Investigation of respiratory muscle strength and its influence on exercise capacity and quality of life in patients with idiopathic pulmonary fibrosis.

BACKGROUND: Adequate respiratory muscle strength is required to meet the increased ventilatory demand during physical activities. However, it is not well known whether respiratory muscle strength is impaired in patients with idiopathic pulmonary fibrosis (IPF). OBJECTIVES: This study aimed to investigate the relationship between respiratory muscle strength and exercise capacity, quality of life, physical activity level, and fatigue in IPF patients. METHODS: The study comprised 30 individuals with idiopathic pulmonary fibrosis (IPF) and 30 healthy controls. Maximal inspiratory pressure (MIP) and maximal expiratory pressure (MEP) were measured to assess respiratory muscle strength. The International Physical Activity Questionnaire-Short Form, 6-minute walk test distance (6MWD), St George Respiratory Questionnaire (SGRQ), and Fatigue Severity Scale (FSS) were employed to evaluate physical activity level, exercise capacity, quality of life, and fatigue severity, respectively. RESULTS: MIP (81±29 vs.73±20 cmH2O) and MEP (93±31 vs. 93±34 cmH2O) did not differ significantly between IPF patients and controls (p>0.05). In patients with IPF, MIP was significantly correlated with 6MWD (r=0.533), SGRQ (r=-0.428), and FSS (r=-0.433). Multivariate models including MIP, MEP, FEV1, FVC, and PA level explained 74% of the variance in the 6MWD (p<0.001), and MIP, FEV1, and PA level were independent predictors of the 6MWD, with FEV1 being the strongest predictor (ß=0.659). Multivariate models predicting SGRQ revealed none of MIP, FEV1 or PA level was directly influencing the SGRQ score. CONCLUSIONS: This study suggests that patients with IPF do not have respiratory muscle weakness. Inspiratory muscle strength has a direct influence on exercise capacity but an indirect effect on quality of life, probably by influencing exercise capacity.

The clinical, functional, and radiological effect of long-term used immunosuppressive therapy for post-COVID-19 interstitial lung disease.

BACKGROUND AND AIM: Persistent interstitial lung disease (ILD) after COVID-19 infection can lead to severe loss of respiratory function and a decrease in the quality of life. There is no consensus regarding the treatment of post-COVID-19-ILD. This study aims to investigate the effectiveness of immunosuppressive treatment for this group of patients. METHODS: This retrospective observational study included patients diagnosed with post-COVID-19-ILD from 2021 to 2022. Patients who had pulmonary symptoms, required prolonged oxygen therapy, and/or had restrictive pulmonary function test (PFT) and/or DLCO <80%, with diffuse parenchymal involvement on high-resolution computed tomography (HRCT), were given immunosuppressive treatment with methylprednisolone and/or mycophenolate mofetil (MMF) and followed up for 6 months. RESULTS: Among the 48 patients, 35 were treated. Two patients were excluded due to discontinued treatment and passed away before the study period ended. Of 33 cases, 21 (66.6%) were treated with methylprednisolone, 11 (33%) with methylprednisolone + MMF, and 1 (0.4%) with MMF alone. Comparing baseline and 6th-month data revealed significant improvement in mMRC score, saturation (SpO2), FVC, FVC%, FEV%, and DLCO% values (p<0.005). While regression was observed in all radiologic findings, regression in ground glass and reticulation was statistically significant (p<0.005). When the 1st and 6th-month data were compared, a significant increase was observed in SpO2 and DLCO% values (p=0.016) and there was a significant regression in reticulation radiologically (p=0.01). CONCLUSIONS: Long-term immunosuppressive therapy may be preferred in proper cases of post-COVID-19-ILD as an effective and safe treatment option that improves the quality of life, respiratory parameters, and radiologic findings.

Serum Specific Antibodies Do Not Seem to Have an Additional Role in the Diagnosis of Hypersensitivity Pneumonitis.

