Radiological and histological characteristics of patients with interstitial lung disease undergoing lung biopsy in a reference institution from eastern Colombia / Características radiológicas e histológicas de pacientes con enfermedad pulmonar intersticial llevados a biopsia pulmonar en una institución del oriente colombiano

Abstract Introduction: Interstitial lung disease (ILD) diagnosis requires a mukidisciplinary approach and, in some cases, lung biopsy. Objective: To describe the sociodemographic and clinical characteristics, as well as the radiological and histological findings, of patients with ILD who required lung biopsy after a mukidisciplinary board (pneumology, radiology, and pathology) of a reference center for respiratory diseases in Bucaramanga, Colombia, failed to reach the ILD diagnosis. Materials and methods: Cross-sectional study. The medical records of 56 patients treated at the Instituto Neumológico del Oriente who underwent lung biopsy between 2015 and 2019 were reviewed. Measures of central tendency and dispersion were calculated for demographic and clinical variables, respectively, to characterize them. A bivariate analysis was performed using Fisher's exact test to determine whether there were differences in the distribution of the sociodemographic and clinical variables according to the radiological patterns and the final histological diagnosis. Results: Participants' median age was 67 years (IQR: 59-72) and 55.35% were men. 43 patients had a radiological pattern inconsistent with usual interstitial pneumonia (UIP); 4 had a pattern consistent with possible UIP; and 9 had a pattern consistent with UIP. The most common histologic diagnoses were hypersensitivity pneumonitis (HP) (32.14%), nonspecific interstitial pneumonia (NSIP) (17.86%), and UIP (19.64%). Conclusion: In the study population, the primary reason for performing a lung biopsy was the presence of a radiologic pattern inconsistent with UIP, with HP being the predominant histopathological diagnosis. This is the first study to characterize patients with ILD who underwent lung biopsy in eastern Colombia, making a significant contribution to our understanding of the disease's epidemiology in the country.

Resumen Introducción. El diagnóstico de la enfermedad pulmonar intersticial (EPI) requiere un enfoque multidisciplinar y, en ocasiones, de una biopsia pulmonar. Objetivo. Describir las características sociodemográficas y clínicas, y los hallazgos radiológicos e histológicos de pacientes con EPI que requirieron biopsia pulmonar luego de no lograrse un diagnóstico de esta enfermedad por la junta médica multidisciplinar (neumología, radiología y patología) de un centro de referencia en enfermedades respiratorias de Bucaramanga, Colombia. Materiales y métodos. Estudio transversal. Se revisaron las historias clínicas de 56 pacientes atendidos en el Instituto Neumológico del Oriente y que fueron remitidos a biopsia pulmonar entre 2015 y 2019. Se analizaron variables demográficas y clínicas, calculando medidas de tendencia central y de dispersión para su respectiva caracterización. Se realizó un análisis bivariado mediante test exacto de Fisher para determinar si existían diferencias en la distribución de las variables sociodemográficas y clínicas de acuerdo con los patrones radiológicos y el diagnóstico histológico definitivo. Resultados. La mediana de edad fue 67 años (RIC: 59-72), 55.35% fueron hombres. 43 pacientes presentaron patrón radiológico inconsistente con neumonía intersticial usual (NIU); 4, patrón de posible NIU y, 9, patrón de NIU. Los diagnósticos histológicos más frecuentes fueron neumonitis por hipersensibilidad (NH) (32.14%), neumonía intersticial no específica (17.86%) y NIU (19.64%). Conclusión. La principal razón para realizar biopsia pulmonar en la población de estudio fue la presencia de un patrón radiológico inconsistente con NIU, siendo la NH el principal diagnóstico histopatológico. Este es el primer trabajo que caracteriza a pacientes con EPI del oriente colombiano llevados a biopsia pulmonar, lo que representa un importante aporte al conocimiento de la epidemiología de esta enfermedad en Colombia.

