Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrosing interstitial pneumonia, which presents with a progressive worsening dyspnea, and thus a poor outcome. The members of the Korean Academy of Tuberculosis and Respiratory Diseases as well as the participating members of the Korea Interstitial Lung Disease Study Group drafted this clinical practice guideline for IPF management. This guideline includes a wide range of topics, including the epidemiology, pathogenesis, risk factors, clinical features, diagnosis, treatment, prognosis, and acute exacerbation of IPF in Korea. Additionally, we suggested the PICO for the use of pirfenidone and nintendanib and for lung transplantation for the treatment of patients with IPF through a systemic literature review using experts' help in conducting a meta-analysis. We recommend this guideline to physicians, other health care professionals, and government personnel in Korea, to facilitate the treatment of patients with IPF.
Delivery of Health Care ; Diagnosis ; Disease Management ; Dyspnea ; Epidemiology ; Humans ; Idiopathic Pulmonary Fibrosis ; Korea ; Lung Diseases, Interstitial ; Lung Transplantation ; Prognosis ; Risk Factors ; Tuberculosis

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrosing interstitial pneumonia, which presents with a progressive worsening dyspnea, and thus a poor outcome. The members of the Korean Academy of Tuberculosis and Respiratory Diseases as well as the participating members of the Korea Interstitial Lung Disease Study Group drafted this clinical practice guideline for IPF management. This guideline includes a wide range of topics, including the epidemiology, pathogenesis, risk factors, clinical features, diagnosis, treatment, prognosis, and acute exacerbation of IPF in Korea. Additionally, we suggested the PICO for the use of pirfenidone and nintendanib and for lung transplantation for the treatment of patients with IPF through a systemic literature review using experts' help in conducting a meta-analysis. We recommend this guideline to physicians, other health care professionals, and government personnel in Korea, to facilitate the treatment of patients with IPF.

Evidence suggests that diabetes mellitus (DM) is associated with idiopathic pulmonary fibrosis (IPF). According to the new IPF guidelines, high-resolution computed tomography (HRCT) is an essential means of diagnosing IPF. We investigated the relationship between IPF and DM in patients treated between 2003 and 2007. Newly diagnosed IPF patients in large university teaching hospitals in Korea were enrolled from January 2003 to December 2007. We retrospectively analyzed 1,685 patients using the interstitial lung disease (ILD) registry. In total, 299 IPF patients (17.8%) also had DM. The mean age of our subjects was 68.0 +/- 9.4 yr. HRCT showed significantly more reticular and honeycomb patterns in IPF patients with DM than in IPF patients without DM (P = 0.014, P = 0.028, respectively). Furthermore, significantly higher incidences of hypertension, cardiovascular diseases, and other malignancies (except lung cancer) were found in IPF patients with DM than in IPF patients without DM. In conclusion, IPF patients with DM are more likely to have the usual interstitial pneumonia (UIP) pattern, including reticular and honeycomb patterns, on HRCT than are those without DM.
Aged ; Cardiovascular Diseases/epidemiology/etiology ; Diabetes Mellitus, Type 2/*complications ; Female ; Humans ; Hypertension/epidemiology/etiology ; Idiopathic Pulmonary Fibrosis/complications/*diagnosis/radiography ; Incidence ; Male ; Middle Aged ; Neoplasms/epidemiology/etiology ; Registries ; Republic of Korea/epidemiology ; Retrospective Studies ; Tomography, X-Ray Computed

Patients with idiopathic pulmonary fibrosis (IPF) have an increased risk for developing tuberculosis (TB). However, no studies have been reported regarding the development of nontuberculous mycobacterium (NTM) lung disease (NTMLD). We reviewed 795 patients with IPF from five university hospitals who were diagnosed by histological or radio-clinical criteria. In the 795 patients with IPF, pulmonary infections with mycobacterium tuberculosis (MTB) and NTM were found in 35 (4.4%) and 16 patients (2.0%), respectively, which was a higher frequency than that found in the general population. TB was more common in patients treated with immunosuppressants than in those who did not receive immunosuppressants (2.6% vs 1.4%, P = 0.12). Among the IPF patients who had mycobacterial infections,immunosuppressant users developed TB or NTMLD within 1 yr after treatment with immunosuppressants,while those occurred later than 2 yr after diagnosis of IPF in the subjects that did not receive immunosuppressants. Among 51 IPF patients who had mycobacterial infections, 9 (18%) died during follow-up. Of these, three died due to progression of pulmonary tuberculosis. TB and NTMLD is relatively common in patients with IPF in Korea and may be fatal in some groups. Careful evaluation of TB and NTMLD is necessary not only for immunosuppressant users, but also for nonusers with IPF.
Aged ; Cohort Studies ; Female ; Follow-Up Studies ; Hospitals, University ; Humans ; Idiopathic Pulmonary Fibrosis/complications/*diagnosis/pathology ; Immunosuppressive Agents/therapeutic use ; Male ; Middle Aged ; Mycobacterium Infections/complications/*diagnosis/drug therapy ; Mycobacterium Infections, Nontuberculous/*diagnosis/drug therapy/pathology ; Retrospective Studies ; Tuberculosis, Pulmonary/complications/*diagnosis/pathology