BACKGROUND: We aimed to investigate the contribution of serum IgG testing to the history of exposure in the diagnosis of fibrotic hypersensitivity pneumonitis. METHODS: A single-center, retrospective, cross-sectional study including 63 patients pathologically diagnosed with fibrotic hypersensitivity pneumonitis in line with the guidelines of the American Thoracic Society. Descriptive statistics were presented and Kappa statistic was performed to evaluate the compatibility between panel and the history of exposure. RESULTS: The median age was 63 (22-81) years and 34 (54%) were male. Forty-six patients (73%) had a positive history of exposure. Thirty-nine patients (61.9%) had a positive HP/Avian panel. The most common exposure agent was mold (34.9%), followed by parakeet (31.7%). The antibody detected the most was penicillium chrysogenum lgG (36.5%), followed by aspergillus fumigatus (31.8%). There was no compatibility between HP/Avian panel and history of exposure (kappa coefficient= 0.18, p= 0.14). When the exposure was only assessed based on the history, 4 (6.35%) patients were diagnosed as fibrotic hypersensitivity pneumonitis with low confidence, 6 (9.52%) with moderate confidence, 11 (17.46%) with high confidence and 42 (66.67%) with definite confidence; whereas 4 (6.35%) patients were diagnosed as fibrotic hypersensitivity pneumonitis with low confidence, 6 (9.52%) with moderate confidence, 9 (14.29%) patients with high confidence and 44 (69.84%) patients with definite confidence if exposure was evaluated with history and/or panel. CONCLUSIONS: Serum specific precipitating antibody panel does not seem to provide additional value to the history of exposure in the diagnosis of fibrotic hypersensitivity pneumonitis.

Protective efficacy of pirfenidone in rats with pulmonary fibrosis induced by bleomycin.

BACKGROUND: Bleomycin causes increased production of reactive oxygen species, leads to pulmonary toxicity, fibroblast activation, and fibrosis. OBJECTIVES: This study aimed to evaluate the protective effect of pirfenidone on bleomycin-induced lung toxicity in rats. METHODS: Twenty-eight adult rats were randomly divided into 3 groups; Bleomycin (B group, n=10), Bleomycin and Pirfenidone (B-PND group, n=13), and the control group (n=5). The bleomycin regimen was administered for 9 weeks. Pirfenidone was administered at 100 mg/kg daily. Total antioxidant level (TAS), total oxidant level (TOS), tumor necrosis factor (TNF-α), transforming growth factor (TGF-ß1), matrix metalloproteinase-2 (MMP-2), plasminogen activator inhibitor (PAI) levels were studied. Histopathologically, sections were stained with Hematoxylin-eosin and Masson-trichrome for grading-scoring according to the Ashcroft score. RESULTS: Stage 3 fibrosis was observed in 50% of the B group rats, stage 3 and higher fibrosis was never detected in the B-PND group and the difference was statistically significant (p=0.003). When evaluating tissue inflammation, the inflammation was higher in the B-PND group than in the other groups (p<0.001). Pleuritis was detected in all rats in group B, while was not observed in B-PND and control group (p<0.001). The TAS level was found to be significantly higher in group B than in group B-PND (p=0.034), while no difference was found between TOS, TNF-α, MMP-2, PAI, TGF-ß1. CONCLUSIONS: Pirfenidone had a statistically significant protective effect in bleomycin-induced lung fibrosis and pleuritis in rats.  Despite the presence of inflammation in the tissue, no significant changes were observed in inflammation markers in the peripheral blood. Novel serum biomarkers are needed to indicate the presence of inflammation and fibrosis in the lung.

A rare case: Endobronchial solitary mixed papilloma.

Endobronchial solitary papillomas are extremely rare lung neoplasms originating from the bronchial surface epithelium. They often present with cough or recurrent hemoptysis. These tumors are benign, but they should be followed closely because they may even have a low probability of malignant transformation features. It should be kept in mind that malignancy may develop especially if the patient is a smoker. Although the etiology is not known for certain, it is thought to be caused by human papillomavirus in some cases. A 43-year-old male patient was admitted with a complaint of chronic cough. Rigid bronchoscopy was performed for diagnostic and therapeutic purposes after imaging techniques revealed a lesion obstructing the lumen of the right main bronchus. The pathology result was reported as mixed bronchial papilloma. We aimed to present our case because of its rarity and to indicate that chronic cough must be further evaluated.

The role of endobronchial ultrasonography elastography in the diagnosis of hilar and mediastinal lymph nodes.

