Usual interstitial pneumonia end-stage features from explants with radiologic and pathological correlations.

Idiopathic pulmonary fibrosis (IPF) is the most frequent and severe idiopathic interstitial pneumonia, with typical high-resolution computed tomography (HRCT) features and histologic pattern of usual interstitial pneumonia (UIP); its main differential diagnosis is fibrotic nonspecific interstitial pneumonia (F-NSIP). Usual interstitial pneumonia was mainly described from lung biopsies, and little is known on explants. Twenty-two UIP/IPF explants were analyzed histologically and compared with previous open lung biopsies (OLBs; n = 11) and HRCT (n = 19), when available. Temporospatial heterogeneity and subpleural and paraseptal fibrosis were similarly found in UIP/IPF explants and OLB (91%-95%). Fibroblastic foci were found in 82% of OLBs and 100% of explants, with a higher mean score in explants (P = .023). Honeycombing was present in 64% of OLBs and 95% of explants, with a higher mean score in explants (P = .005). Almost 60% of UIP/IPF explants showed NSIP areas and 41% peribronchiolar fibrosis; inflammation, bronchiolar metaplasia, and vascular changes were more frequent in UIP/IPF explants; and Desquamative Interstitial Pneumonia (DIP)-like areas were not common (18%-27%). Numerous large airspace enlargements with fibrosis were frequent in UIP/IPF explants (59%). On HRCT, honeycombing was observed in 95% of the cases and ground-glass opacities in 53%, correlating with NSIP areas or acute exacerbation at histology. Six patients had combined IPF and emphysema. Lesions were more severe in UIP/IPF explants, reflecting the worsening of the disease. Usual interstitial pneumonia/IPF explants more frequently presented with confounding lesions such as NSIP areas, peribronchiolar fibrosis, and airspace enlargements with fibrosis sometimes associated with emphysema.

Safety, utility and clinical efficacy of cryobiopsy of lung in paediatric population-A single centre experience.

Background: Surgical lung biopsy (SLB) or video-assisted thoracic surgery (VATS) has been the traditional gold standard modality for diagnosing paediatric interstitial lung diseases. Cryobiopsy of the lung has recently been shown to be a novel technique with very good sensitivity and specificity in the diagnosis of various interstitial lung disorders in adults. Although there are a few case reports of the same in children, pediatric cryo lung biopsies are rarely performed due to the lack of the necessary equipment and the lack of expertise. Methods: A retrospective single-centre study was conducted with twelve consecutive children with diffuse parenchymal lung disease diagnosed both clinically and on high-resolution computed tomography (HRCT) of the chest which were included in the study between October 2020 and September 2022 to measure the diagnostic yield and safety of the procedure. The site from where cryobiopsy was to be done was chosen after a multidisciplinary meeting with the paediatric radiologist. Results: Twelve children (eight males and four females) were included in the study who underwent a cryobiopsy in the duration of two years. The mean age of the cases involved was 8 years and 3 months. With the youngest and oldest being 12 days and 15 years, respectively, all children underwent cryobiopsy as mentioned above. Diagnostic yield was achieved in 92% of cases. Conclusion: Cryobiopsy is a valuable diagnostic tool in childhood interstitial lung diseases, which offers a less invasive option for obtaining lung tissue samples with a better yield which can aid in accurate diagnosis, a good safety profile and a shorter hospital stay. Our study emphasizes that in trained centres, TBCB is a safe, effective and less invasive way to obtain tissue diagnosis in children with ChILD.

Outcome and Post-Surgical Lung Biopsy Change in Management of ARDS: A Proportional Prevalence Meta-Analysis.