BACKGROUND/AIMS: To determine whether female smokers are more or less susceptible to the detrimental pulmonary-function effects of smoking when compared to male smokers among patients with lung cancer. METHODS: Pack-years and pulmonary function indices were compared between 1,594 men and women with lung cancer ifferences in individual susceptibility to smoking were estimated using a susceptibility index formula. RESULTS: Of the patients, 959 (92.8%) men and 74 (7.2%) women were current smokers. Common histological types of lung cancer were squamous cell carcinoma, adenocarcinoma, and small cell carcinoma, among others. Women had a lower number of pack-years, forced expiratory volume in 1 second (FEV1, liters), forced vital capacity (FVC, liters), and total lung capacity (TLC, liters) compared to those of men (25.0 +/- 19.2 vs. 42.9 +/- 21.7 for pack-years; 1.4 +/- 0.5 vs. 2.0 +/- 0.6 for FEV1; 3.0 +/- 0.7 vs. 2.0 +/- 0.6 for FVC; 4.5 +/- 0.8 vs. 5.7 +/- 1.0 for TLC; all p < 0.001). The susceptibility index for women was significantly higher compared to that of men (1.1 +/- 4.1 vs. 0.7 +/- 1.1; p = 0.001). A significant inverse association was shown between the susceptibility index and TLC and FVC (r = -0.200 for TLC, -0.273 for FVC; all p < 0.001). CONCLUSIONS: The results suggest that the detrimental effects of smoking on pulmonary function are greater in women, as compared to those in men, among patients with lung cancer.
Adult ; Aged ; Aged, 80 and over ; Chi-Square Distribution ; Cohort Studies ; Female ; *Gender Identity ; Humans ; Korea/epidemiology ; Lung Neoplasms/complications/*epidemiology/pathology ; Lung Volume Measurements ; Male ; Middle Aged ; Respiratory Function Tests ; Risk Assessment ; Sex Factors ; Smoking/*adverse effects/epidemiology

Slowly growing lung cancers are quite rare and the leading cause of length time bias and over-diagnosis bias in lung cancer screening. We report 6 cases of slowly growing lung cancer in a tertiary hospital between January 1999 and December 2008. The clinical characteristics of these 6 cases with slowly growing lung cancer were examined. The median age at diagnosis was 68 years (range, 49~72), and 5 patients (83%) were female. The most common histology type was adenocarcinoma (83%). After excluding two patients who showed no change in the tumor size, the median tumor doubling time was 189 months (range, 86~387). The proportion of patients with slowly growing lung cancer appears to be particularly large in women, especially among patients with adenocarcinoma. Our experience shows that slowly growly lung cancers are more heterogeneous and diverse.
Adenocarcinoma ; Bias (Epidemiology) ; Female ; Humans ; Lung ; Lung Neoplasms ; Mass Screening ; Tertiary Care Centers

Idiopathic pulmonary alveolar proteinosis (PAP) is a rare disorder in which lipoproteinaceous material accumulates within alveoli. There were few reports on Asian populations with idiopathic PAP. We retrospectively reviewed 38 patients with idiopathic PAP in Korea. We assessed clinical features, therapeutic efficacy and outcomes of whole lung lavage in patients with idiopathic PAP. The mean age at diagnosis was 52 yr. Eighty six percent of patients were symptomatic at diagnosis. Dyspnea and cough were the most common symptoms. Crackles were the most common physical examination finding. On pulmonary function test, a mild restrictive ventilatory defect was common, with a predicted mean forced vital capacity (FVC) of 77% and forced expiratory volume in one second (FEV1) of 84.6%. Diffusing capacity was disproportionately reduced at 67.7%. Arterial blood gas analysis revealed hypoxemia with a decreased PaO2 of 69.0 mmHg and an increased D(A-a)O2 of 34.2 mmHg. After whole lung lavage, PaO2, D(A-a)O2 and DLCO were significantly improved, but FVC and total lung capacity (TLC) were not different. This is the first multicenter study to analyze 38 Korean patients with idiopathic PAP. The clinical features and pulmonary parameters of Korean patients with idiopathic PAP are consistent with reports in other published studies. Whole lung lavage appears to be the most effective form of treatment.
Adult ; Biopsy ; Bronchoalveolar Lavage ; Female ; Humans ; Korea ; Male ; Middle Aged ; Pulmonary Alveolar Proteinosis/diagnosis/pathology/*physiopathology/*therapy ; Respiratory Function Tests ; Retrospective Studies ; Survival Rate ; Treatment Outcome

