factors of ILD and the trial of new treatment. In particular, reports on the serum biomarkers such as CC-chemokine ligand 18, surfactant protein, circulating fibrocytes, and acute exacerbation of IPF are sufficient to be mentioned here. Pirfenidone therapy is the most important trial for the treatment of IPF. Other newer treatment trials such as interferon-gamma, sildenafil and imatinib have been reported to be unsuccessful. On the other hand, the sirolimus trial for lymphangioleiomyomatosis is promising. Combined pulmonary fibrosis and emphysema and IgG4-related disease are established to be the new disease entities of ILD.