Korean Guidelines for Diagnosis and Management of Interstitial Lung Diseases: Part 2. Idiopathic Pulmonary Fibrosis / 결핵및호흡기질환

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.

Korean Guidelines for Diagnosis and Management of Interstitial Lung Diseases: Part 2. Idiopathic Pulmonary Fibrosis / 결핵

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.

Sleep problems in children and adolescents at pediatric clinics / 소아과

PURPOSE: To investigate the frequency of childhood sleep problems at pediatric clinics in Seoul and Gyeonggi provinces. METHODS: Children (n=936) and their parents who visited 5 primary and 1 secondary pediatric outpatient clinics were invited to complete a Pediatric Sleep Questionnaire. RESULTS: Among patients, 901 (96.3%) answered questionnaires in sufficient detail for evaluation. The participant's mean age was 4.35±3.02 years (range, 0–18 years). The male to female ratio was 1:0.93 (466 boys, 435 girls). Habitual snoring (>3 day/week) was reported in 16.9% of the participants. The prevalence of habitual snoring in children 2 years. Insomnia was reported in 13.2% of children. The prevalence of sleepwalking, night terrors, and bruxism, is 1.6%, 19%, and 21.1%, respectively. Snoring was associated with increased incidence of sleepwalking, night terrors, and bruxism. Age was associated with insomnia and habitual snoring (P<0.05). Insomnia was more prevalent in younger (21%) than in older children (6%). Snoring was more frequent in both preschool (34%) and school-aged children (33%). The frequency of sleep disordered breathing and insomnia did not vary significantly with gender. However, snoring was more prevalent in boys. CONCLUSION: Sleep problems are frequent among children in Korea. Children with snoring have an increased risk of sleepwalking, night terror, and bruxism. Primary clinicians should consider children's sleep habits to improve their health.

Clinical Significance of Serum Autoantibodies in Idiopathic Interstitial Pneumonia

Although autoantibodies are routinely screened in patients with idiopathic interstitial pneumonia, there are no reliable data on their clinical usefulness. The aim of this study was to investigate the prognostic value of autoantibodies for predicting the development of new connective tissue disease in these patients and also mortality. We conducted retrospective analysis of the baseline, and follow-up data for 688 patients with idiopathic interstitial pneumonia (526 with idiopathic pulmonary fibrosis, 85 with nonspecific interstitial pneumonia, and 77 with cryptogenic organizing pneumonia) at one single tertiary referral center. The median follow-up period was 33.6 months. Antinuclear antibody was positive in 34.5% of all subjects, rheumatoid factor in 13.2%, and other specific autoantibodies were positive between 0.7%-6.8% of the cases. No significant difference in patient survival was found between the autoantibody-positive and -negative groups. However, the presence of autoantibodies, especially antinuclear antibody with a titer higher than 1:320, was a significant predictor for the future development of new connective tissue diseases (relative risk, 6.4), although the incidence was low (3.8% of all subjects during follow-up). In conclusion, autoantibodies are significant predictors for new connective tissue disease development, although they have no prognostic value.

Causes and Predictive Factors Associated with "Diagnosis Changed" Outcomes in Patients Notified as Tuberculosis Cases in a Private Tertiary Hospital / 결핵및호흡기질환

BACKGROUND: The aim of our study was to evaluate the "diagnosis changed" rate in patients notified as tuberculosis (TB) on the Korean TB surveillance system (KTBS). METHODS: A total of 1,273 patients notified as TB cases on the KTBS in one private tertiary hospital in 2011 were enrolled in the present study. Patients were classified into three groups: "diagnosis maintained", "diagnosis changed" (initially notified as TB, but ultimately diagnosed as non-TB), and "administrative error" (notified as TB due to administrative errors). RESULTS: Excluding 17 patients in the "administrative error" group, the "diagnosis maintained" and "diagnosis changed" groups included 1,097 (87.3%) and 159 patients (12.7%), respectively. Common causes of "diagnosis changed" were nontuberculous mycobacterial (NTM) disease (51.7%, 61/118), and pneumonia (17.8%) in cases notified as pulmonary TB, and meningitis (19.5%, 8/41) and Crohn's disease (12.2%) in cases notified as extrapulmonary TB. Being older than 35 years of age (odds ratio [OR], 2.18) and a positive acid-fast bacilli stain (OR, 1.58) were positive predictors and a TB-related radiological finding (OR, 0.42) was a negative predictor for a "diagnosis changed" result via multivariate logistic regression analysis in pulmonary TB cases. CONCLUSION: Because of a high "diagnosis changed" rate in TB notifications to the KTBS, the TB incidence rate measured by the KTBS may be overestimated. Considering the worldwide trend toward increased NTM disease, the "diagnosis changed" rate may increase over time. Thus, when reporting the annual TB notification rate in Korea, the exclusion of "diagnosis changed" cases is desirable.

