Interstitial Emphysema Research Articles

“Challenging acquired pulmonary interstitial emphysema in an extreme preterm infant: A case report and review of literature”

Background Pulmonary interstitial emphysema (PIE) can occur in the immature lungs of preterm infants due to overdistension from mechanical ventilation. Case Report Preterm female infant (26 weeks of gestational age, 950 grams) born via urgent cesarean section, APGAR 1/3/6. Invasive mechanical ventilation during the first day of life transitioned to noninvasive positive pressure ventilation (NIPPV), afterward initially stable. Apnea and bradycardia noted at 20 days of life, requiring endotracheal intubation, with complementary studies showing right PIE. Progressive emphysema deterioration, air trapping, and left lung atelectasis, with hemodynamic repercussion despite positioning, high-frequency oscillatory ventilation, and hemodynamic support with dopamine and dobutamine. Selective left intubation attempted positioning the infant, unsuccessful. Due to failure of conventional therapies and impossibility to achieve selective intubation, pediatric surgery performed endobronchial balloon occlusion with angioplasty balloon inflated with contrast with fluoroscopy control. Progressive improvement, weaning ventilatory settings with acceptable oxygenation. Balloon was retired after 5 days. Mechanical ventilation was discontinued at 52 days of life. Infant was discharged without respiratory support nor oxygen at 100 days of life. Conclusions PIE treatment is based in conventional measures (respiratory support and positioning) but eventually might require alternative techniques when the first fail, with limited work in pediatric population and further technical limitations in the preterm infants.

Correction to: Pulmonary Interstitial Emphysema in an Infant with Critical Congenital Heart Defect Associated with TNNC1 Gene Mutation and 22q11.2 Microdeletion: a Case Report

Correction to: Pulmonary Interstitial Emphysema in an Infant with Critical Congenital Heart Defect Associated with TNNC1 Gene Mutation and 22q11.2 Microdeletion: a Case Report

Deleterious impact of trivial to severe interstitial pneumonia and emphysema on mortality and acute exacerbation of interstitial pneumonia in patients with lung cancer: a retrospective cohort study

BackgroundInterstitial pneumonia and emphysema may complicate patients with lung cancer. However, clinical significance of trivial and mild pulmonary abnormalities remains unclear. In this study, we aimed to investigate whether trivial and mild interstitial pneumonia and emphysema, in addition to their advanced forms, impact the prognosis and lead to acute exacerbation of interstitial pneumonia (AEIP) in patients with lung cancer.MethodsThis retrospective cohort study was conducted at a tertiary hospital and included patients with lung cancer. Computed tomography images were evaluated using the interstitial lung abnormality (ILA) score for interstitial pneumonia, which included no ILA, equivocal ILA, ILA, interstitial lung disease (ILD), and the Goddard score for emphysema. Cox analyses were performed using the ILA and Goddard scores as the main explanatory variables, adjusting for multiple covariates.ResultsAmong 1,507 patients with lung cancer, 1,033 had no ILA, 160 had equivocal ILA, 174 had ILA, and 140 had ILD. In total, 474 patients (31.5%) exhibited interstitial pneumonia and 638 (42.3%) showed emphysema. The log-rank trend test showed that survival probability was significantly better in patients with no ILA, followed by those with equivocal ILA, ILA, and ILD (P < 0.001). After adjustment, the ILA and Goddard scores remained significant variables for increased hazard ratios (HR) for mortality: no ILA (HR, 1.00: reference), equivocal ILA (HR, 1.31; 95% confidence interval [CI], 1.18–1.46; P < 0.001), ILA (HR, 1.71; 95% CI, 1.39–2.12; P < 0.001), ILD (HR, 2.24; 95% CI, 1.63–3.09; P < 0.001), and Goddard score (HR, 1.03; 95% CI, 1.01–1.06; P < 0.010). Moreover, both scores were associated with increased cause-specific HRs for AEIP.ConclusionOur results revealed that approximately one-third of patients with lung cancer had interstitial pneumonia when incorporating trivial and mild cases. Because interstitial pneumonia and emphysema, ranging from trivial to severe, significantly impact mortality and AEIP in patients with lung cancer, we should identify even trivial and mild cases of these pulmonary abnormalities among patients with lung cancer in addition to the advanced ones.

