• Tuberculosis and Respiratory Diseases
• /
• 제87권2호
• /
• pp.123-133
• /
• 2024

Interstitial lung diseases (ILDs) are a diverse collection of lung disorders sharing similar features, such as inflammation and fibrosis. The diagnosis and management of ILD require a multidisciplinary approach using clinical, radiological, and pathological evaluation. Progressive pulmonary fibrosis (PPF) is a distinct form of progressive and fibrotic disease, occurring in ILD cases other than in idiopathic pulmonary fibrosis (IPF). It is defined based on clinical symptoms, lung function, and chest imaging, regardless of the underlying condition. The progression to PPF must be monitored through a combination of pulmonary function tests (forced vital capacity [FVC] and diffusing capacity of the lung for carbon monoxide), an assessment of symptoms, and computed tomography scans, with regular follow-up. Although the precise mechanisms of PPF remain unclear, there is evidence of shared pathogenetic mechanisms with IPF, contributing to similar disease behavior and worse prognosis compared to non-PPF ILD. Pharmacological treatment of PPF includes immunomodulatory agents to reduce inflammation and the use of antifibrotics to target progressive fibrosis. Nintedanib, a known antifibrotic agent, was found to be effective in slowing IPF progression and reducing the annual rate of decline in FVC among patients with PPF compared to placebos. Nonpharmacological treatment, including pulmonary rehabilitation, supplemental oxygen therapy, and vaccination, also play important roles in the management of PPF, leading to comprehensive care for patients with ILD. Although there is currently no cure for PPF, there are treatments that can help slow the progression of the disease and improve quality of life.

• Journal of Chest Surgery
• /
• 제51권1호
• /
• pp.22-28
• /
• 2018

Background: Lung transplantation is a life-saving procedure in patients with end-stage lung disease, and is increasingly performed in Korea. Methods: We retrospectively evaluated the outcomes of patients who received a lung transplant at Asan Medical Center between January 2008 and December 2016. Thirteen of 54 patients experienced multiorgan transplantation; the remaining 41 who received only lung grafts were included. Results: The mean age of the lung transplant recipients was 44.6 years; 27 were men and 14 were women. The most frequent reasons were idiopathic interstitial pneumonia (21 of 41 patients, 51.2%), interstitial lung disease (9 of 41, 22.0%), and bronchiolitis obliterans after bone marrow transplantation (7 of 41, 17.1%). The median waiting time was 47 days, and many patients received preoperative intensive care (27 of 41, 65.9%), ventilator support (26 of 41, 63.4%), or extracorporeal life support (19 of 41, 46.3%). All 41 patients received bilateral lung grafts. Ten deaths occurred (24.3%), including 5 cases of early mortality (12.2%) and 5 cases of late mortality (12.2%). The 1-, 3-, and 5-year survival rates were 78.9%, 74.2%, and 69.3%, respectively. Conclusion: Despite a high percentage of patients who required preoperative intensive care, the transplantation outcomes were acceptable.

• Tuberculosis and Respiratory Diseases
• /
• 제69권5호
• /
• pp.375-380
• /
• 2010

Lymphoid interstitial pneumonia (LIP) is a rare benign lymphoproliferative interstitial lung disease. LIP has been associated with autoimmune disorders, HIV, viral infections, and so on. Once underlying systemic diseases have been excluded, a diagnosis of idiopathic LIP can be made. Although 6 cases of pathologically confirmed LIP have occurred in Korea, thus far none has been associated with primary Sjogren's syndrome. A 44-year-old man was admitted to hospital due to a dry cough and dypsnea on exertion that had been ongoing for 2 months. A chest radiography showed multiple and variable-sized cystic lesions, on both lungs and both interstitial infiltration and consolidation in both lower lung fields. Tests for autoantibody showed positive results of anti-nuclear antibody and anti-Ro/La antibody. The patient underwent a video assisted thoracoscopic surgery biopsy and pathologically confirmed LIP. We report the first known case of LIP-associated with primary Sjogren's syndrome in Korea.