Increasing Trend of Isolation of Non-Tuberculous Mycobacteria in a Tertiary University Hospital in South Korea / 결핵및호흡기질환

BACKGROUND: The isolation of non-tuberculous mycobacteria (NTM) has been increasing in South Korea. To date, however, the cause of this increase has not been determined, and it remains unclear whether the use of liquid media has contributed to this increase. The aim of this study was to evaluate the factors associated with NTM isolation and the impact of liquid media on NTM culture. METHODS: Mycobacterial smear/culture results of respiratory specimens (sputum and bronchial aspirates), obtained during the years 2002, 2005, and 2010, were retrieved and analyzed retrospectively. RESULTS: During the years 2002, 2005, and 2010, 83,096 sputum specimens were collected from 31,104 patients, and were cultured for mycobacteria, using solid media only in the 2002 and 2005 specimens and both solid and liquid media in the 2010. Of these, 3,516 (4.2%) specimens were smear-positive for acid-fast bacilli (AFB). The annual rate of NTM among positive culture specimens increased from 21% in 2002 to 57.8% in 2010 (p50 than < or =50 years. CONCLUSION: The rate of NTM isolation has steadily been increasing at the hospital in South Korea, likely due in part to the use of liquid media for the culture.

A Case of Pulmonary Alveolar Proteinosis Improved with Inhaled Granulocyte-Macrophage Colony-Stimulating Factor (GM-CSF) / 대한내과학회지

Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by the accumulation of lipoproteinaceous material within the alveoli. Several studies have recently found that autoantibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF) play a major role in the pathogenesis of idiopathic PAP. Consequently, inhaled or systemic injection of GM-CSF has been suggested as a promising treatment for PAP. A 54-year-old male visited our hospital for progressive dyspnea. Four years earlier, he was diagnosed with PAP based on a surgical lung biopsy in another institution. Whole-lung lavage was performed four times before he visited our hospital. We administered high-dose inhaled GM-CSF therapy for 12 weeks followed by 12 weeks of low-dose therapy. After the GM-CSF treatment, the patient's symptoms, lung function, and radiological findings were improved significantly.

A Case of POEMS Syndrome Associated with Sarcoidosis / 대한내과학회지

POEMS syndrome is a monoclonal plasma cell disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. Pulmonary manifestations of POEMS syndrome are common, with frequent symptomatic and asymptomatic respiratory involvement on presentation. However, there have been no reported cases of concomitant sarcoidosis and POEMS syndrome. We treated a 55-year-old woman who presented with dyspnea and bilateral lower leg pitting edema. She had been diagnosed with sarcoidosis 4 years earlier. We reassessed the patient meticulously and found polyneuropathy, IgG lambda type monoclonal gammopathy, lymphadenopathy, hepatosplenomegaly, hypothyroidism, hypertrichosis, and pleural and pericardial effusions, all of which led us to diagnose POEMS syndrome. Immunosuppression with high-dose dexamethasone led to clinical and radiological improvement.

A Case of Paratesticular Leiomyosarcoma Successfully Treated with Orchiectomy and Chemotherapy / Journal of the Korean Cancer Association, 대한암학회지

A 50-year-old male patient presented with a right scrotal mass that had been growing rapidly for more than one year. A heterogeneous enhancing right scrotal mass (12x9 cm) with para-aortic and peri-caval lymphadenopathies was found on abdominal computed tomography (CT). Right orchiectomy was performed and the gross finding had shown intact testis with a well-defined, huge, whitish solid mass adjacent to the testis. According to pathology, the mass was characterized as a leiomyosarcoma, grade 3 (by National Cancer Instituted [NCI] system). Therefore, the diagnosis was stage III, grade 3 paratesticular leiomyosarcoma. The patient underwent additional systemic chemotherapy using ifosfamide and adriamycin. After nine cycles of chemotherapy, positron emission tomography-CT was performed and no FDP uptake was observed. The patient has been followed up for 12 months after systemic chemotherapy, and he has maintained a complete response. We report here on a rare case of paratesticular leiomyosarcoma treated successfully with orichiectomy and additional systemic chemotherapy.