BACKGROUND: Limited epidemiological data are available on changes in management, benefits, complications, and outcomes after open lung biopsy in patients with ARDS. METHODS: We performed a literature search of PubMed, Ovid, and Cochrane databases for articles from the inception of each database till November 2020 that provided outcomes of lung biopsy in ARDS patients. The primary outcome was the proportion of patients that had a change in management with alteration of treatment plan, after lung biopsy. Secondary outcomes included pathological diagnoses and complications related to the lung biopsy. Pooled proportions with a 95% confidence interval (CI) were calculated for the prevalence of outcomes. RESULTS: After analysis of 22 articles from 1994 to 2018, a total of 851 ARDS patients (mean age 59.28 ± 7.41, males 56.4%) that were admitted to the ICU who underwent surgical lung biopsy for ARDS were included. Biopsy changed the management in 539 patients (pooled proportion 75%: 95% CI 64-84%). There were 394 deaths (pooled proportion 49%: 95% CI 41-58%). The most common pathologic diagnosis was diffuse alveolar damage that occurred in 30% (95% CI 19-41%), followed by interstitial lung disease in 10% (95% CI 3-19%), and viral infection in 9% (95% CI 4-16%). Complications occurred among 201 patients (pooled proportion 24%, 95% CI 17-31%). The most common type of complication was persistent air-leak among 115 patients (pooled estimate 13%, 95% CI 9-17%). CONCLUSION: Despite the high mortality risk associated with ARDS, lung biopsy changed management in about 3/4 of the patients. However, 1/4 of the patients had a complication due to lung biopsy. The risks from the procedure should be carefully weighed before proceeding with lung biopsy.

Relationship between Mechanical Ventilation and Histological Fibrosis in Patients with Acute Respiratory Distress Syndrome Undergoing Open Lung Biopsy.

BACKGROUND: Mechanical ventilation brings the risk of ventilator-induced lung injury, which can lead to pulmonary fibrosis and prolonged mechanical ventilation. METHODS: A retrospective analysis of patients with acute respiratory distress syndrome (ARDS) who received open lung biopsy between March 2006 and December 2019. RESULTS: A total of 68 ARDS patients receiving open lung biopsy with diffuse alveolar damage (DAD; the hallmark pathology of ARDS) were analyzed and stratified into non-fibrosis (n = 56) and fibrosis groups (n = 12). The duration of ventilator usage and time spent in the intensive care unit and hospital stay were all significantly higher in the fibrosis group. Hospital mortality was higher in the fibrosis than in the non-fibrosis group (67% vs. 57%, p = 0.748). A multivariable logistic regression model demonstrated that mechanical power at ARDS diagnosis and ARDS duration before biopsy were independently associated with histological fibrosis at open lung biopsy (odds ratio 1.493 (95% CI 1.014-2.200), p = 0.042; odds ratio 1.160 (95% CI 1.052-1.278), p = 0.003, respectively). CONCLUSIONS: Our findings indicate that prompt action aimed at staving off injurious mechanical stretching of lung parenchyma and subsequent progression to fibrosis may have a positive effect on clinical outcomes.

A comparison of computed tomography with magnetic resonance imaging for the diagnosis of thoracic extramedullary hemopoiesis in patients with leukemia: A non-inferiority retrospective diagnostic study.

At present, MRI is the primary choice of examination for the diagnosis of thoracic extramedullary hemopoiesis. When thoracic extramedullary hemopoiesis presents as posterior mediastinum masses in specific clinical contexts, the diagnosis is not challenging. Other radiological presentations may be more difficult for diagnosis and require biopsy. Needle biopsy is typically preferred for the diagnosis of extramedullary hemopoiesis however, the high vascularization of tissues is one of the complications of this method thus, it is avoided. The aim of the present study was to compare the diagnostic parameters of CT with MRI for the diagnosis of thoracic extramedullary hemopoiesis in patients with leukemia, with an open lung biopsy as a reference standard. Chest CT, chest MRI and open lung biopsy data from a total of 912 patients with leukemia with a sign(s) and symptoms of suspected paravertebral and/or pulmonary extramedullary hemopoiesis were reviewed. In the present study, thoracic extramedullary hemopoiesis was defined as diffusivity of both lung fields being increased compared with the blood pool and no other abnormal focal of lungs being increased compared with the blood pool. The beneficial score was calculated for CT and MRI and plotted for the decision making of irradiation. With respect to open lung biopsy, MRI had a higher sensitivity compared with CT (0.865 vs. 0.809; P<0.0001; q=1691) however, CT had a higher accuracy compared with MRI (0.833 vs. 0.733; P<0.0001; q=3020). The low rate of overdiagnosis was observed for both methods for the detection of thoracic extramedullary hemopoiesis however, the working area for detecting thoracic extramedullary hemopoiesis at least once in images was higher for MRI compared with CT. CT and MRI both have diagnostic importance in the detection of thoracic extramedullary hemopoiesis in patients with leukemia however, chest MRI misdiagnoses the condition while CT can confirm it (level of evidence, 3).