A Case of Pulmonary Infarction Caused by Diffuse Alveolar Damage Confirmed by Open-Lung Biopsy / 대한내과학회지

We report a case of a 63-year-old man with adult respiratory distress syndrome and pulmonary infarction. The patient presented with fever, dyspnea, pleuritic chest pain, and acute respiratory failure, and we applied mechanical ventilation and steroid therapy. Pulmonary infarction and diffuse alveolar damage were confirmed by open-lung biopsy. Diffuse alveolar damage activated the blood coagulation system, resulting in thrombosis in the pulmonary vasculature. After anticoagulation therapy, the patient improved rapidly. We report a rare pulmonary infarction caused by diffuse alveolar damage confirmed by open-lung biopsy.

A Case of Pulmonary Infarction Caused by Diffuse Alveolar Damage Confirmed by Open-Lung Biopsy / 대한내과학회지

We report a case of a 63-year-old man with adult respiratory distress syndrome and pulmonary infarction. The patient presented with fever, dyspnea, pleuritic chest pain, and acute respiratory failure, and we applied mechanical ventilation and steroid therapy. Pulmonary infarction and diffuse alveolar damage were confirmed by open-lung biopsy. Diffuse alveolar damage activated the blood coagulation system, resulting in thrombosis in the pulmonary vasculature. After anticoagulation therapy, the patient improved rapidly. We report a rare pulmonary infarction caused by diffuse alveolar damage confirmed by open-lung biopsy.

Pathological and ultrastructural analysis of surgical lung biopsies in patients with swine-origin influenza type A/H1N1 and acute respiratory failure

BACKGROUND: Cases of H1N1 and other pulmonary infections evolve to acute respiratory failure and death when co-infections or lung injury predominate over the immune response, thus requiring early diagnosis to improve treatment. OBJECTIVE: To perform a detailed histopathological analysis of the open lung biopsy specimens from five patients with ARDS with confirmed H1N1. METHODS: Lung specimens underwent microbiologic analysis, and examination by optical and electron microscopy. Immunophenotyping was used to characterize macrophages, natural killer, T and B cells, and expression of cytokines and iNOS. RESULTS: The pathological features observed were necrotizing bronchiolitis, diffuse alveolar damage, alveolar hemorrhage and abnormal immune response. Ultrastructural analysis showed viral-like particles in all cases. CONCLUSIONS: Viral-like particles can be successfully demonstrated in lung tissue by ultrastructural examination, without confirmation of the virus by RT-PCR on nasopharyngeal aspirates. Bronchioles and epithelium, rather than endothelium, are probably the primary target of infection, and diffuse alveolar damage the consequence of the effect of airways obliteration and dysfunction on innate immunity, suggesting that treatment should be focused on epithelial repair.

Clinical Spectrum and Lung Pathology in Children with Interstitial Lung Disease

PURPOSE: Interstitial lung disease(ILD) is a rare and poorly characterized disorder in children with poor prognosis. To understand the ILD in children, we reviewed our experience with 21 patients who were diagnosed interstitial lung disease during 9-year period at Asan Medical Center retrospectively. METHODS: Severity-of-illness score was measured by the Denver protocol. We evaluated underlying diseases, clinical manifestations, high resolution computed tomography findings, lung pathology and clinical responses after steroid therapy and prognosis. Fifteen patients were performed open lung biopsy, but six patients were diagnosed bronchiolitis obliterans by HRCT. RESULTS: The median ages at onset of ILD was 1 year 5 month old. Common clinical findings were tachypnea(90.0%), hypoxemia(90.0%). 14 patients among 15 patients were diagnosed specifically after open lung biopsy. Interstitial pneumonitis was 6 cases, including 3 nonspecific interstitial pneumonitis, 2 cases of desquamative interstitial pneumonitis and one usual interstitial pneumonitis. Other diagnosis included idiopathic pulmonary fibrosis, cytomegalovirus pneumonitis, diffuse aspiration bronchiolitis, pulmonary lymphangiomatosis, BOOP(bronchiolotis obliterans organizing pneumonia) and pulmonary histiocytosis. Six patients showed clinical and HRCT findings consistent with bronchilitis obliterans. Common radiologic findings were mosaic perfusion(12/21), bronchial wall thickening(9/21). 13 patients among 15 patients with methylprednisolone pulse therapy showed improvement of clinical symptoms. The severity-of-illness score was improved significantly after methylprednisolone pulse therapy. CONCLUSION: Pediatric ILD includes heterogeneous disorders. Open lung biopsy was helpful to make early diagnosis. Severity-of-illness score is a noninvasive and useful measure of disease progression or response to therapy. Methylprednisolone pulse therapy may be effective to treat ILD.