Clinical Characteristics of Idiopathic Pulmonary Fibrosis Patients with Diabetes Mellitus: the National Survey in Korea from 2003 to 2007

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.

A Case of Pulmonary Alveolar Proteinosis Improved with Inhaled Granulocyte-Macrophage Colony-Stimulating Factor (GM-CSF) / 대한내과학회지

Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by the accumulation of lipoproteinaceous material within the alveoli. Several studies have recently found that autoantibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF) play a major role in the pathogenesis of idiopathic PAP. Consequently, inhaled or systemic injection of GM-CSF has been suggested as a promising treatment for PAP. A 54-year-old male visited our hospital for progressive dyspnea. Four years earlier, he was diagnosed with PAP based on a surgical lung biopsy in another institution. Whole-lung lavage was performed four times before he visited our hospital. We administered high-dose inhaled GM-CSF therapy for 12 weeks followed by 12 weeks of low-dose therapy. After the GM-CSF treatment, the patient's symptoms, lung function, and radiological findings were improved significantly.

Mycobacterial Pulmonary Infections in Patients with Idiopathic Pulmonary Fibrosis

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.

A Case of Pulmonary Fibrosis with Microscopic Polyangiitis / 결핵및호흡기질환

A 65-year-old woman was admitted due to poor oral intake and a dry cough over the previous 3 months. The physical examination was remarkable for bibasilar crackles, and plain chest radiography showed reticulation in both lower lung fields. A pulmonary function test demonstrated a restrictive pattern with a reduced diffusing capacity of the lung for carbon monoxide. High resolution computed tomography showed reticulation and honey-combing in both peripheral lung zones, which was consistent with usual interstitial pneumonia pattern. Her skin showed livedo reticularis. The erythrocyte sedimentation rate and C-reactive protein level were elevated, and hematuria was noted on urinary analysis. A serologic test for auto-antibodies showed seropositivity for Myeloperoxidase-Anti-neutrophil cytoplasmic antibody (MPO-ANCA). A kidney biopsy was performed and showed focal segmental glomerulosclerosis. She was diagnosed as having pulmonary fibrosis with microscopic polyangiitis (MPA) and treated with high dose steroids. Here we report a case of pulmonary fibrosis coexistent with microscopic polyangiitis.

A Case of Acute Eosinophilic Pneumonia after Hematopoietic Stem Cell Transplantation / 결핵및호흡기질환

Pulmonary complications occur in 40~60% of patients who receive hematopoietic stem cell transplantation (HSCT) and are a source of substantial morbidity and mortality. Acute eosinophilic pneumonia (AEP) is an uncommon, non-infectious pulmonary complication occurring in HSCT recipients. We now report the case of a 52-year-old man with AEP who was treated with allogenic HSCT due to acute myeloid leukemia. He complained of fever, cough and dyspnea 390 days after allogenic HSCT. He also had skin and hepatic graft versus host disease (GVHD). Hypoxemia, diffuse pulmonary infiltrates on a chest x-ray and eosinophilia in bronchoalveolar lavage fluid were also noted in several tests. His symptoms, pulmonary infiltrates, hepatic dysfunction and skin lesions rapidly improved after treatment with corticosteroid therapy. Our case supports the idea that AEP is a late phase non-infectious pulmonary complication and one of the manifestations of chronic GVHD.

The Risk of Obstructive Lung Disease by Previous Pulmonary Tuberculosis in a Country with Intermediate Burden of Tuberculosis

We evaluated the effects of previous pulmonary tuberculosis (TB) on the risk of obstructive lung disease. We analyzed population-based, the Second Korea National Health and Nutrition Examination Survey 2001. Participants underwent chest X-rays (CXR) and spirometry, and qualified radiologists interpreted the presence of TB lesion independently. A total of 3,687 underwent acceptable spirometry and CXR. Two hundreds and ninty four subjects had evidence of previous TB on CXR with no subjects having evidence of active disease. Evidence of previous TB on CXR were independently associated with airflow obstruction (adjusted odds ratios [OR] = 2.56 [95% CI 1.84-3.56]) after adjustment for sex, age and smoking history. Previous TB was still a risk factor (adjusted OR = 3.13 [95% CI 1.86-5.29]) with exclusion of ever smokers or subjects with advanced lesion on CXR. Among never-smokers, the proportion of subjects with previous TB on CXR increased as obstructive lung disease became more severe. Previous TB is an independent risk factor for obstructive lung disease, even if the lesion is minimal and TB can be an important cause of obstructive lung disease in never-smokers. Effort on prevention and control of TB is crucial in reduction of obstructive lung disease, especially in countries with more than intermediate burden of TB.