Spontaneous pulmonary interstitial emphysema, pneumomediastinum and cervical subcutaneous emphysema secondary to the Macklin effect in dogs with no clinical signs: 12 cases

Pneumomediastinum denotes the presence of gas within the mediastinum and generally occurs by leakage of air from an aerated viscus that traverses or abuts the mediastinal plane. The Macklin effect has been described in several veterinary studies and describes gas tracking along the perivascular interstitium following alveolar rupture causing interstitial emphysema, pneumomediastinum and subsequently cervical subcutaneous emphysema. This retrospective case series describes incidental spontaneous pulmonary interstitial emphysema, pneumomediastinum and cervical subcutaneous emphysema secondary to the Macklin effect in dogs with no related clinical signs. Twelve dogs were identified from the author’s institution, of which 75 % were Sighthounds (Greyhounds, Whippets or Lurchers). Pulmonary interstitial emphysema had a predominantly paravascular distribution, although in some cases a parabronchial distribution was also identified. We conclude that incidental pulmonary interstitial emphysema, pneumomediastinum and secondary cervical subcutaneous emphysema can be incidental, presumed secondary to the Macklin effect and that Sighthound breeds may be overrepresented.

Pulmonary interstitial emphysema and spontaneous pneumomediastinum are more prevalent in sighthounds than other dog breeds undergoing thoracic CT.

Spontaneous pneumomediastinum is an uncommon condition described in veterinary medicine with concurrent respiratory disease. It can be caused by the Macklin effect, which is when gas leaks from the alveoli into the surrounding interstitial lung tissue. Pulmonary interstitial emphysema (PIE) is the presence of gas within the pulmonary vascular sheaths and indicates the presence of the Macklin effect. In the authors' experience, spontaneous pneumomediastinum and PIE are more prevalent in sighthound dogs than in other breeds and are often considered incidental findings. This retrospective, observational, cross-sectional study compared the prevalence of PIE and subsequent pneumomediastinum in sighthound with other purebred dogs. It characterized the appearance of PIE in CT and analyzed a possible association with concomitant pulmonary pathologies or with the use of general anesthesia. Medical records and thoracic CTs of sighthounds and nonsighthound dogs from two institutions were reviewed. A total of 256 dogs, comprising 127 sighthounds and 129 other purebred dogs, were included. The prevalence of PIE and pneumomediastinum was statistically higher in sighthound (14.2%) compared with other nonsighthound dogs (2.2%). There was no statistical association between the presence of PIE and pneumomediastinum with different age and weight groups or with sex. There was no statistical difference in the prevalence of PIE and pneumomediastinum between dogs with and without pulmonary pathology or in dogs undergoing CT with sedation or general anesthesia. Spontaneous pneumomediastinum in sighthounds is more prevalent than in other breeds, and its prevalence is not associated with the presence of pulmonary pathology or the use of general anesthesia.

Pulmonary Interstitial Emphysema in an Infant with Critical Congenital Heart Defect Associated with TNNC1 Gene Mutation and 22q11.2 Microdeletion: A Case Report

Pulmonary Interstitial Emphysema in an Infant with Critical Congenital Heart Defect Associated with TNNC1 Gene Mutation and 22q11.2 Microdeletion: A Case Report

Significant Spontaneous Pneumomediastinum and Extensive Subcutaneous Emphysema in a COVID-19 Patient

