• Tuberculosis and Respiratory Diseases
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• 제84권4호
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• pp.255-262
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• 2021

Microscopic polyangiitis (MPA) is an antineutrophil cytoplasmic antibody (ANCA)-associated necrotizing vasculitis, which mainly affects small vessels in various organs, especially the lungs. The two key pulmonary manifestations, interstitial lung disease (ILD) and diffuse alveolar hemorrhage (DAH), increase the morbidity and death rate of patients with MPA. ILD is more common in MPA than in other ANCA-associated vasculitis subsets and is primarily associated with myeloperoxidase-ANCA. Unlike alveolar hemorrhage due to pulmonary capillaritis, ILD can initially manifest as isolated pulmonary fibrosis. Of note, its most frequent radiographic pattern is the usual interstitial pneumonia pattern, similar to the characteristic pattern seen in idiopathic pulmonary fibrosis. In this review we present the pathogenesis, clinical manifestations, and radiographic and histopathologic features of ILD and DAH in MPA. We also briefly summarize the outcome and therapeutic options for the two conditions.

• Korean Journal of Radiology
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• 제22권5호
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• pp.811-828
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• 2021

Following the introduction of a novel pathological concept of usual interstitial pneumonia (UIP) by Liebow and Carrington in 1969, diffuse interstitial pneumonia has evolved into UIP, nonspecific interstitial pneumonia (NSIP), and interstitial lung abnormality (ILA); the histopathological and CT findings of these conditions reflect the required multidisciplinary team approach, involving pulmonologists, radiologists, and pathologists, for their diagnosis and management. Concomitantly, traction bronchiectasis and bronchiolectasis have been recognized as the most persistent and important indices of the severity and prognosis of fibrotic lung diseases. The traction bronchiectasis index (TBI) can stratify the prognoses of patients with ILAs. In this review, the evolutionary concepts of UIP, NSIP, and ILAs are summarized in tables and figures, with a demonstration of the correlation between CT findings and pathologic evaluation. The CT-based UIP score is being proposed to facilitate a better understanding of the spectrum of pulmonary fibrosis, from ILAs to UIP, with emphasis on traction bronchiectasis/bronchiolectasis.

• Tuberculosis and Respiratory Diseases
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• 제41권6호
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• pp.597-603
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• 1994

연구배경 : 최근 HRCT의 개발로 폐실질의 미세한 변화까지 관찰할 수 있게 되었고, 특히 비침습적 방법으로 용이하게 여러번 반복하여 실시할 수 있는 장점이 있기 때문에, HRCT가 다른 검사보다 병의 경과, 또는 스테로이드 등 치료의 효과를 객관적으로 판단하는데 도움을 줄 수 있을 것으로 추정하여 본 연구를 실시하였다. 방법: 폐섬유증으로 진단받고 두번 이상 HRCT를 시행하였던 환자 13명에 대하여 치료 전후의 임상 증상(호흡 곤란 지수)의 변화, 폐기능 검사 소견의 변화와 영상 분석기로 측정한 HRCT상의 병변 범위의 변화와의 관계를 분석하였다. 결과: 1) 영상 분석기를 이용한 HRCT에서의 병변 범위 변화와 호흡 곤란 지수의 변화는 r=0.716, p=0.0012로 매우 유의한 상관관계를 나타내었다. 2) 호흡 곤란 지수의 변화와 확산능 변화 사이에는 r=-0.707, p=0.0047로 유의한 역상관관계가 있는 것으로 나타났다. 3) HRCT의 변화와 폐확산능의 변화는 유의한 상관관계를 보였으나 FVC나 TLC는 뚜렷한 연관성을 찾을 수 없었다. 결론: HRCT의 변화는 임상 증상의 변화 및 폐확산능의 변화와 서로 유의한 상관 관계를 보이는 것을 알았고, 향후 환자의 경과 관찰에 임상 증상, 폐확산능 및 HRCT의 추적 검사가 도움이 될 것으로 사료되었다.

