Interstitial lung diseases (ILDs) are a diverse collection of lung disorders sharing similar features, such as inflammation and fibrosis. The diagnosis and management of ILD require a multidisciplinary approach using clinical, radiological, and pathological evaluation. Progressive pulmonary fibrosis (PPF) is a distinct form of progressive and fibrotic disease, occurring in ILD cases other than in idiopathic pulmonary fibrosis (IPF). It is defined based on clinical symptoms, lung function, and chest imaging, regardless of the underlying condition. The progression to PPF must be monitored through a combination of pulmonary function tests (forced vital capacity [FVC] and diffusing capacity of the lung for carbon monoxide), an assessment of symptoms, and computed tomography scans, with regular follow-up. Although the precise mechanisms of PPF remain unclear, there is evidence of shared pathogenetic mechanisms with IPF, contributing to similar disease behavior and worse prognosis compared to non-PPF ILD. Pharmacological treatment of PPF includes immunomodulatory agents to reduce inflammation and the use of antifibrotics to target progressive fibrosis. Nintedanib, a known antifibrotic agent, was found to be effective in slowing IPF progression and reducing the annual rate of decline in FVC among patients with PPF compared to placebos. Nonpharmacological treatment, including pulmonary rehabilitation, supplemental oxygen therapy, and vaccination, also play important roles in the management of PPF, leading to comprehensive care for patients with ILD. Although there is currently no cure for PPF, there are treatments that can help slow the progression of the disease and improve quality of life.
Lee Jae-Sung;Jung Hee-Jae;Jung Sung-Ki;Rhee Hyung-Goo
The Journal of Internal Korean Medicine
/
v.25
no.1
/
pp.71-80
/
2004
Objective : Idiopathic Lung Fibrosis(IPF) is chronic fibrotic interstitial pneumonia. The pathogenesis is unclear. Lonicerae Flos is known to prevent the inflammation and reinforce the immune system. The effects of Lonicerae Flos on bleomycin-induced lung fibrosis is evaluated. Material and Methods: Lonicerae Flos extract was given to the Normal rats, control(bleomycin) rats everyday and treated(bleomycin and lonicerae flos) rats 21.0 mg per body weight 109 for 14 days. 14 days after, we observed the change of leukocyte count and percentage of IFN-gamma and IL-4 in BALF. and that of Semiquantative histological index(SHI). Results : Compared to control rats, Lonicerae Flos decreased leukocyte count(P<0.01) lymphocyte, neutrophil percentage(P<0.05), SHI(P<0.01), IFN-gamma and IL-4(P<0.05) in Treated rats. Otherwise, macrophage percentage was increased(P<0.01) in Treated rats. Conclusion : This study showed that Lonicerae Flos reduced the change of inflammatory cells and cytokines in bleomycin-induced lung fibrosis and reduced the fibrosis of tissue. And, we needed many other distinct researches on lung fibrosis.
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.
The Journal of Korean Medicine Ophthalmology and Otolaryngology and Dermatology
/
v.34
no.1
/
pp.56-65
/
2021
Objectives : The purpose of this study is to report the effectiveness of Korean medical treatment on ptosis in myasthenia gravis. Methods : We treated the patient who had suffered from rt. ptosis and was diagnosed with myasthenia gravis with acupuncture, electropuncture, herbal acupuncture, cupping therapy, herbal medicines and western medicine such as corticosteroids and acetylcholinesterase inhibitors. The effectiveness of treatment was evaluated through Relative Interpalpebral Fissure(IPF) and photography. Results : After the treatments, relative Interpalpebral Fissure(IPF) was increased and improvement remained for three months after the treatment was finished. Conclusions : The result indicates that combination therapy of Korean medical treatment and western medicine had an effect on treatment of ptosis with myasthenia gravis.
Although chronic obstructive pulmonary disease (COPD) and interstitial lung disease (ILD) have distinct clinical features, both diseases may coexist in a patient because they share similar risk factors such as smoking, male sex, and old age. Patients with both emphysema in upper lung fields and diffuse ILD are diagnosed with combined pulmonary fibrosis and emphysema (CPFE), which causes substantial clinical deterioration. Patients with CPFE have higher mortality compared with patients who have COPD alone, but results have been inconclusive compared with patients who have idiopathic pulmonary fibrosis (IPF). Poor prognostic factors for CPFE include exacerbation, lung cancer, and pulmonary hypertension. The presence of interstitial lung abnormalities, which may be an early or mild form of ILD, is notable among patients with COPD, and is associated with poor prognosis. Various theories have been proposed regarding the pathophysiology of CPFE. Biomarker analyses have implied that this pathophysiology may be more closely associated with IPF development, rather than COPD or emphysema. Patients with CPFE should be advised to quit smoking and undergo routine lung function tests, and pulmonary rehabilitation may be helpful. Various pharmacologic agents and surgical approaches may be beneficial in patients with CPFE, but further studies are needed.