• Journal of Yeungnam Medical Science
• /
• 제39권2호
• /
• pp.153-160
• /
• 2022

Multicentric Castleman disease (MCD) is an uncommon systemic lymphoproliferative disorder that may cause multiple organ damage. Castleman disease-associated diffuse parenchymal lung disease (DPLD) has not been well studied. A 32-year-old man was referred to our hospital for progressive generalized weakness, light-headedness, and dyspnea on exertion for more than one year. Laboratory evaluations showed profound anemia, an elevated erythrocyte sedimentation rate, and an increased C-reactive protein level with polyclonal hypergammaglobulinemia. Chest radiography, computed tomography (CT), and positron emission tomography-CT scan demonstrated diffuse lung infiltration with multiple cystic lesions and multiple lymphadenopathy. In addition to these clinical laboratory findings, bone marrow, lung, and lymph node biopsies confirmed the diagnosis of idiopathic MCD (iMCD). Siltuximab, an interleukin-6 inhibitor, and glucocorticoid therapy were initiated. The patient has been tolerating the treatment well and had no disease progression or any complications in 4 years. Herein, we report this case of human herpesvirus-8-negative iMCD-associated DPLD accompanied by multiple cystic lesions, multiple lymphadenopathy, and polyclonal hypergammaglobulinemia with elevated immunoglobulin G (IgG) and IgG4 levels. We recommend a close evaluation of MCD in cases of DPLD with hypergammaglobulinemia.

• Tuberculosis and Respiratory Diseases
• /
• 제43권5호
• /
• pp.746-754
• /
• 1996

목적: 미만성 간질성 폐질환은 공통의 임상 양상, 방사선 소견, 폐기능 장애 소견을 갖는, 무수히 많은 원인 질환들로 구성되어 있어 개흉폐생검 등의 조직학적 진단이 표준으로 받아들여지고 있지만 관혈적 진단 방법이라는 단점이 있다. 고해상도 전산화단층촬영은 해상력을 높이고 음영의 중첩 효과를 최소화함으로써 폐간질의 미세한 변화까지 관찰할 수 있게 하겨 미만성 간질성 폐질환의 진단에 매우 유용한 진단 방법이다. 저자들은 이런 고해상도 전산화단층촬영을 한 환자에서 개흉폐생검을 시행하여 진단 및 치료에 어느 정도의 추가 이익을 얻을 수 있었는지 알아보고자 하였다. 방법: 1988년 3월부터 1994년 7월까지 미만성 간질성 폐질환의 진단적 목적으로 개흉폐생검을 시행한 30예의 환자들을 대상으로 후향적으로 고해상도 전산화단층촬영의 진단과 개흉폐생검외 진단 결과를 비교하고 각 환자들의 치료 방침을 조사하였다. 결과: 고해상도 전산화단층촬영은 28예에서 시행되었는데 이 중 22예(78.6%)에서 특이적 진단이 가능하였고 22예 중 20예에서는 개흉폐생검에 의해 같은 진단으로 확진되었다. 나머지 2예 중 1예는 개흉폐생검 결과가 비특이적이었다. 개흉폐생검은 전체 30예 중 25예(83.3%)에서 특이적 진단을 하였다. 또한 고해상도 전산화단층촬영으로 특이 진단이 불가능하였다. 6예 중 5 예(83.3%)에서 특이적 진단을 하였다. 전체 30예 중 단지 2예에서만 치료 방침의 결정적인 변화가 있었다. 결론: 미만성 간질성 폐질환 환자에서 고해상도 전산화단층촬영이 특이적 진단을 강력히 시사하는 경우에 개흉폐생검의 펼요성 여부에 대해서는 재검토가 필요할 것으로 생각된다.

• Tuberculosis and Respiratory Diseases
• /
• 제87권2호
• /
• pp.185-193
• /
• 2024

Background: The mechanisms leading to lung fibrosis are still under investigation. This study aimed to demonstrate whether antacids could prevent the development of interstitial lung disease (ILD). Methods: This population-based longitudinal cohort study was conducted between January 2006 and December 2010 in South Korea. Eligible subjects were ≥40 years of age, exposed to proton pump inhibitors (PPI)±histamine-2 receptor antagonists (H-2 blockers) or H-2 blockers only, and had no history of ILD between 2004 and 2005. Exposure to antacids was defined as the administration of either PPI or H-2 receptor antagonists for >14 days, whereas underexposure was defined as antacid treatment administered for less than 14 days. Newly developed ILDs, including idiopathic pulmonary fibrosis (IPF), were counted during the 5-year observation period. The association between antacid exposure and ILD development was evaluated using adjusted Cox regression models with variables, such as age, sex, smoking history, and comorbidities. Results: The incidence rates of ILD with/without antacid use were 43.2 and 33.8/100,000 person-years, respectively and those of IPF were 14.9 and 22.9/100,000 person-years, respectively. In multivariable analysis, exposure to antacid before the diagnosis of ILD was independently associated with a reduced development of ILD (hazard ratio [HR], 0.57; 95% confidence interval [CI], 0.45 to 0.71; p<0.001), while antacid exposure was not associated with development of IPF (HR, 0.88; 95% CI, 0.72 to 1.09; p=0.06). Conclusion: Antacid exposure may be independently associated with a decreased risk of ILD development.