Secondary pulmonary alveolar proteinosis treated by lung transplant: A case report.

BACKGROUND: Pulmonary alveolar proteinosis (PAP) is a pulmonary disease characterized by disruption of surfactant homeostasis resulting in its accumulation in the alveoli. PAP is classically classified into three categories (Table 1): 1/primary (or autoimmune) with antibodies targeting the GM-CSF pathway, 2/secondary to another disease, typically a hematologic malignancy, and 3/genetic. CASE-REPORT: A 30 year-old woman received an allogenic hematopoietic stem cell transplantation (HSCT) after treatment for acute myeloid leukemia (AML). Within the first 6 months post HSCT, she developed an ocular, oral, digestive and hepatic graft-versus-host disease associated with a mixed ventilatory defect with a very severe obstructive syndrome and a severe CO diffusion impairment. High resolution computed tomography showed a classical "crazy paving" pattern. Aspect and differential cell count of BAL were normal. All microbiological samples remained culture negative. Histo-pathological analysis of transbronchial biopsies was unremarkable. Because of the severity of the respiratory insufficiency, open-lung biopsy (OBL) could not be performed. Despite multiple immunosuppressive therapies, lung function deteriorated rapidly; the patient also developed an excavated fungal lesion unresponsive to treatment. She underwent a bilateral lung transplant 48 months after HSCT. Histo-pathological analysis of explanted lungs showed obliterative bronchiolitis (OB), diffuse PAP and invasive cavitary pulmonary aspergillosis. CONCLUSIONS: This case illustrates the simultaneous occurrence of OB, PAP and a fungal infection in a 30-year old female patient who underwent HSCT for acute myeloid leukemia (AML). To our knowledge this is the only documented case of PAP associated with OB treated by lung transplantation.

Chronic, Silent Microaspiration Masquerading as Interstitial Lung Disease.

Chronic, silent microaspiration is a common but underrecognized pathologic process in pulmonary medicine. The clinical presentation is variable and diagnosis can be challenging. We present the case of a 55-year-old woman with known emphysema, who was referred to us for progressive respiratory failure that was unresponsive to therapy. The patient had 9 hospital admissions in the preceding 5 months and was treated with multiple courses of antibiotics and systemic steroid therapy for a diagnosis of cryptogenic organizing pneumonia. The steroid therapy was complicated by 51 pounds of weight gain. She had conversational as well as profound exertional shortness of breath. Physical examination revealed a woman in moderate distress and bilateral diffuse wheezing and rhonchi. Computed tomography of the chest revealed areas of bronchocentric consolidation and bronchial wall thickening in the bilateral lower lobes. She underwent surgical lung biopsy and the histopathology was consistent with chronic aspiration pneumonia.

Impact and safety of open lung biopsy in patients with acute respiratory distress syndrome (ARDS).

INTRODUCTION: Acute respiratory distress syndrome (ARDS) is an inflammatory lung disorder, and its pathological hallmark is diffuse alveolar damage (DAD). Given that open lung biopsy (OLB) can sometimes result in severe side effects, it is rarely performed in patients with ARDS. AIM: The aims of this study were to describe: (a) the rate of treatment change associated with the histological result; and (b) the incidence of side effects induced by OLB. DESIGN AND PATIENTS: A retrospective, single-center, descriptive observational study was carried out in Hospital Santa Clara (Bogotá, Colombia) from February 2007 to January 2014. INCLUSION CRITERIA: Critically ill patients over 18 years of age, undergoing invasive mechanical ventilation, diagnosed with ARDS of unknown etiology, and with OLB performed at the bedside. ARDS was diagnosed according to the Berlin definition. DAD was defined by the presence of a hyaline membrane plus at least one of the following: intra-alveolar edema, alveolar type I cell necrosis, alveolar type II cell (cuboidal cells) proliferation progressively covering the denuded alveolar-capillary membrane, interstitial proliferation of fibroblasts and myofibroblasts, or organizing interstitial fibrosis. The rate of treatment change (RTC) was established according to whether the OLB pathology report resulted in: a) the prescription or discontinuation of an antimicrobial; b) the indication of new procedures; c) medical interconsultation; or d) limitation of therapeutic effort. Patients were followed-up until death or hospital discharge. This study was approved by the Ethics Committee. RESULTS: A total of 32 OLBs were performed during the study period; 17 were ruled out as they did not involve ARDS, and 15 were considered for further analysis. A histological diagnosis was reached in 14 of the 15 patients (12 DAD, one case of bronchiolitis obliterans organizing pneumonia and one case of Wegener's granulomatosis with alveolar hemorrhage). The RTC rate was 0.73. The most frequent intervention was discontinuation of antimicrobial or corticosteroid treatment. No deaths but four side effects (3 airway leaks and one hemothorax) were associated with the OLB procedure. All were resolved before ICU discharge. CONCLUSION: The information provided by OLB performed at the bedside in ARDS patients of unknown etiology could be relevant, as it may optimize treatment. The risk associated with OLB seems to be acceptable.