Journal of Radiopharmaceuticals and Molecular Probes
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v.8
no.2
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pp.103-111
/
2022
Idiopathic pulmonary fibrosis (IPF) is a progressive disease caused by some risk factors, including smoking, viral infection, toxic substances, and radiation, that decline lung function of fresh oxygen and blood delivery throughout the body. Patients with pulmonary fibrosis have suffered from breathing and cough and the average survival rate is only 3 years after diagnosis. Therefore, it is significant to diagnose IPF and start treatment in enough time. Usually, lung biopsy is available to diagnose localized pulmonary fibrotic sites directly. However, it is insufficient to visualize whole lung tissue, and also it has a risk of infection for patients. In the clinic, medical imaging systems can diagnose pulmonary fibrosis non-invasively without infection. In this review, we introduce current medical imaging systems used to diagnose pulmonary fibrosis, including CT, MRI, and nuclear medicine. Further, we introduce several molecular imaging probes targeting specific biomarkers which are expressed in pulmonary fibrosis. Through this paper, it is expected that it would be helpful to understand the latest knowledge and research trends on pulmonary fibrosis diagnostic imaging.
Background: Analysis of cells in bronchoalveolar lavage(BAL) fluid had been used to predict the histologic changes of the bronchioles and alveoli in patients with interstitial lung diseases(ILD). Definitive diagnosis can be a1so made in some cases of ILD, such as histiocytosis. However, there are a few data of the cellular components in BAL fluid in normal Korean individuals and in patients with ILD. In order to evaluate the role of the cellular analysis of BAL fluid in prediction of alveolitis and differential diagnosis among ILDs, we compared the cellular components in BAL fluid from 50 normal individuals and 86 ILD patients. Method: BAL was performed by instillation and retrievement of normal saline with fiberoptic bronchoscopy. The cell number was counted by Hemocytometer. Differential count was done up to 500 cells on slides prepared by Diff-Quik stain and non-specific esterase stain. We compared the recovery rate(RR), cell numbers(CN), and percentages of each cellular components(CP). Results: The results were as follows: 1) There was no difference in RR, CN and CP between the normal smoker group and normal non-smoker group. 2) Total cell numbers recoverd in BAL fluid increased in collagen vascular diseases(CVD), hypersensitivity pneumonitis(HP), idiopathic pulmonary fibrosis(IPF), and miliary tuberculosis(Mil TBC) groups. 3) The percentage of lymphocytes increased in HP, IPF and Mil TBC groups. Macrophage percentages increased in HP, IPF, and Mil TBC groups. Neutrophil percentages were increased in CVD, HP, IPF and Mil TBC groups. Eosinophil percentages were increased in HP, IPF and Mil TBC groups. The numbers of each cells showed same findings as the percentages did. Conclusion: The analysis of cellular components of BAL fluid can predict the presence of alveolitis in many cases of ILDs. However, It was not helpful in differential diagnosis among ILDs.
Background : Idiopathic pulmonary fibrosis (IPF) is a diffuse inflammatory and fibrosing process that occurs within the interstitium and alveolus of the lung with invariably poor prognosis. The major problem in management of IPF results from the variable rate of disease progression and the difficulties in predicting the response to therapy. The purpose of this retrospective study was to evaluate the short-term efficacy of steroid and immunosuppressive therapy for IPF and to identify the pre-treatment determinants of favorable response. Method : Twenty patients of IPF were included. Diagnosis of IPF was proven by thoracoscopic lung biopsy and they were presumed to have active progressive disease. The baseline evaluation in these patients included clinical history, pulmonary function test, bronchoalveolar lavage (BAL), and chest high resolution computed tomography (HRCT). Fourteen patients received oral prednisolone treatment with initial dose of 1mg/kg/day for 8 to 12 weeks and then tapering to low-dose prednisolone (0.25mg/kg/day). Six patients who previously had experienced significant side effects to steroid received 2mg/kg/day of oral cyclophosphamide with or without low-dose prednisolone. Follow-up evaluation was performed after 6 months of therapy. If patients met more than one of followings, they were considered to be responders : (1) improvement of more than one grade in dyspnea index, (2) improvement in FVC or TLC more than 10% or improvement in DLco more than 20% (3) decreased extent of disease in chest HRCT findings. Result : One patient died of extrapulmonary cause after 3 month of therapy, and another patient gave up any further medical therapy due to side effect of steroid. Eventually medical records of 18 patients were analyzed. Nine of 18 patients were classified into responders and the other nine patients into nonresponders. The histopathologic diagnosis of the responders were all nonspecific interstitial pneumonia (NSIP) and that of nonresponders were all usual interstitial pneumonia (UIP) (p<0.001). The other significant differences between the two groups were female predominance (p<0.01), smoking history (p<0.001), severe grade of dyspnea (p<0.05), lymphocytosis in BAL fluid ($23.8{\pm}16.3%$ vs $7.8{\pm}3.6%$, p<0.05), and less honeycombing in chest HRCT findings (0% vs $9.2{\pm}2.3%$, p<0.001). Conclusion : Our results suggest that patients with histopathologic diagnosis of NSIP or lymphocytosis in BAL fluid are more likely to respond to steroid or immunosuppressive therapy. Clinical results in large numbers of IPF patients will be required to identify the independent variables.