Trends and Factors in Health Care Utilization of Patients with Chronic Obstructive Pulmonary Disease in Korea: A Nationwide Survey from 1990 through 2008 / 결핵및호흡기질환

BACKGROUND: Chronic obstructive pulmonary disease (COPD) is a major cause of death and disability worldwide and one of the most prevalent diseases in Korea. We examined trends and risk factors of health care utilization for COPD in Korea. METHODS: We retrospectively analyzed the database of Patient Surveys from 1990 through 2008, which were nationwide surveys of health services utilization through outpatient department (OPD) visits and hospitalization. Physician-diagnosed COPD patients whose ages were 45 years and older were included. RESULTS: OPD visits and hospitalization of COPD patients between 1990 and 2008 were estimated to be 68,552 and 17,774 persons, respectively. Trends in OPD visits and hospitalization for COPD significantly increased from 1990 through 2008 (p=0.019, p=0.001, respectively). The increment rate for OPD visits was 2.0 fold over those years; for hospitalization it was 3.3 fold. Risk factors for OPD visits for COPD were male gender (odd ration [OR], 1.41; 95% confidence interval [CI], 1.39~1.43), those aged 65 years and older (OR, 1.50; 95% CI, 1.47~1.53), residential area other than a metropolis (OR, 1.08; 95% CI, 1.07~1.010) and access to a physician's office (OR, 1.17; 95% CI, 1.14~1.21). Risk factors for hospitalization were male gender (OR, 2.15; 95% CI, 2.07~2.23), those aged 65 year and older (OR, 2.86; 95% CI, 2.72~3.00), residential area other than a metropolis (OR, 1.98; 95% CI, 1.90~2.07) and access to a hospital (OR, 2.88; 95% CI, 2.59~3.22) (p<0.001, both). CONCLUSION: Health care utilization for COPD subjects increased from 1990 to 2008. Risk factors for the utilization were male gender, older age, and residential area other than a metropolis.

Hypoxemia and Arrhythmia during Daily Activities and Six-minute Walk Test in Fibrotic Interstitial Lung Diseases

We performed 24-hr monitoring of pulse oximetric saturation (SpO2) with ECG and six-minute walk test (6MWT) in 19 patients with fibrotic interstitial lung diseases (ILD) to investigate; 1) The frequency and severity of hypoxemia and dysrhythmia during daily activities and 6MWT, 2) safety of 6MWT, and 3) the parameters of 6MWT which can replace 24-hr continuous monitoring of SpO2 to predict hypoxemia during daily activities. All patients experienced waking hour hypoxemia, and eight of nineteen patients spent > 10% of waking hours in hypoxemic state. Most patients experienced frequent arrhythmia, mostly atrial premature contractions (APCs) and ventricular premature contractions (VPCs). There were significant correlation between the variables of 6MWT and hypoxemia during daily activities. All of the patients who desaturated below 80% before 300 meters spent more than 10% of waking hour in hypoxemia (P = 0.018). In contrast to waking hour hypoxemia, SpO2 did not drop significantly during sleep except in the patients whose daytime resting SpO2 was already low. In conclusion, patients with fibrotic ILD showed significant period of hypoxemia during daily activities and frequent VPCs and APCs. Six-minute walk test is a useful surrogate marker of waking hour hypoxemia and seems to be safe without continuous monitoring of SpO2.