Risk and relevance of open lung biopsy in pediatric ECMO patients: the Dutch experience.

BACKGROUND: Open lung biopsy can help differentiate between reversible and irreversible lung disease and may guide therapy. To assess the risk-benefit ratio of this procedure in pediatric extracorporeal membrane oxygenation (ECMO) patients, we reviewed data of all patients who underwent an open lung biopsy during ECMO in one of the two pediatric ECMO centers in a nationwide study in the Netherlands. RESULTS: In nineteen neonatal and six pediatric patients (0-15.5years), twenty-five open lung biopsies were performed during the study period. In 13 patients (52%), a classifying diagnosis of underlying lung disease could be made. In another nine patients (36%), specific pathological abnormalities were described. In three patients (12%), only nonspecific abnormalities were described. The histological results led to withdrawal of ECMO treatment in 6 neonates with alveolar capillary dysplasia/misalignment of pulmonary veins (24%) and in another 6 patients, corticosteroids were started (24%). All patients survived the biopsy procedure. Hemorrhagic complications were rare. CONCLUSION: An open lung biopsy during an ECMO run in neonates and children is a safe procedure with a minimum risk for blood loss and biopsy-related death. It can be very useful in diagnosing the underlying pathology and can guide cessation of ECMO treatment and thereby avoid continuation of futile treatment, especially in neonatal patients. LEVEL OF EVIDENCE: III. TYPE OF STUDY: Diagnostic study.

The diagnostic yield and complications of open lung biopsies in kidney transplant patients with pulmonary disease.

BACKGROUND: The purpose of this study was to assess the efficacy of open lung biopsy (OLB) in determining the specific diagnosis and the related complications in patients with undiagnosed diffuse pulmonary infiltrates. METHODS: This single center, retrospective study included adult kidney transplant patients who underwent OLB. The patients had diffuse pulmonary infiltrates without definitive diagnoses and failed to respond to empiric antibiotic treatment. We analyzed the number of specific diagnoses, changes in treatment and the occurrence of complications in these patients. A logistic regression was used to determine which variables were predictors of hospital mortality. RESULTS: From April 2010 to April 2014, 87 patients consecutively underwent OLB. A specific diagnosis was reached in 74 (85.1%) patients. In 46 patients (53%), their therapeutic management was changed after the OLB results. Twenty-five (28.7%) patients had complications related to the OLB. The hospital mortality rate was 25.2%. Age, SAPS3 score and complications related to the procedure were independent predictors of all-cause mortality. CONCLUSIONS: OLB is a high-risk procedure with a high diagnostic yield in kidney transplant patients with diffuse pulmonary infiltrates who did not have a definitive diagnosis and who failed to respond to empiric antibiotic treatment. Complications related to OLB were common and were independently associated with intra-hospital mortality.

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.

The Presence of Diffuse Alveolar Damage on Open Lung Biopsy Is Associated With Mortality in Patients With Acute Respiratory Distress Syndrome: A Systematic Review and Meta-Analysis.