Background : Nonspecific interstitial pneumonitis (NSIP) is most likely to be confused with usual interstitial pneumonitis (UIP). Unlike patients witþ UIP, the majority of patients with NSIP have a good prognosis, with most patients improving after treatment with corticosteroids. Therefore it is clinically important to differentiate NSIP from UIP. Up to now, the only means of differentiating these two diseases was by means of surgical lung biopsy. American Thoracic Society (ATS) proposed a clinical diagnostic criteria for UIP to provide assistance to clinicians in its diagnosis without surgical lung biopsy. This study is aimed to investigate whether there were clinical and radiological differences between NSIP and UIP, and the usefulness of ATS clinical diagnostic criteria for UIP in Korea. Methods : We studied 60 patients with UIP and NSIP confirmed by surgical lung biopsy. Clinical manifestations, pulmonary function test, arterial blood gas analysis, bronchoalveolar lavage (BAL), and high resolution computed tomography (HRCT) were evaluated and analyzed by Chi-square test or t-test. The clinical criteria for UIP proposed by ATS were applied to all patients with idiopathic interstitial pneumonia. Results : Forty-two patients with UIP and 18 with NSIP were pathologically identified. Among the 18 patients with NSIP (M : F=1 : 17), the mean age was 55.2$\pm$8.4 (44~73) yr. Among the 42 patients with UIP (M : F=33 : 9), the mean age was 59.5$\pm$7.1 (45~74) yr (p=0.046). Fever was more frequent in NSIP (39%) (p=0.034), but clubbing was frequently observed in UIP (33%) (p=0.023). BAL lymphocytosis was more frequent (23%) (p=0.0001) and CD4/CD8 ratio was lower in NSIP (p=0.045). On HRCT, UIP frequently showed honeycomb appearance (36 of 42 patients) though not in NSIP (p=0.0001). Six of 42 UIP patients (14.3%) met the ATS clinical criteria for IPF, and 3 of 16 NSIP patients (18.8%) met the diagnostic criteria. Conclusion : Being a relatively young female and having short duration of illness, fever, BAL lymphocytosis, low CD4/CD8 ratio with the absence of clubbing and honeycomb appearance in HRCT increase the likelihood of the illness being NSIP. The usefulness of ATS clinical diagnostic criteria for UIP may be low in Korea.
Halliday, Gary M.;Poon, Terence S.C.;Damian, Diona L.;Barnetson, Ross St.C.
Journal of Photoscience
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v.9
no.2
/
pp.236-239
/
2002
Sunscreens have been advocated as an important means of preventing skin cancer. UV-induced immunosuppression is important for skin cancer development, yet the effectiveness of sunscreens in protecting the human immune system from UV radiation is unclear. The only currently accepted method of sunscreen rating is the Sun Protection Factor (SPF) based on prevention of erythema. We developed an in vivo non-invasive method for evaluating protection of the human immune system from UV radiation based on recall contact sensitivity to nickel, a common allergen. Using this system we showed that broad-spectrum sunscreens provide greater protection to the immune system than sunscreens which protect from UVB only. UVA was found to be immunosuppressive. We developed this technique to enable the study of solar simulated UV radiation dose responses and determined Immune Protection Factors (IPFs) for six commercially available sunscreens based on limits of protection from the dose response data. We found that the IPF did not correlate with the SPF and that protection from erythema therefore cannot be used to predict protection of the immune system. However, IPF was significantly correlated to the UVA protective capability of the sunscreens, indicating that sunscreen protection from UVA is important for prevention of immunosuppression. We recommend that sunscreens should be rated against their immune protective capability to provide a better indication of their ability to protect against skin cancer.
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