• Tuberculosis and Respiratory Diseases
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• v.65 no.4
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• pp.328-333
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• 2008

Desquamative interstitial pneumonia is an uncommon form of interstitial lung diseases and it has a good prognosis compared with other types of idiopathic interstitial pneumonia. A 69-year old man was admitted to our hospital because of a 3-month history of dyspnea. The patient presented with hypoxemia. High-resolution computerized tomography of the patient showed ground glass opacity and traction bronchiectasis with subpleural early honeycombing on the both lung fields. The pathologic findings of the video-assisted thoracoscopy lung biopsy were compatible with desquamative interstitial pneumonia, and irregularly distributed interstitial fibrosis and inflammation were observed at the peripheral parenchyme. Oral predinsolone was started; his symptoms and chest x-ray were improved, and so he stopped taking the prednisolone. Ten months later, the desquamative interstitial pneumonia recurred. We report here on a case of recurrent desquamative interstitial pneumonia with fibrotic lung disease.

• Tuberculosis and Respiratory Diseases
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• v.54 no.2
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• pp.129-144
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• 2003

Usual interstitial pneumonia/Idiopathic pulmonary fibrosis, nonspecific interstitial pneumonia, Cryptogenic organizing pneumonia(bronchiolitis obliterans organizing pneumonia : BOOP), Acute interstitial pneumonia, respiratory bronchiolitis-associated interstitial lung disease, Desquamative interstitial pneumonia, Lymphoid interstitial pneumonia.

• Tuberculosis and Respiratory Diseases
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• v.58 no.4
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• pp.330-343
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• 2005

특발성 간질성 폐렴은 폐포보다는 폐간질을 주로 침범하는 미만성 염증성 섬유화 병변으로 병변의 분류에 임상적 및 병리학적으로 많은 혼동과 변화를 겪어왔다. 최근에는 미국흉부학회와 유럽호흡기학회의 공동 모임에서 이 질환 군에 해당되는 모든 임상과들이 모여서 7가지의 병변으로 재분류 하였는데, 이는 Idiopathic pulmonary fibrosis, Nonspecific interstitial pneumonia, Cryptogenic organizing pneumonia, Acute interstitial pneumonia, Respiratory bronchiolitis interstitial lung disease, Desquamative interstitial pneumonia, Lymphocytic interstitial pneumonia 등이다. 이에 저자는 최근 분류에 의한 특발성 간질성 폐렴의 7가지 병변을 영상 소견을 중심으로 기술하고자 한다.

• Clinical and Experimental Pediatrics
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• v.45 no.4
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• pp.529-534
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• 2002

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 ($FEV_1$) 29% and $FEV_1/FVC$ 90%, so a restrictive pulmonary insufficiency was found.

• Tuberculosis and Respiratory Diseases
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• v.82 no.4
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• pp.269-276
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• 2019

Idiopathic interstitial pneumonia (IIP) is a histologically identifiable pulmonary disease without a known cause that usually infiltrates the lung interstitium. IIP is largely classified into idiopathic pulmonary fibrosis, idiopathic non-specific interstitial pneumonia, respiratory bronchiolitis-interstitial lung disease (ILD), cryptogenic organizing pneumonia, desquamative interstitial pneumonia, and acute interstitial pneumonia. Each of these diseases has a different prognosis and requires specific treatment, and a multidisciplinary approach that combines chest high-resolution computed tomography (HRCT), histological findings, and clinical findings is necessary for their diagnosis. Diagnosis of IIP is made based on clinical presentation, chest HRCT findings, results of pulmonary function tests, and histological findings. For histological diagnosis, video-assisted thoracoscopic biopsy and transbronchial lung biopsy are used. In order to identify ILD associated with connective tissue disease, autoimmune antibody tests may also be necessary. Many biomarkers associated with disease prognosis have been recently discovered, and future research on their clinical significance is necessary. The diagnosis of ILD is difficult because patterns of ILD are both complicated and variable. Therefore, as with other diseases, accurate history taking and meticulous physical examination are crucial.