• Tuberculosis and Respiratory Diseases
• /
• 제74권1호
• /
• pp.23-27
• /
• 2013

Multicentric Castleman's disease (CD) is a rare atypical lymphoproliferative disorder, which is characterized by various systemic manifestations. Some patients with multicentric CD may have concomitant lung parenchymal lesions, for which lymphoid interstitial pneumonia (LIP) is known to be the most common pathologic finding. Follicular bronchiolitis and LIP are considered to be on the same spectrum of the disease. We describe a case of multicentric CD with pulmonary involvement, which was pathologically proven as follicular bronchiolitis.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• 제18권4호
• /
• pp.286-291
• /
• 2015

Ulcerative colitis (UC) is a chronic idiopathic inflammatory bowel disease. Mesalizine for the first-line therapy of UC has adverse effects include pancreatitis, pneumonia and pericarditis. UC complicated by two coexisting conditions, however, is very rare. Moreover, drug-related pulmonary toxicity is particularly rare. An 11-year-old male patient was hospitalized for recurring upper abdominal pain after meals with vomiting, hematochezia and exertional dyspnea developing at 2 weeks of mesalizine therapy for UC. The serum level of lipase was elevated. Chest X-ray and thorax computed tomography showed interstitial pneumonitis. Mesalizine was discontinued and steroid therapy was initiated. Five days after admission, symptoms were resolved and mesalizine was resumed after a drop in amylase and lipase level. Symptoms returned the following day, however, accompanied by increased the serum levels of amylase and lipase. Mesalizine was discontinued again and recurring symptoms rapidly improved.

• Tuberculosis and Respiratory Diseases
• /
• 제77권3호
• /
• pp.145-148
• /
• 2014

Valproic acid is one of the most common antiepileptic drugs used for the treatment of several seizure disorders. A 20-year-old man presented with a sudden decline of consciousness. He had a neurosurgery operation for intracranial and intraventricular hemorrhage. Following surgery, antiepileptic medication was administered to the patient in order to control his seizure events. On valproic acid treatment, he began to complain of fever and dyspnea. His symptoms persisted despite receiving empirical antibiotic treatment. All diagnostic tests for infectious causes were negative. A high-resolution computed tomography scan of the chest revealed predominantly dependent consolidation and ground-glass opacities in both lower lobes. The primary differential was drug associated with interstitial lung disease. Therefore, we discontinued valproic acid treatment and began methylprednisolone treatment. His symptoms and radiologic findings had significantly improved after receiving steroid therapy. We propose that clinicians should be made aware of the potential for valproic acid to induce lung injury.

• 대한방사선기술학회지:방사선기술과학
• /
• 제44권2호
• /
• pp.91-100
• /
• 2021

High-Resolution thoracic CT (HRCT) is a scanning protocol in which thin slice thickness and sharpness algorithm are utilized to enhance image resolution for diagnosis and assessment of interstitial lung disease (ILD). This examination is sometimes performed in both supine and prone position to improve sensitivity to early changes of these conditions. Anatomical structures (the size of lung field and heart and descending aorta) of 150 patients who underwent HRCT were retrospectively compared. HRCT had been conducted in two positions (supine and prone). Data were divided into five groups according to patient body weights (from 40 to more than 80kg, 10kg intervals, 60 patients/each group). Quantitative analysis was utilized in Image J program. In the supine position defined as the control group, the average values of lung fields and heart size and aorta were compared with the prone position defined as the experimental group. The size of the lungs was found to be higher in the supine position, and it was confirmed that there was a statistically significant difference in patients over 70 kg (p<0.05). In addition, both sizes of the heart and descending aorta were larger in prone position, but in the case of the heart, there was no correlation with the presence or absence of ILD disease (p>0.05). Also, the area of prone in the descending aorta was higher than supine position, but there was no statistically significant difference between supine and prone position (p>0.05). In conclusion, when the severity of ILD disease was severe, there was no statistically significant difference in the area difference between supine and prone position, so it is considered that it will be helpful in diagnostic decision.