BACKGROUND: Endobronchial ultrasonography (EBUS) is a minimally invasive diagnostic tool in the diagnosis of mediastinal lymph nodes (LNs) and has sonographic features. We aimed to investigate the diagnostic accuracy of EBUS elastography, which evaluates tissue compressibility integrated into EBUS, on malignant vs. benign mediastinal-hilar LNs. METHODS: A single-center, prospective study was conducted at the University of Health Sciences Yedikule Chest Diseases and Thoracic Surgery Training and Research Hospital between 01/10/2019 and 15/11/2019. The features of 219 LNs evaluated by thoracic computed tomography (CT), positron emission tomography (PET)/CT, EBUS sonography and EBUS elastography were recorded. The LNs sampled by EBUS-guided fine needle aspiration were classified according to EBUS elastography color distribution findings as follows: type 1, predominantly nonblue (green, yellow, and red); type 2, part blue, part nonblue; type 3, predominantly blue. The strain ratio (SR) was calculated based on normal tissue with the relevant region. RESULTS: The average age of 131 patients included in the study was 55.86 ± 13 years, 76 (58%) were male. Two hundred and nineteen lymph nodes were sampled from different stations. Pathological diagnosis of 75 (34.2%) LNs was malignant, the rest was benign. When EBUS B-mode findings and pathological results were compared, sensitivity was 65.33%, specificity 63.19%, positive predictive value (PPV) 48%, negative predictive value (NPV) 77.8%, and diagnostic yield (DY) 64%. When the pathological diagnoses and EBUS elastography findings were compared, while type 1 LNs were considered to be benign and type 3 LNs malignant, sensitivity 94.12%, specificity 86.54%, PPV 82.1%, NPV 95.7%, and DY 89.5%. SR of malignant LNs was significantly higher than benign LNs (p < 0.001). When the classification according to color scale and SR were compared, no difference was found in DY (p = 0.155). DISCUSSION: The diagnostic accuracy of EBUS elastography is high enough to distinguish malignant LN from benign ones with the SR option. When compared with EBUS-B mode sonographic findings, it was found to have a higher diagnostic yield.

Factors Associated With Diagnostic Accuracy of Robotic Bronchoscopy With 12-Month Follow-up.

BACKGROUND: Robotic bronchoscopy (RB) aims to increase the diagnostic yield of guided bronchoscopy by providing improved navigation, farther reach, and stability during lesion sampling. METHODS: We reviewed data on consecutive cases in which RB was used to diagnose lung lesions from June 15, 2018, to December 15, 2019, at the University of Chicago Medical Center. RESULTS: The median lesion size was 20.5 mm. All patients had at least 12 months of follow-up. The overall diagnostic accuracy was 77% (95 of 124). The diagnostic accuracy was 85%, 84%, and 38% for concentric, eccentric, and absent radial endobronchial ultrasound (r-EBUS) views, respectively (P < .001). A positive r-EBUS view and lesions size of 20 to 30 mm had higher odds of achieving a diagnosis on multivariate analysis. The 12-month diagnostic accuracy, sensitivity, specificity, and positive and negative predictive value for malignancy were 77%, 69%, 100%, 100%, and 58%, respectively. Pneumothorax was noted in 1.6% (n = 2) patients with bleeding reported in 3.2% (n = 4). No postprocedure respiratory failure was noted. CONCLUSIONS: The overall diagnostic accuracy using RB for pulmonary lesion sampling in our cohort with 12-month follow-up compared favorably with established guided bronchoscopy technologies. Lesion size ≥20 mm and confirmation by r-EBUS predicted higher accuracy independent of concentric or eccentric r-EBUS patterns.

Modified successful desensitization protocol of infliximab in a patient with sarcoidosis unable to tolerate corticosteroids and refractory to alternative therapeutics.

Glucocorticoids are the primary treatment choices for sarcoidosis. However, some patients are resistant to corticosteroids or have side effects and may not respond to alternative treatments added to reduce corticosteroid therapy. Evidence has demonstrated the critical role of Infliximab [anti-tumor necrosis factor (TNF)-α] which is a chimeric IgG1 monoclonal antibody in the pathogenesis of granulomatous inflammation. In this paper, we present a patient who improved clinically and radiologically with infliximab treatment, which was initiated due to the development of serious side effects associated with corticosteroids; however, following unresponsiveness to other therapeutic drugs initiated due to relapse, restarted infliximab, and developed an early hypersensitivity reaction. With infliximab, the frequency of early-type hypersensitivity reactions is 2-3%. In such cases, drug desensitization is an effective and safe treatment option. Different desensitization protocols have been defined with infliximab, and the frequency of reactions during desensitization has been reported as 29%, especially in the last step. With the desensitization protocol we have modified, patients with a history of early-type hypersensitivity reaction with infliximab will have the chance to take this effective drug more safely and effortlessly.