Diagnosis of Interstitial Lung Disease: Comparison of HRCT, Transbronchial Lung Biopsy and Open Lung Biopsy / 결핵

BACKGROUND: Open lung biopsy(OLB) has conventionally been regarded as the gold standard for the diagnosis in interstitial lung disease. With recent advances in diagnostic technique such as high resolution computed tomography(HRCT), and transbronchial lung biopsy(TBLB) which provide relatively accurate diagnosis of ILD, it is necessary to reevaluate the role of these methods in the diagnosis of ILD. METHODS: We carried out a retrospective analysis of nineteen patients who underwent OLB at Dankook University Hospital for the diagnosis of acute and chronic ILD, between May 1995 and June 1998. By reviewing the medical records, the demographic findings, underlying conditions, HRCT and TBLB findings, OLB diagnosis, therapy after OLB, and complication of OLB were evaluated. RESULTS: 1) Thirteen patients(68.4%) had chronic ILD(symptom duration over 2 weeks prior to OLB), and six patients(31.6%) had acute ILD(symptom duration less than 2 weeks). 2) Specific diagnosis were reached in 92%(12/13) of chronic ILD(5 Bronchiolitis obliterans organizing pneumonia(BOOP), 2 constrictive bronchiolitis, 3 Usual interstitial pneumonia, 1 hypersensitivity pneumonitis, 1 eosinophilic pneumonia), and in all patients of acute ILD(5 acute interstitial pneumonia, 1 pneumocystis carinii pneumonia). 3) HRCT were performed in all patients and a correct first choice diagnosis rate of HRCT was 42%(5/12) in chronic ILD. 4) In chronic ILD patients, 62%(8/13) received specific therapy(steroid therapy in 7 patients and moving in one patient), after OLB, but in acute ILD, all patients received specific therapy(steroid therapy in 5 patients and steroid and antibiotic therapy in one patient) after OLB. 5) The in-hospital mortality after OLB was 5.3%(1/19). CONCLUSION: OLB is an excellent diagnostic technique with relatively low complications in patients with ILD. Therefore OLB should be considered in patients with ILD when the specific diagnosis is important for the treatment, especially in patients with acute ILD.

A Case of Respiratory Bronchiolitis-Associated Interstitial Lung Disease / 대한내과학회지

Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD) is an inflammatory lung disorder associated with cigarette smoking. The clinical and radiographic findings of RB-ILD are nonspecific and most patients with RB-ILD are thought to have some form of idiopathic pulmonary fibrosis prior to lung biopsy. Open lung biopsy of patients with RB-ILD reveals inflammation of the respiratory bronchioles, filling of the bronchiolar lumens and surrounding alveoli with finely pigmented macrophages, associated interstitial inflammation. Alveolar septa, particularly around terminal and membranous bronchioles, are mildly fibrotic. We recently experienced a case of RB-ILD proven by open lung biopsy.

A Case of Primary Pulmonary Low-grade B-cell Lymphoma of Bronchus-associated lymphoid tissue with Bilateral Consolidation and Indolent Clinical Course / 결핵