A 64-year-old man, who had no pre-existing health conditions, was admitted to the intensive care unit due to progressive shortness of breath resulting from COVID-19. Initially, the patient responded to non-invasive mechanical ventilation, which improved his breathing function. However, after six days, his respiratory function worsened significantly, requiring invasive ventilation. Out of nowhere, the person experienced spontaneous pneumomediastinum and extensive subcutaneous emphysema. The next day, a spontaneous pneumothorax occurred and was successfully drained later. It became evident that there was extensive subcutaneous emphysema also. The CT scan of the chest confirmed the presence of spontaneous pneumomediastinum, but it did not have any effect on the patient’s hemodynamics. The physicians performed a bronchoscopy and imaging with a contrast medium, which ruled out any lesions to the trachea or esophagus. No other issues related to the problem were identified during the examination. Unfortunately, microscopic bullae of interstitial emphysema, or micro air leaks, are visible when they are an adequate size on computed tomography. Recent literature and studies not available during the case report have shown that if the Macklin effect is detected on the baseline CT scan, it could predict the occurrence of pneumothorax or pneumomediastinum.

Subtle pulmonary interstitial emphysema in attempted suicide by ingestion of a cleaning product: Computed Tomography features

AbstractPulmonary interstitial emphysema (PIE) is a rare condition most commonly encountered in mechanically ventilated patients, either infants or adults. This condition can also be diagnosed in blunt chest trauma or other conditions while performing Valsalva-like maneuvers and is often associated with pneumothorax and subcutaneous emphysema. We present the case of a patient with subtle PIE, subcutaneous emphysema and massive pneumomediastinum after protracted vomiting following a suicidal attempt by caustic ingestion. Radiologists should be aware of the potentially subtle Computed Tomography features of PIE in order to make the correct diagnosis and rule out other tracheobronchial and esophageal injuries.

Hepatic venous gas secondary to pulmonary barotrauma: rat model study.

Intrahepatic gas (IHG) is commonly observed during early postmortem examinations of humans with upper or lower airway obstructions. We conducted a study to test the hypothesis that intrapulmonary gas could retrogradely spread to the hepatic vein following pulmonary barotrauma (PB). To establish a rat model of pulmonary barotrauma, we utilized a controllable pressure-vacuum pump to apply airway pressure (40, 60, or 80mmHg). The rats were dissected directly at the end of the experiment, and histological analysis was performed through microscopic examination of the rats. Additionally, the rats were ventilated with meglumine diatrizoate under pressures of 160 and 250mmHg to observe the signal dynamic diffusion using X-ray fluoroscopy examination. Rats exhibited classical changes associated with PB, such as alveolar rupture, pulmonary interstitial emphysema, and hemorrhage, as well as IHG characterized by the presence of gas in the hepatic vein and hepatic sinusoids. Air emboli were not observed in the liver in any of the 40mmHg groups. However, they were observed in the liver in the 60 and 80mmHg groups,the amount and size of air emboli in the 80mmHg group were greater than those in the 60mmHg group (p < 0.05). The 80mmHg group presented radial grape-like bubbles in the centrilobular portion of the liver accompanied by congestion in the peripheral region of the hepatic lobule. X-ray fluoroscopy examination revealed a gradual enhancement of dynamic contrast medium signals from the lung to the inferior vena cava and then to the liver. Our findings indicate that pulmonary barotrauma can lead to the retrograde spread of intrapulmonary gas to the hepatic vein. When it is clear that no decomposition of the body has occurred, the presence of IHG serves as a novel indicator for the diagnosis of obstructive pulmonary disease or obstruction in the upper or lower airway.