Frequency of alpha-1 antitrypsin deficiency and unexpected results in COPD patients in Turkey; rare variants are common.

BACKGROUND: Alpha-1 antitrypsin (α1-AT) is a protease inhibitor that is largely released from liver cells. It inhibits neutrophil elastase and its deficiency increases the risk of developing chronic obstructive pulmonary disease (COPD). The frequency of α1-AT deficiency has been reported with different prevalence rates in different parts of the world. The most common α1-AT variant causing α1-AT deficiency is the Pi*Z allele. In this study, we aimed to determine the frequency of the α1-AT genotypic variant in COPD patients in our country. METHODS: In this study, 196 consecutive COPD patients admitted to our clinic were included. In addition to recording the demographic data of the volunteers, a dry drop of blood sample was taken from the fingertip for the SERPINA1 genotype study. RESULTS: One hundred and fifty-eight (80.6%) of the patients were male and the mean age was 56.92 ± 9.84 years. A variant in the SERPINA1 gene was detected in a total of 14 (7.1%) COPD patients. Pi*ZZ homozygous variant was detected in only 1 (0.51%) patient, while Pi*MZ was detected in 3 (1.53%) patients. The Pi*S variant was never detected. Various rare heterozygous variants were detected in 9 (4.6%) patients and a single point mutation was found in one (0.51%) patient. Serum α1-AT levels were significantly lower in patients with variants compared to the Pi*MM group (p < 0.001). DISCUSSION: In this study, which investigated the genotypic α1-AT variant frequency in COPD patients for the first time in our country, we found that the percentage of homozygous Pi*ZZ patients was 0.51%, but when heterozygous α1-AT gene variant and single point mutation were included, the frequency was 7.1%. At the same time, while the Pi*S variant was never detected, rare variants were found more frequently than expected.

Transbronchial cryobiopsy for diagnosing parenchymal lung diseases: real-life experience from a tertiary referral center.

BACKGROUND AND OBJECTIVES: Transbronchial cryobiopsy (cryo-TBB) is increasingly being used in the diagnosis of diffuse parenchymal lung diseases (DPLD). Varying diagnostic success and complication rates have been reported. Herein we report our experience with cryo-TBB, focusing on diagnostic yield, factors affecting diagnosis, and safety. METHODS: This retrospective study was conducted in a tertiary referral chest diseases hospital. Data regarding the patients, procedures, complication rates, diagnostic yield, and the final diagnosis made by a multidisciplinary committee at all diagnosis stages were evaluated. RESULTS: We recruited 147 patients with suspected DPLD. The definitive diagnosis was made pathologically in 98 of 147 patients (66.6%) and using a multidisciplinary approach in 109 of 147 (74.1%) cases. The number of samples had a significant effect on diagnostic success. Histopathologic diagnostic yield and diagnostic yield with a multidisciplinary committee after a single biopsy were 50%, and histopathological diagnostic yield and diagnostic yield with multidisciplinary committee increased to 71.4% and 85.7%, respectively, with a second biopsy (p = 0.034). The incidence of mild-to-moderate hemorrhage was 31.9%; no severe hemorrhage occurred. Pneumothorax rate was 15.6%, and the mortality rate was 0.68%. CONCLUSIONS: Cryo-TBB has sufficient diagnostic yield in the context of a multidisciplinary diagnosis with acceptable complication rates. Performing at least 2 biopsies and from at least 2 segments increases diagnostic success.

Assessment of epicardial adipose tissue thickness and total calcium score in sarcoidosis patients.

OBJECTIVE: Increased thickness of epicardial adipose tissue (EAT) and the total coronary artery calcium score (TCACS) are independent predictors of atherosclerosis. The aim of this study was to investigate whether EAT thickness, measured using thoracic computed tomography, and TCACS were greater in patients with sarcoidosis. METHODS: This was a retrospective study. The details of participants who presented at the cardiology and pulmonology outpatient clinics between January 2011 and December 2018 with dyspnea, chest pain, or palpitations from the hospital data system were reviewed. Patients with transthoracic echocardiography and thorax computed tomography (CT) (CT) records were identified, and those who were diagnosed with sarcoidosis, had no other health problems, and did not take any medication were included in the study. RESULTS: A total of 45 controls and 78 sarcoidosis patients were enrolled. The mean age of the controls was 46.15±13.1 years, while it was 46.26±12.37 years in the sarcoidosis group, which represented no significant difference between the groups (p>0.05). When the groups were compared in terms of a fasting blood test, erythrocyte sedimentation rate (ESR), TCACS, EAT thickness, levels of C-reactive protein (CRP), total cholesterol, low-density lipoprotein (LDL), and triglycerides, it was observed that CRP and EAT thickness were higher in the sarcoidosis group. CONCLUSION: The results of this study indicated that the thickness of EAT calculated using thorax CT was greater in sarcoidosis patients; however, the TCACS was similar in both groups. In addition, there was a positive correlation between EAT thickness and the level of total cholesterol, LDL, triglycerides, CRP, and the sedimentation rate. These findings suggest that atherosclerosis may start earlier in those with sarcoidosis than in the healthy population.