Primary pulmonary lymphoma (PPL) is an uncommon tumor, which constitutes 3-4% of all exuanodal lymphomas and 0.3-0.5% of all primary pulmonary malignant tumors. Low-grade B-cell lymphomas of bronchus-associated lymphoid tissue (BALT) accounted for the majority of PPL. This BALT lymphomas are frequently asymptomatic and have an excellent prognosis and an indolent clinical course by contrast with T-cell type. Therefore, determination of the B- or T-immunophenotype of the tumor cells is known 13 be very important from a clinical aspect Recent advances in immunohistochemical techniques, cytogenetics, and molecular biology have allowed better definition of type, maturation and clonality of lymphoma cells and have made it possible to better understand the PPL. We experienced an asymptomatic 43-year-old man who was evaluated for infiltrates on both sides discovered incidentally after a routine chest roentgenogram. He was eventually diagnosed as low-grade B-cell lymphoma of BALT by immunohistochemical staining from specimens obtained by open lung biopsy. He was treated with combination chemotherapy. At follow up 12 months following initial diagnosis he remains in stable. We report this case, who showed a relatively favorable prognosis and indolent clinical course compatible with low-grade B-cell lymphoma.

A Case of Necrotizing Sarcoid Granulomatosis / 대한내과학회지

Necrotizing sarcoid granulomatosis was first described by Liebow, who included it in the category of pulmonary angiitis and granulomatosis. Wegener's granulomatosis and limited Wegener's granulomatosis are also included in this category. Necrotizing sarcoid granulomatosis is characterized by histologically sarcoid-like granulomata, prominent and granulomatous vasculitis, and varying degrees of necrosis, radiographically pulmonary nodules but no enlarged hilar lymph nodes, clinically benign course. Whether this entity is a variant of necrotizig vasculitis with sarcoid reaction or sarcoidosis with prominent vascultic features and necrosis is not yet, clear, but many authors suggest the relationship with sarcoidosis from the observation of the similarity of histologic features and clinical course between the two entities. Patients are asymptomatic in about one fourth. Even symptomatic patients have vague symptoms such as cough, chest pain. Extrapulmonary involvement is rare. The prognosis is good even without therapy or with steroid alone, if necessary. We report a case that was diagnosed by open lung biopsy as necrotizing sarcoid granulomatosis, and it is assumed to be the first report of this entity in Korea.

A Case of Spontaneous Resolution of Idiopathic Mediastinal Fibrosis / 결핵

Mediastinal fibrosis is pathologically characterized by chronic inflammation and fibrosis of mediastinal soft tissue. Mediastinal fibrosis is local expression of a family of systemic fibrosing syndroms. This can result in compression of adjacent mediastinal structures. Idiopathic fibrosing syndromes include retroperitoneal fibrosis, sclerosing cholangitis of the orbit and fibrosis of the thyroid gland(Riedel's struma). The cause of these disorders is obscure, in some instance there is an underlying malignancy, infection, history of drug ingestion, or trauma with retoperitoneal bleeding. Treatment of mediastinal fibrosis depends on structures involved by the fibrotic process. The disease is self limited in most case or improved by steroids uses. We experienced a case of idopathic solerosing mediastinitis with orbital fibrous dysplasia of unknown cause, which was confirmed by open lung biopsy, so reported it with a review of literature.

A Case of Pulmonary Siderosis / 대한내과학회지

Pulmonary siderosis is one kind of pneumoconiosis caused by the long term inhalation of iron dust. Iron is deposited in the lungs, usually in the form of iron oxides. Iron oxides are relatively inert particles with a minimal fibrotic response. It occurs in a number of occupations including welding, steel rolling and grinding, casting, iron ore mining and oxyacetylene cutters. We have experienced the first case of pulmonary siderosis in Korea. A 42-year-old woman who had engaged in a metalware manufacturing factory as a manager for 7 years was admitted because of dyspnea on exertion. A pulmonary function test disclosed a mildly obstructive ventilatory defect. Open lung biosy revealed deposition of iron-laden macrophages in most alveolar spaces by which confirmed the diagnosis of pulmonary siderosis. We report a case of pulmonary siderosis with a review of the literature.