• Tuberculosis and Respiratory Diseases
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• 제85권2호
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• pp.122-136
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• 2022

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 Chest Surgery
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• 제37권12호
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• pp.1032-1035
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• 2004

미만간질폐병에서 확진을 위해 개흉 폐 생검을 한다. 미만간질페병은 많은 경우에 있어서 페암과 병발한다. 미만간질페병환자의 폐생검후 발견된 폐 선암 증례 1예를 통해서, 미만간질폐병과 폐암의 상관관계의 고찰과, 폐 생검시 부위 및 생검 횟수에 대한 일반적 기준의 정립에 관해서 논의해보고자 문헌 고찰과 함께 보고한다.

• 대한영상의학회지
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• 제82권4호
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• pp.770-790
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• 2021

최신 국제 임상진료지침을 기반으로 한 특발폐섬유증의 진단은 부합하는 임상 소견과 함께 고해상 CT에서 전형적인 상용간질폐렴 소견을 보일 때 조직학적 폐 생검 없이 진단 가능하다. 영상 검사는 특발폐섬유증의 평가 및 진단에 중추적인 역할을 하며, 정확한 진단을 위하여 임상적, 영상의학적 및 병리학적 소견에 대한 다학제 검토의 중요성이 강조된다. 간질성폐이상(interstitial lung abnormality)은 우연히 발견된 영상의학적 이상 소견을 지칭하며, 간질성폐이상과 임상적으로 의미 있는 간질폐질환에 대한 구분은 적절한 임상 평가를 기반으로 이루어져야 한다. 저자들은 이번 종설을 통하여 특발폐섬유증 진단의 최신 지견 및 간질성폐이상에 대한 이해를 도움으로써 미만성 간질폐섬유증 환자의 정확한 진단과 치료 및 예후 증진에 도움이 되고자 한다.

• Tuberculosis and Respiratory Diseases
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• 제82권4호
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• pp.277-284
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• 2019

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%.

• Journal of Yeungnam Medical Science
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• 제36권1호
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• pp.8-15
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• 2019

Connective tissue diseases (CTDs) can affect all compartments of the lungs, including airways, alveoli, interstitium, vessels, and pleura. CTD-associated lung diseases (CTD-LDs) may present as diffuse lung disease or as focal lesions, and there is significant heterogeneity between the individual CTDs in their clinical and pathological manifestations. CTD-LDs may presage the clinical diagnosis a primary CTD, or it may develop in the context of an established CTD diagnosis. CTD-LDs reveal acute, chronic or mixed pattern of lung and pleural manifestations. Histopathological findings of diverse morphological changes can be present in CTD-LDs airway lesions (chronic bronchitis/bronchiolitis, follicular bronchiolitis, etc.), interstitial lung diseases (nonspecific interstitial pneumonia/fibrosis, usual interstitial pneumonia, lymphocytic interstitial pneumonia, diffuse alveolar damage, and organizing pneumonia), pleural changes (acute fibrinous or chronic fibrous pleuritis), and vascular changes (vasculitis, capillaritis, pulmonary hemorrhage, etc.). CTD patients can be exposed to various infectious diseases when taking immunosuppressive drugs. Histopathological patterns of CTD-LDs are generally nonspecific, and other diseases that can cause similar lesions in the lungs must be considered before the diagnosis of CTD-LDs. A multidisciplinary team involving pathologists, clinicians, and radiologists can adequately make a proper diagnosis of CTD-LDs.

• Journal of Chest Surgery
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• 제33권9호
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• pp.773-776
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• 2000

Accidental or suicidal fatalities of paraquat(Gramoxon) poisong are occasionally seen in the emergency room or intensive care unit in this country. In most cases, respiratory symptoms and eventual death by respiratory distress occur within several days. The most striking pathologic change is fibrosis of the lung due to widespread proliferation of fibroblastic cell. We experience a 21-year-old woman with huge bulla on left lung and diffuse fibrosis in other site, who ingested paraquat 10 months ago. After thoracoscopic removal of bulla, the patient survive without progression of pulmonary complication till now.

• Tuberculosis and Respiratory Diseases
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• 제51권1호
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• pp.59-64
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• 2001

급속히 악화되는 임상 경과를 보인 비특이성 간질성폐렴을 경험하였기에 보고하는 바이다.