Real life results of coil treatment for bronchoscopic lung volume reduction in emphysema.

INTRODUCTION: The lung volume reduction coil treatment is a minimally invasive bronchoscopic treatment option for emphysema patients who suffer from severe hyperinflation. Previous studies have reported successful outcomes in selected cases using coil for bronchoscopic lung volume reduction (BLVR). Our aim is to determine the changes in respiratory function tests, perception of dyspnea and exercise capacities after 12 months in patients treated with endobronchial coil. MATERIALS AND METHODS: The data of patients with severe emphysema and treated with coils between 2014-2017 were evaluated retrospectively. Dynamic and static lung volume capacities at baseline and 12 months, modified Medical Research Council (mMRC) questionnaire and six-minute walk test (6-MWT) results were recorded. RESULT: BLVR was performed in thirty patients (one female, twenty-nine males). Five patients were treated bilaterally and twentyfive unilaterally. One patient died after 7 days and 4 patients died during follow-up. Five patients were lost to follow-up. A total of twenty patients with available data were included in the study. A statistically significant difference was found in mMRC results in pre-treatment and 12-month evaluations. There was no significant difference in FEV1, TLC and RV values at the end of 12 months. There was an increase of 18.9 meters (± 83.5 m) between the baseline and 12 months in 6-MWT. 45% of the patients improved their walking distance over 26 meters which is known as minimal clinically important difference (MCID). CONCLUSIONS: Although no significant changes were observed in pulmonary function tests and lung volumes, the increase in exercise capacity and decreased perception of dyspnea indicate the efficacy of endobronchial coil.

A comparative study of the effectiveness of hospital-based versus home-based pulmonary rehabilitation in candidates for bronchoscopic lung volume reduction.

BACKGROUND: The Bronchoscopic Lung Volume Reduction (BLVR) is recommended in patients with severe Chronic Obstructive Pulmonary Disease (COPD) who are still symptomatic and have hyperinflation despite having received optimal medical therapy and Pulmonary Rehabilitation (PR). However, the small number of PR centers is insufficient to compensate for the need for existing hospital-based PR programs. OBJECTIVE: This article aimed to compare between hospital-based and home-based PR programs in terms of effectiveness on BLVR candidates. METHODS: This study is a prospective, controlled, nonrandomized clinical trial. Stable COPD patients who were referred to our PR clinic prior to BLVR were recruited consecutively. Patients were evaluated in two groups, hospital-based PR (Group 1) or home-based PR (Group 2). Both groups were admitted to the recommended PR for eight weeks. Pulmonary function tests, modified Medical Research Council (mMRC) dyspnea scale, COPD Assessment Test (CAT) and the 6-min walk distance (6MWD) were assessed for each patient before and after PR. RESULTS: A total of 67 patients were enrolled in the study. The max. age was 79 years and min. age was 49 years, with 65(±7.45) as a mean ±SD. Improvements in the mMRC and CAT scores after PR in both groups were significant and a similar level. Whereas, 6MWD was only significantly increased in Group 1. CONCLUSIONS: This study, demonstrated that both home-based and hospital-based PR provided significant and similar improvements in the mMRC and CAT scores but 6MWD was only significantly increased in the hospital-based PR. Since 6MWD after PR plays a major role in BLVR eligibility, our findings suggest that hospital-based PR may be the most appropriate method for BLVR candidates.

The impact of co-existing comorbid diseases on long-term mortality in bronchoscopic lung volume reduction.