• Tuberculosis and Respiratory Diseases
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• v.66 no.2
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• pp.141-151
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• 2009

Background: There is limited data on the epidemiology and relative frequency of idiopathic interstitial pneumonia (IIP) worldwide. This survey was performed to assess the epidemiology and relative frequency of IIP in Korea. Methods: The patients with IIP and who were confirmed by lung biopsy, except those patients with idiopathic pulmonary fibrosis, (IPF) over a 5 year period (from Jan. $1^{st}$, 2003 to Dec. $31^{st}$, 2007) were registered by a web-base questionnaire. Results: A total of 3,156 cases were registered, but 970 cases were excluded due to duplicative registration, inadequate data and the unmet ATS/ERS diagnostic criteria. A total of 2,186 cases were analyzed. The male to female ratio was about 2 : 1 and their mean age was 65 (range: 11-94). The most frequent disease was IPF (77.1%), followed in decreasing order by nonspecific interstitial pneumonia (NSIP) (11.9%), cryptogenic organizing pneumonia (COP) (8.5%), acute interstitial pneumonia (AIP) (1.1%), desquamative interstitial pneumonia (DIP) (0.9%), respiratory bronchiolitis-interstitial lung disease (RB-ILD) (0.4%) and lymphocytic interstitial pneumonia (LIP) (0.1%). The mean age of the patients with IPF, NSIP and COP was 67.8, 57.1 and 57.7 years old, respectively. The most frequent symptom was dyspnea on exertion (69%) followed by coughing (61%) and sputum (33%) for the whole population. The three year survival rate was 62% for the patients with IPF and the five year survival rate was 85% in both the NSIP and COP patients. Conclusion: This survey provides helpful information for the management of IIP and to produce management guidelines for this illness in Korea.

• Tuberculosis and Respiratory Diseases
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• v.49 no.5
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• pp.601-613
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• 2000

Background : Idiopathic pulmonary fibrosis (IPF) is a fatal progressive fibrous disease of the lung of unknown etiology. Recently it has been classified into several distinct entities on the basis of pathologic and clinical characteristics, ie : usual interstitial pneumonia (UIP), desquamative interstitial pneumonia (DIP), acute interstitial pneumonia (AIP), bronchiolitis obliterans with organizing pneumonia (BOOP), and nonspecific interstitial pneumonia (NSIP). IPF is now applied only for UIP, which has the worst prognosis. The previous reports of 3-5 year median survival appears to be overoptimistic because other types with better prognosis like NSIP or BOOP might have been included. Therefore, this study was performed to determine the clinical course and the prognostic factors of UIP as diagnosed by surgical lung biopsy. Methods : The subjects were 72 UIP patients (age $58.2{\pm}11.6$ years, M : F=45 : 27, median follow up period : 18.1 months (0.7-103.6) diagnosed by surgical lung biopsy at the Asan Medical Center (68 patients) and the Paik Hospital in Seoul (4 patients). Clinical scores (level of dyspnea : 1-20 points), radiologic score (honeycombing : HC score 0-5 points, ground glass : GG score 0-5 points), and physiologic scores (FVC : 1-12 points, $FEV_1$ : 0-3 points, TLC : 0-10 points, $D_{LCO)$ : 0-5 points, $AaDO_2$ : 0-10 points) were summed into a total CRP score. Results : 1) The one year survival rate was 78.3%, while the rate for three year survival was 58.1%, and the median survival period was 42.5months. 2) Short term (1 year) prognosis : The patients who died within one year of diagnosis (14 patients) had the higher initial total CRP score ($28.6{\pm}8.3$ vs. $16.6{\pm}9.7$) than those who lived longer than one year (46 patients). The difference in the total CRP score was attributed to the symptom score ($8.4{\pm}2.1$ vs. $5.7{\pm}3.9$) and the physiologic score ($15.7{\pm}7.1$ vs. $6.7{\pm}5.7$) including FVC, $D_{LCO)$ and $AaDO_2$. 3) Long-term (3year) prognosis : The total CRP score ($12.2{\pm}6.7$ vs. $28.7{\pm}7.9$ : including symptom score, FVC, $D_{LCO)$ and $AaDO_2$) at the time of diagnosis were also different for the long-term survivors and those who lived less than 3 years. 4) Cox regression analysis showed $D_{LCO)$ (${\geq}$60%) (Hazard ratio : 4.56, 95% CI : 2.30-16.04) was the independent prognostic factors of UIP (P<0.05). Conclusion : These results suggest that $D_{LCO)$ at the time of diagnosis seem to be a prognostic markers of biopsy-proven UIP.