Comparison of Usual Interstitial Pneumonia and Nonspecific Interstitial Pneumonia: Quantification of Disease Severity and Discrimination between Two Diseases on HRCT Using a Texture-Based Automated System

OBJECTIVE: To evaluate the usefulness of an automated system for quantification and discrimination of usual interstitial pneumonia (UIP) and nonspecific interstitial pneumonia (NSIP). MATERIALS AND METHODS: An automated system to quantify six regional high-resolution CT (HRCT) patterns: normal, NL; ground-glass opacity, GGO; reticular opacity, RO; honeycombing, HC; emphysema, EMPH; and consolidation, CONS, was developed using texture and shape features. Fifty-four patients with pathologically proven UIP (n = 26) and pathologically proven NSIP (n = 28) were included as part of this study. Inter-observer agreement in measuring the extent of each HRCT pattern between the system and two thoracic radiologists were assessed in 26 randomly selected subsets using an interclass correlation coefficient (ICC). A linear regression analysis was used to assess the contribution of each disease pattern to the pulmonary function test parameters. The discriminating capacity of the system between UIP and NSIP was evaluated using a binomial logistic regression. RESULTS: The overall ICC showed acceptable agreement among the system and the two radiologists (r = 0.895 for the abnormal lung volume fraction, 0.706 for the fibrosis fraction, 0.895 for NL, 0.625 for GGO, 0.626 for RO, 0.893 for HC, 0.800 for EMPH, and 0.430 for CONS). The volumes of NL, GGO, RO, and EMPH contribute to forced expiratory volume during one second (FEV1) (r = 0.72, beta values, 0.84, 0.34, 0.34 and 0.24, respectively) and forced vital capacity (FVC) (r = 0.76, beta values, 0.82, 0.28, 0.21 and 0.34, respectively). For diffusing capacity (DLco), the volumes of NL and HC were independent contributors in opposite directions (r = 0.65, beta values, 0.64, -0.21, respectively). The automated system can help discriminate between UIP and NSIP with an accuracy of 82%. CONCLUSION: The automated quantification system of regional HRCT patterns can be useful in the assessment of disease severity and may provide reliable agreement with the radiologists' results. In addition, this system may be useful in differentiating between UIP and NSIP.

The Efficacy of Endobronchial Ultrasound-Guided Transbronchial Needle Aspiration in Mediastinal Staging of Non-Small Cell Lung Cancer in a University Hospital / 결핵및호흡기질환

BACKGROUND: In mediastinal lymph node sampling in non-small cell lung cancer (NSCLC) it is important to determine the appropriate treatment as well as to predict an outcome. Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is a recently developed, accurate, safe technique in patients with NSCLC for sampling mediastinal lymph nodes. We sought to determine the usefulness of EBUS-TBNA in mediastinal staging with NSCLC considered to be operable. METHODS: We retrospectively reviewed the records of 142 patients who underwent EBUS-TBNA for mediastinal staging in the Asan Medical Center, Korea from July 2008 to July 2010. If patients were in an operable state, they underwent subsequent surgical staging. Diagnoses based on biopsy results were compared with those based on surgical results. RESULTS: We performed EBUS-TBNA in 184 mediastinal lymph nodes in 142 NSCLC patients. Almost all of the EBUS-TBNA samples were from the lower paratracheal (112, 60.9%) and subcarinal (57, 31.0%) lymph nodes. In 142 patients, 51 patients (35.9%) were confirmed with malignant invasion of the mediastinal lymph node by EBUS-TBNA and 91 (64.1%) patients were not confirmed. Among the 91 patients, 64 patients (70.3%) underwent surgical staging. 3 patients (4.7%) who were misdiagnosed by the EBUS-TBNA were confirmed by surgery. After Diagnostic sensitivity of EBUS-TBNA, the prediction of mediastinal metastatsis was 94.4% and specificity was 100%. The procedures were performed safely and no serious complications were observed. CONCLUSION: We demonstrated the high diagnostic value of EBUS-TBNA for mediastinal staging.

A Case of Pulmonary Lymphangiomatosis / 결핵및호흡기질환

Pulmonary lymphangiomatosis is a rare disorder involving the entire intrathoracic lymphatic system from the mediastinum to the pleura. Pulmonary lymphangiomatosis mostly occurs in children and young adults without gender predilection. Although it is pathologically benign, it shows a progressive and fatal course with variable initial presentation. We now report a case of pulmonary lymphangiomatosis in a 35-year-old man. He presented with hemoptysis 6 months previously. Chest x-ray and a chest computed tomography scan showed diffuse interstitial thickening with left pleural effusion. Chylothorax was confirmed by thoracentesis. Lymphangiography showed dilated and tortuous lymphatic channels. Surgical lung biopsy revealed proliferation of complex anastomosing lymphatic channels. He was diagnosed with pulmonary lymophangiomatosis. Closed thoracostomy and chemical pleurodesis were done and the dyspnea was reduced.