BACKGROUND: Pulmonary Langerhans cell histiocytosis (PLCH) is characterized by a proliferation of Langerhans cells and this results in granulomas that involve multiple organs of the body. Because the incidence of PLCH is very low in Korea and worldwide, collecting the clinical data of patients with PLCH nationwide is needed to determine the clinical features of Korean patients with PLCH. METHODS: The patients with PLCH confirmed by biopsy at any body site were included and the patients should have lung lesions present. A questionnaire that had items on the symptoms, lung function tests, the roentgenographic findings and the treatment was collected retrospectively at a Korean ILD Research Meeting. RESULTS: A total of 56 cases were collected. The number of males and females was 48 and 8, respectively, and their median age was 43 years (range: 18~67 years). The patients were current or ex-smokers in 79% of the cases. The most frequent symptom was coughing (39%), followed in decreasing order by dyspnea (38%), sputum (20%) and chest pain (20%). Pneumothorax was observed in 16 (29%) patients. Lung function tests showed a normal, restrictive, mixed or obstructive pattern in 26 (61%), 7 (16%), 7 (16%) and 3 patients (7%), respectively. Nodular-cystic lesion was most frequently observed in 59% of the patients on HRCT. The lung lesions were located in the middle and upper lobes in almost the cases. The median follow-up period was 90 months (range: 1~180 months) and only two patients died during this period. CONCLUSION: This study provides a national survey of the patients with PLCH during a long follow-up period.
Biopsy ; Chest Pain ; Cough ; Dyspnea ; Female ; Follow-Up Studies ; Granuloma ; Histiocytosis ; Histiocytosis, Langerhans-Cell ; Humans ; Incidence ; Korea ; Langerhans Cells ; Lung ; Lung Diseases ; Male ; Pneumothorax ; Surveys and Questionnaires ; Respiratory Function Tests ; Retrospective Studies ; Sputum

We report a case of pulmonary adenocarcinoma complicated by the syndrome of inappropriate secretion of antidiuretic hormone (SIADH) following adjuvant chemotherapy. A 51-year-old woman with stage IIIA adenocarcinoma received left lower lobe lobectomy in July, 2006. And then combination chemotherapy with paclitaxel and cisplatin was given to the patient. In five days after completion of second cycle of the chemotherapy, she visited emergency room because of general weakness and seizure. Her brain MRI was shown to be no evidence of brain metastasis. Serum sodium, urine and plasma osmolarities were 117mEq/L, 589 and 244mOsm/kg, respectively. She was improved with fluid restriction. Although occurrence of SIADH following chemotherapy is rare, physician should give an attention the potential for development of SIADH in the course of chemotherapyin non-small cell lung cancer patient.
Adenocarcinoma ; Brain ; Carcinoma, Non-Small-Cell Lung* ; Chemotherapy, Adjuvant* ; Cisplatin* ; Drug Therapy ; Drug Therapy, Combination ; Emergency Service, Hospital ; Female ; Humans ; Inappropriate ADH Syndrome ; Magnetic Resonance Imaging ; Middle Aged ; Neoplasm Metastasis ; Osmolar Concentration ; Paclitaxel* ; Plasma ; Seizures ; Sodium

BACKGROUND: The diagnostic and therapeutic approaches to mediastinal tumors and cysts have changed over the past three decades. This report summarizes our forty-two years of experience with these tumors. METHODS: This study retrospectively reviewed 479 patients with primary mediastinal tumors and cysts that were diagnosed and managed over the past 17-year period (1985~2002) and compared them to the report of the previous 25-year result (1960~1985) in Yonsei University College of Medicine, Severance Hospital in Seoul, Korea. RESULTS: During the 17 years, there were 479 cases of pathologically proven mediastinal tumors and cysts. Thymoma (38.2%) was the most common mediastinal tumor and has increased noticeably during recent years. The gender ratio showed a male predominance (1.3:1) and the age distributions were even over all the age groups. The most common sites of the tumor and the proportion(28.6%) of malignant tumors were the same as that previously reported. A diagnosis of a tumor in asymptomatic patients was possible in 174 cases (36.3%), which was higher that reported previously. The diagnostic yield of a fine needle aspiration biopsy was 68.6% in the total tumors and 80.9% in the malignant tumors. A surgical resection was the most frequently chosen treatment modality and was performed in 405 cases (84.6%). The complete resection rate was 91.1%, which is higher than the previous result of 78.8%. CONCLUSION: These results showed that the prevalence of mediastinal tumors and cysts, particularly thymoma, increased. A fine needle aspiration biopsy was a valuable preoperative differential diagnostic method for malignant tumors. The surgical and complete resection rate increased remarkably possibly due to the better applicable chest CT scans, the more frequent health check-up provided by the regular health promotion program for all people as a health insurance policy, and the improved diagnostic techniques in the pathologic, radiological, and clinical fields.
Age Distribution ; Biopsy ; Biopsy, Fine-Needle ; Diagnosis* ; Health Promotion ; Humans ; Insurance, Health ; Korea ; Male ; Prevalence ; Retrospective Studies ; Seoul ; Thymoma ; Tomography, X-Ray Computed