OBJECTIVE: Diffuse alveolar damage (DAD) is considered the histologic hallmark of ARDS although DAD is absent in approximately half of patients with ARDS. The clinical implications of having the syndrome of ARDS with DAD vs other histologic patterns is unknown. To address this question, we conducted a meta-analysis of lung biopsy series for patients with ARDS. METHODS: Studies were identified using MEDLINE, EMBASE, Cochrane Register of Controlled Trials, LILACS, and citation review from January 1, 1967, to September 1, 2015. Studies were included if they included all of the following: open lung biopsies (OLB) performed after ARDS diagnosis; a clear definition of ARDS and DAD; histologic results of the OLB indicated the presence or absence of DAD; and mortality reported for the DAD and non-DAD groups. We excluded studies conducted solely on a specific histology subgroup (eg, DAD) and studies with fewer than 5 patients. Two authors independently selected studies for inclusion, and there were no language restrictions. RESULTS: Of 8 included studies, 4 were high-quality (n = 228) and 4 were middle-quality trials (n = 122). The meta proportion of DAD between all the groups was 0.45 (95% CI, 0.35-0.56; Q test, 21.1; I(2), 66.8%; P ≤ .01). The pooled OR for mortality in ARDS with DAD compared with ARDS without DAD was 1.81 (95% CI, 1.14-2.80; Q test, 8.8; I(2), 20.2%; P = .269). Age, sex, and days elapsed between ARDS diagnosis and OLB as well as sequential organ failure assessment score and Pao2/Fio2 ratio on the day of OLB did not differ between DAD and non-DAD groups. CONCLUSIONS: This meta-analysis demonstrated that ARDS with DAD is associated with higher mortality than ARDS without DAD.

Diffuse Parenchymal Lung Diseases with Clinicoradiological Discordance: Role of Transbronchial Lung Biopsy as a Diagnostic Tool - An Observational Study.

INTRODUCTION: The diagnosis of Diffuse Parenchymal Lung Disease (DPLD) requires a multidisciplinary approach with reconciliation of clinicoradiological and histopathological data. But when the preliminary clinicoradiological profile fails to suggest a diagnosis, an adequate lung biopsy specimen with meticulous histological examination and a multidisciplinary approach usually yields results. There is also a high chance of sampling error due to patchy and heterogeneous involvement of the disease process and due to the small volume of tissue taken. As seen in our study, Trans-Bronchial Lung Biopsy (TBLB) if performed by an experienced bronchoscopist can be done as an outpatient procedure yielding adequate specimens for diagnosis and guide effective treatment in these patients. AIM: To study the utility and diagnostic yield of TBLB in DPLD patients when there is clinicoradiological discordance. MATERIALS AND METHODS: The current retrospective observational study was undertaken in the Institute of Chest Diseases, Government Medical College, Kozhikode, Kerala, India, from January 2012 to December 2014. Out of 169 DPLD patients who attended the tertiary care centre, 66 patients without a definite diagnosis by clinicoradiological assessment were included in the study. They underwent TBLB using a fibre-optic video bronchoscope. An open lung biopsy was advised if the TBLB did not yield a definite diagnosis. RESULTS: Among the 66 patients, histopathological confirmation was obtained in 51 patients, 39 of which were by TBLB (59%). Few diagnoses like invasive adenocarcinoma, Pneumocystis jirovecii pneumonia and Aspergillus infection were least expected. CONCLUSION: TBLB if performed correctly can be an effective intervening modality in establishing the diagnosis of DPLD before going for an invasive surgical biopsy.

Correlation between Radiological and Pathological Findings in Patients with Mycoplasma pneumoniae Pneumonia.

Studies focused on the pathological-radiological correlation of human Mycoplasma (M) pneumoniae pneumonia have rarely been reported. Therefore, we extensively reviewed the literature regarding pathological and radiological studies of Mycoplasma pneumonia, and compared findings between open lung biopsy specimen and computed tomography (CT). Major three correlations were summarized. (1) Peribronchial and perivascular cuffing characterized by mononuclear cells infiltration was correlated with bronchovascular bundles thickening on CT, which was the most common finding of this pneumonia. (2) Cellular bronchitis in the small airways accompanied with exudates or granulation tissue in the lumen revealed as centrilobular nodules on CT. (3) Neutrophils and exudates in the alveolar lumen radiologically demonstrated as air-space consolidation or ground-glass opacities. In M. pulmonis-infected mice model, pathologic patterns are strikingly different according to host cell-mediated immunity (CMI) levels; treatment with interleukin-2 lead to marked cellular bronchitis in the small airways and treatment with prednisolone or cyclosporin-A lead to neutrophils and exudates in the alveolar lumen. Patients with centrilobular nodules predominant radiologic pattern have a high level of CMI, measuring by tuberculin skin test. From these findings, up-regulation of host CMI could change radiological pattern to centrilobular nodules predominant, on the other hand down-regulation of host CMI would change radiological pattern to ground-glass opacity and consolidation. It was suggested the pathological features of M. pneumoniae pneumonia may be altered by the level of host CMI.