The Role of Open Lung Biopsy in Diagnosis and Treatment of Diffuse Interstitial Lung Disease in High-resolution Computed Tomography Era / 결핵

Objective: Diffuse interstitial lung disease (DILD) is a group of diverse diseases that share common clinical, radiologic, and pulomonary function features. Open lung biopsy (OLB) has been regarded as gold standard in differential diagnosis of DILD. However open lung biopsy is a invasive diagnostic tool not free of its own risk or complications. These days, high-resolution computed tomography (HRCT) has become an important diagnostic tool in DILD through its precise image analysis. In many instances, HRCT could provide specific diagnosis or, at least, provide information on the disease activity of DILD. The authors re-evaluate the role of open lung biopsy in this "HRCT era" by investigating the additional diagnostic gain and impacts on the treatment plan in patients who have undergone high-resoluticm CT. Method: Diagnoses obtained by high-resolution CT and open lung biopsy were compared and changes of treatment plans were evaluated retrospectively in 30 patients who had undergone open lung biopsy for the purpose of diagnosis of diffuse interstitial lung disease from March 1988 to June 1994. Results: High-resolution CT suggeted specific diagnoses in 22 out of 28 patients (78.6%) and the diagnoses were confirmed to be correct by open lung biopy in 20 of those 22 cases (91%). Open lung biopsy could not give specific diagnosis in 5 out of 30 cases (16.7%). In 5 out of 6 cases (83.3%) in whom high reolution CT was not able to suggest specific diagnosis, open lung biopsy gave specific diagnoses. Treatment plan was altered by the result of open lung biopsy in only 2 cases. Conclusion: The above findings suggest that in "HRCT era", when HRCT could suggest specific diagnosis, the need for open lung biopsy should be re-evaluated.

The Role of Open Lung Biopsy in Diagnosis and Treatment of Diffuse Interstitial Lung Disease in High-resolution Computed Tomography Era / 결핵

Objective: Diffuse interstitial lung disease (DILD) is a group of diverse diseases that share common clinical, radiologic, and pulomonary function features. Open lung biopsy (OLB) has been regarded as gold standard in differential diagnosis of DILD. However open lung biopsy is a invasive diagnostic tool not free of its own risk or complications. These days, high-resolution computed tomography (HRCT) has become an important diagnostic tool in DILD through its precise image analysis. In many instances, HRCT could provide specific diagnosis or, at least, provide information on the disease activity of DILD. The authors re-evaluate the role of open lung biopsy in this "HRCT era" by investigating the additional diagnostic gain and impacts on the treatment plan in patients who have undergone high-resoluticm CT. Method: Diagnoses obtained by high-resolution CT and open lung biopsy were compared and changes of treatment plans were evaluated retrospectively in 30 patients who had undergone open lung biopsy for the purpose of diagnosis of diffuse interstitial lung disease from March 1988 to June 1994. Results: High-resolution CT suggeted specific diagnoses in 22 out of 28 patients (78.6%) and the diagnoses were confirmed to be correct by open lung biopy in 20 of those 22 cases (91%). Open lung biopsy could not give specific diagnosis in 5 out of 30 cases (16.7%). In 5 out of 6 cases (83.3%) in whom high reolution CT was not able to suggest specific diagnosis, open lung biopsy gave specific diagnoses. Treatment plan was altered by the result of open lung biopsy in only 2 cases. Conclusion: The above findings suggest that in "HRCT era", when HRCT could suggest specific diagnosis, the need for open lung biopsy should be re-evaluated.

Open lung biopsy in patients with pulmonary infiltrations.

A retrospective study is composed of 23 patients with pulmonary infiltrations whose hospital records were available who underwent open lung biopsy at Siriraj Hospital from January, 1982, to December, 1986. The patients were separated into two broad groups. The first, there were those patients who were in clinically stable condition. The second group identified patients who were systemically ill and whose course was rapidly deteriorating. Time delayed in diagnosis was critical in these patients whose urgent and correct treatment was necessary. Open lung biopsy revealed correct diagnosis in all patients. The prospective diagnosis was changed in 60.87 percent of all patients and the biopsy results altered therapy in 56.52 percent. Hence open lung biopsy appears to be a valuable procedure with low morbidity and mortality in patients who have otherwise undiagnosable pulmonary infiltrations.