A Case of Pulmonary Interstitial Emphysema and Systematic Review of Literature in Management Approach

Background: Pulmonary interstitial emphysema (PIE) is a rare pathology characterized by the abnormal and harmful presence of air in the interstitial tissues of the lung. This condition is often related to barotrauma caused by mechanical ventilation, but it can be exceptionally seen in healthy infants. The main causes of PIE are respiratory distress syndrome (RDS), mechanical ventilation or positive pressure ventilation, prematurity, meconium aspiration syndrome, pulmonary infection, amniotic fluid aspiration, and incorrect endotracheal tube placement. To date, there is no standard treatment for PIE, based on the clinical and localization of PIE conservative and non-- conservative therapies are described. Methods: We describe a case of a very premature infant with severe respiratory failure secondary to RDS and unilateral left sided PIE with lung herniation treated with conservative therapy like selective intubation, steroid therapy, lateral position, and lastly, oxygen supplementation without ventilatory assistance. Furthermore, we have carried out a systematic literature review for the past 15 years (2007-2022). A systematic review, using an evidence-based algorithmic approach, was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Results: Our search, after the selection, produced a total of 24 articles, which were revised. Conclusion: In our case oxygen supplementation without ventilatory assistance resulted in successful resolution of the left PIE. Discontinuation of ventilatory care played a crucial role. Considering the management reported in literature, our aim is to perform a systematic literature review by adding our experience to the available knowledge on therapy for unilateral PIE.

Recurrent thoracic air leak syndrome in patients affected by pulmonary graft-versus-host disease: Surgical strategies and outcome.

Thoracic air leak syndrome (TALS) is a complication related to chronic pulmonary graft-versus-host disease (pGvHD) that affects approximately 0.83%-3.08% patients after allogenic hematopoietic stem cell transplant. Such complication is defined as the occurrence of any form of air leak in the thorax, including spontaneous pneumomediastinum or pneumopericardium, subcutaneous emphysema, interstitial emphysema and pneumothorax and has a negative impact on post-transplant survival. The aim of the present study is to describe a single-center experience in the surgical management of recurrent TALS in adolescents and young adults and its outcome. We retrospectively reviewed the clinical notes of patients with previous allogenic hematopoietic stem cell transplant who underwent surgical procedures for recurrent TALS from January 2016 until March 2021. We analyzed clinical data, number of episodes of thoracic air leak, surgical procedures and relative outcome. In the examined period, four patients, aged 16-25 years, underwent surgical procedures for TALS, including thoracostomy tube placement, thoracoscopic pleurodesis and thoracotomy. All the patients had been diagnosed with pGvHD before the onset of TALS, with a mean time lapse of 276 days (range 42-513). These patients experienced on average 4.5 air leak episodes (range 3-6). All the patients experienced at least two episodes before surgery. One patient underwent emergency tube thoracostomy only, three patients underwent thoracoscopic pleurodesis and two patients underwent thoracotomy. After surgery, patients were free from air leak symptoms for a mean time of 176 days (range 25-477). Pulmonary function progressively deteriorated, and all the patients eventually died because of respiratory failure after a mean time of 483 days (range 127-1045) after the first episode of air leak. Surgery provides temporary relief to symptoms related to TALS but has limited effects on the underlying pathophysiologic process. The development of TALS in a sign of progressive pulmonary function worsening and is associated with high risk of respiratory failure and mortality.

Combined Pulmonary Fibrosis and Emphysema: When Scylla and Charybdis Ally.

Combined pulmonary fibrosis and emphysema (CPFE) is a recently recognized syndrome that, as its name indicates, involves the existence of both interstitial lung fibrosis and emphysema in one individual, and is often accompanied by pulmonary hypertension. This debilitating, progressive condition is most often encountered in males with an extensive smoking history, and is presented by dyspnea, preserved lung volumes, and contrastingly impaired gas exchange capacity. The diagnosis of the disease is based on computed tomography imaging, demonstrating the coexistence of emphysema and interstitial fibrosis in the lungs, which might be of various types and extents, in different areas of the lung and several relative positions to each other. CPFE bears high mortality and to date, specific and efficient treatment options do not exist. In this review, we will summarize current knowledge about the clinical attributes and manifestations of CPFE. Moreover, we will focus on pathophysiological and pathohistological lung phenomena and suspected etiological factors of this disease. Finally, since there is a paucity of preclinical research performed for this particular lung pathology, we will review existing animal studies and provide suggestions for the development of additional in vivo models of CPFE syndrome.