Aim Emphysema is a lung disease in which alveolar capillary units are destroyed supporting tissue lost. Bronchoscopic lung volume reduction (BLVR) is a novel treatment for emphysema. Several comorbidities have been reported to coexist in patients with chronic obstructive pulmonary disease (COPD). The aim of this study was to evaluate comorbidities of patients with severe emphysema who underwent BLVR and association of these comorbidities with mortality. Methods Between January 2011 and December 2017 the records of severe emphysema patients who underwent endobronchial valve (EBV) or lung volume reduction coil (LVRC) placement were reviewed retrospectively. Results There were 37 patients who received EBV therapy and 29 received LVRC therapy. There were no significant differences in the demographic and clinical characteristics between two groups before the treatment. There were seven deaths (10.6%) over the period of twelve months following the BLVR treatment. All deaths occurred in patients with at least one comorbid condition. Mortality was increased in the presence of comorbidities (14.3% vs 0%, respectively; p=0.099), and it was significantly increased in the presence of multiple comorbidities (18.5% vs 0%; p=0.059). The mortality rate was higher (37.5% vs 10.5%) in the LVRC comparing to the EBV treatment group in the presence of multiple comorbid conditions, albeit not reaching statistical significance (p=0.099). Conclusion The presence of more than one comorbidity in patients who underwent the LVRC treatment are associated with significant increase of mortality. For patients with severe emphysema who have more than one comorbidity, EBV is a better choice than LVRC.

The relationship between serum levels of surfactant protein D in COPD exacerbation severity and mortality

Background/aim: Chronic obstructive pulmonary disease (COPD) is a major cause of morbidity and mortality. In COPD patients, various inflammatory markers such as cytokines and acute phase proteins, which show systemic inflammation in the circulation, increase during exacerbations. In our study, we aimed to determine the relationship between serum SP-D levels and exacerbation severity, clinical course of the disease, and early mortality after discharge. Materials and methods: Fifty hospitalized patients with COPD acute exacerbation (46 male and 4 female) were recruited in this study. Thirty-three of the subjects (31 male and 2 female) were reevaluated after discharge. Venous blood samples were taken from all patients and followed up for exacerbation frequency, hospital admission, and mortality for 12 months. Results: Serum SP-D levels in the stable period of the patients were lower than exacerbation (P < 0.001). The median exacerbation period SP-D level of the patients admitted to emergency department in the first month was statistically significantly higher than that of the patients who were not admitted (P < 0.05) after discharge. There was a correlation between the rate of emergency admission and serum SP-D levels during the 12-month period after discharge (P = 0.04 (r = 0.29)). Conclusion: Our study showed that serum SP-D was found to be a useful biomarker in predicting emergency admission and predictor of the health status of COPD patients but did not predict early mortality after the exacerbation.

General features of patients with Pulmonary Langerhans Cell Histiocytosis followed in our instution.

INTRODUCTION: Pulmonary Langerhans Cell Histiocytosis (PLCH) is a rare disease affecting young smokers. It is more common between the ages of 20-40 and equals the male/female ratio. Lung biopsy is the most useful methods for diagnosis. The first treatment is to quit smoking. Corticosteroids or chemotherapeutic agents can be used in severe progressive cases despite of quiting smoking. The patients with PLCH followed in our clinic were assessed with general clinical features in the light of the literature. MATERIALS AND METHODS: We retrospectively evaluated patients with PLCH in our clinic between January 1999 and June 2017. RESULT: The female and male distribution of the 21 patients was 11/10. The average age was 35.04 ± 11.78 years. All patients were active smokers at the time of admission. The most common symptom was dyspnea. The most common finding in the pulmonary function tests was obstructive ventilatory defect. The DLCO value of the 70% patient in the carbonmonooxid diffusion test was below 80%. The most common pathologic findings detected in high-resolution chest tomography (HRCT) were cystic lesions involving bilateral upper and middle areas. There were 3 (14%) patients with pneumothorax at the time of admission and 6 (28.5%) patients with pneumothorax history before. The most common diagnostic method was open lung biopsy. All the patients quit cigarette after the diagnosis. There were 6 patients using steroid therapy, 1 patient receiving steroid and bosentan therapy, and 1 patient made pleurectomy due to recurrent pneumothorax. Lung transplantation was done to patient who received combined bosentan treatment with steroids. CONCLUSIONS: PLCH is a rare disease and should be considered in young, smokers with spontaneous pneumothorax and cystic lung disease in the differential diagnosis. As more diffusions are affected in patients, respiratory functions for follow-up should be evaluated with diffusion tests. It is essential to quit smoking in therapy.