Bronchiolitis obliterans syndrome is not specific for bronchiolitis obliterans in pediatric lung transplant.

BACKGROUND: Bronchiolitis obliterans (BO) is the leading cause of mortality beyond the first year after pediatric lung transplant, but the performance of an open lung biopsy is often required for diagnosis. Bronchiolitis obliterans syndrome (BOS) is a clinical diagnosis based on spirometric data that is the accepted standard for staging chronic allograft dysfunction. METHODS: We determined the sensitivity, specificity, and positive and negative predictive values of BOS for predicting BO in children. A chart review was conducted on 139 open lung biopsies and 43 lung explants performed at our center from 1990 through June 2010 in pediatric recipients of lung transplants. Results were excluded from analysis if insufficient data existed to calculate a stable BOS stage before biopsy/explant. RESULTS: The criteria for inclusion in the study were met by 67 open lung biopsies and 31 lung explants. The sensitivity, specificity, positive predictive value, and negative predictive value of BOS for predicting BO were 91.0%, 25.8%, 72.6%, and 57.1%. CONCLUSIONS: We found that early declines in lung function are sensitive, but not specific, for BO. The low specificity of BOS for identifying BO illustrates the challenge facing clinicians in determining the etiology of pulmonary decline after lung transplant.

Pulmonary veno-occlusive disease in a female gardener.

Pulmonary veno-occlusive disease (PVOD) is a subgroup of pulmonary arterial hypertension with a poor prognosis. The diagnosis is usually delayed and treatment options other than lung transplantation are unfortunately limited. We report the case of 51-year-old female gardener diagnosed with PVOD by open lung biopsy before her death. Although there are many reported cases of hepatic veno-occlusive disease due to toxic agents present in nature, such as pyrrolizidine alkaloid exposure, to date this has not been linked to PVOD.

Invasive diagnostic strategies in immunosuppressed patients with acute respiratory distress syndrome.

Immunosuppression predisposes the host to development of pulmonary infections, which can lead to respiratory failure and the development of acute respiratory distress syndrome (ARDS). There are multiple mechanisms by which a host can be immunosuppressed and each is associated with specific infectious pathogens. Early invasive diagnostic modalities such as fiber-optic bronchoscopy with bronchoalveolar lavage, transbronchial biopsy, and open lung biopsy are complementary to serologic and noninvasive studies and assist in rapidly establishing an accurate diagnosis, which allows initiation of appropriate therapy and may improve outcomes with relative safety.

Corticosteroid monotherapy in a case of bronchocentric granulomatosis with a two-year follow-up.

Bronchocentric granulomatosis (BCG) is a rare disease. Because of the possibility of fungal infection, BCG has usually been treated with corticosteroids and antifungal agent. However, fungi are not detected in all BCG tissues. We report a case of proven BCG by open lung biopsy without fungi in a woman with corticosteroids monotherapy and two-year follow-up.

Diagnostic potential of open lung biopsy in mechanically ventilated patients with diffuse pulmonary infiltrates of unclear aetiology.

Acute respiratory failure with bilateral pulmonary infiltrates is a clinical problem with a wide differential diagnosis. We evaluated whether open lung biopsy offered a diagnosis and therapeutic guidance in cases of unclear diagnosis after non-invasive tests. For this purpose, we reviewed 60 cases of open lung biopsy performed between 2002 and 2009 in patients with acute respiratory failure and bilateral infiltrates at the intensive care unit of Ghent University Hospital. Pathological diagnosis was classified as specific or non-specific and its contribution to therapy and decision-making was evaluated by a panel of three intensive care unit physicians. We found that a specific pathological diagnosis was present in 39 open lung biopsy patients (65%; 95% confidence interval 52 to 76%): idiopathic interstitial pneumonia in 24 patients, malignancy in four patients, pulmonary infectious disease in nine patients, and a combination of specific diagnoses in two patients. Open lung biopsy contributed to patient management in 53 cases (88%) as it led to the initiation, modification or discontinuation of therapeutic drugs in 36, and contributed to the decision to continue or withdraw ventilator support in 17. Complications of open lung biopsy were noted in 14 patients (23%). We conclude that open lung biopsy was a useful diagnostic intervention in of a selected group of patients with acute respiratory failure and bilateral infiltrates of unclear clinical diagnosis, as it offered a specific diagnosis in 65%.