Management of Air Leaks in the Newborn.

Air leaks, especially pulmonary interstitial emphysema (PIE), are increasingly uncommon respiratory complications of the newborn. Despite PIE having a decreasing incidence, it continues to have a high morbidity and mortality rate. This makes PIE one of the most severe respiratory complications to affect a newborn. Air leaks occur when there is overdistension of the terminal airways or alveoli, which results in air dissecting into extra-alveolar spaces. Recognizing the signs of air leaks is paramount to allow for appropriate diagnosis and treatment of the newborn to improve morbidity and mortality. Treatment modalities range from positioning the newborn in the decubitus position to surgical intervention. To summarize current evidence on the pathophysiology, risk factors, diagnosis, treatment, and management of PIE and pneumothorax. Two databases were systematically reviewed with key words later. Articles in English with date restrictions from 2016 to current were included. Inclusion criteria of randomized controlled trials, case studies/reports, and review articles were utilized. Twenty articles, primarily case reports and studies, were reviewed. Guidelines for data abstraction were followed via independent extraction by a single observer. Qualitative results were utilized to formulate current treatment of air leaks. This article provides guidance for frontline neonatal providers on current treatment and management of air leaks in the newborn population.

Pulmonary and Hepatic lesions in slaughtered sheep in Mosul city

The aim of study was designed to determine the prevalence rates, gross and histopathological lesions of lungs and livers in sheep slaughtered house at Mosul city. 9127 slaughtered sheep were examined During April and May 2014. 1235 infected carcasses were observed harboring different pathological gross lesions in the lungs and livers with 10.26% and 3.26% prevalence rates. The highest prevalence rates of gross pathological lesions of the lung were pneumonia of various types and the lightest was observed in T.B-like lesions was 3.34% and 0.04% out of total slaughtered sheep, respectively. In the affected livers, peak and the lowest prevalence of gross lesions were 0.77% for fascioliasis and 0.14% for enlarged and pale livers out of the total sheep ante-mortem inspected, respectively. However in the lungs, different rates of histopathological lesions were recorded for interstitial pneumonia, edema, emphysema, bronchopneumonia, atelectasis, verminous pneumonia, granuloma, necrosis and hemorrhage in variable percentages. As concerned with the liver, these microscopc lesions were coagulative necrosis, hepatic congestion, hydatid cysts, hemosiderin deposition, sinusoidal congestion, vacuolar degeneration and infiltration of inflammatory cells forming various rates. Uniform pathological observations and similar remarks of microscopical changes having identical description and morphological features were entirely demonstrated as explained in pathology references.

A case report of Indium lung with progressive emphysema and fibrosis underwent lung unilateral transplantation 20 years after the end of the exposure

BackgroundIndium lung is characterized by interstitial pneumonia and/or emphysema which occurs in indium-tin oxide (ITO) workers. Indium lung is now known to progress after stopping exposure to ITO, but the long-term influences of ITO remain unclear.Case presentationForty seven years old, a never-smoker, who had been engaged in an ITO manufacturing process for 8 years. Emphysema was indicated by the medical check-up for ex-ITO workers, and he was diagnosed with indium lung. He underwent partial lung resections for pneumothorax two times, and obstructive pulmonary dysfunction had progressed through the years. He underwent right single lung transplant 20 years after ITO exposure. Pathologically, his lung showed severe distal acinar emphysema and honeycomb change. Fibrosis and destruction of the lung tissue significantly progressed compared to the previous partial resections. Scanning electron microscopy combined with energy dispersive spectroscopy revealed that the deposited particles contained indium and tin. After the transplantation, his respiratory function was improved.ConclusionsIn this case, ITO resided in the lung tissue for 20 years, and lung tissue destruction kept progressing. Careful medical follow-up is recommended for ITO-workers even if they are asymptomatic.

“Pneumomediastinum: A marker of severity in Covid-19 disease”

ObjectiveThe goal of this study was to look at the incidence, risk factors, clinical characteristics, and radiological aspects of COVID-19 patients who developed pneumomediastinum and compare these features between those who died and those who survived. Materials and methodsThis retrospective observational study included COVID-19 patients having pneumomediastinum on CT from May 2020 to May 2021 in a COVID-19 care hospital. 1st wave patients were considered between the period of May 2020 to January 2021 and those in the second wave between February 2021 to May 2021. The clinical details were analyzed by a consultant intensivist and CT scans were read by a team of 6 resident radiologists and 5 experienced radiologists. Demographic data, co-morbidities, clinical parameters, hemodynamic markers, radiological involvement and associated complications were analyzed. ResultsDuring the study period, 10,605 COVID-19 patients were admitted to our hospital of which 5689 underwent CT scan. 66 patients were detected to have pneumomediastinum on CT; 26 of them in the first wave and 40 in the second wave. Out of 66, 28 patients were admitted to ICU, 9 during the first wave and 18 during the second wave. The overall incidence of developing pneumomediastinum was 1.16%. Incidence in the 1st wave was 1.0% and in the 2nd wave was 1.29%. The overall mortality rate in admitted COVID-19 patients was 12.83% while it was 43.9% in COVID-19 patients who developed pneumomediastinum. Incidence of pneumomediastinum and pneumothorax was high in patients with extensive parenchymal involvement. 59/66 (89%) cases of pneumomediastinum had severe CT score on imaging. ConclusionWe conclude that pneumomediastinum is a marker of poor prognosis. Timely diagnosis of interstitial emphysema or pneumomediastinum will aid in planning early protective ventilation strategies and timely intervention of complications.

Non-standard therapeutic methods for severe bronchopulmonary dysplasia in a premature newborn – case report

Bronchopulmonary dysplasia is a lung disease that is the most common complication of prematurity and the most common chronic lung disease in neonates treated with mechanical ventilation and/or oxygen therapy. Children with bronchopulmonary dysplasia are at risk of cardiovascular complications, such as pulmonary arterial hypertension and left ventricular hypertrophy. The presented case concerns a premature infant born at 24 weeks gestation with severe bronchopulmonary dysplasia complicated by pulmonary arterial hypertension, interstitial emphysema and emphysema. The patient was subject to non-standard therapeutic procedures including L-citrulline, which may have a beneficial effect on the treatment of complications of severe bronchopulmonary dysplasia. Despite proper treatment, it is not always possible to avoid severe complications of bronchopulmonary dysplasia. Currently, available therapies are not always fully effective and it is necessary to continue searching and implementing new treatments to lower the risk of infant death and minimise long-term complications.

Computed tomography findings of pulmonary interstitial emphysema in COVID-19: Looking through the lens.

Sir, We read Scialpi et al.’s[1] study evaluating the incidence of pulmonary interstitial emphysema (PIE) and other complications detected using computed tomography (CT) in COVID-19 patients. Although the findings are clinically relevant, some key aspects need to be clarified to define a specific impact and better understand. The authors emphasise early detection of the complications using CT that might help us optimise the ventilation option (noninvasive/invasive ventilation) to reduce the severity of complications. Although the basis is essential, there is controversy about the role of applied ventilation options, especially noninvasive ventilation (NIV) as an inducing factor of PIE or as a promoting factor.[2] In this sense, we also do not know how they can be identified and how to establish a better prevention strategy? The authors have summarised the number of PIE patients who received NIV and invasive mechanical ventilation (IMV). The relationship between PIE and NIV will be interesting. We could not establish an adequate interpretation and relationship of NIV with the PIE or other complications as comparative data required are lacking in the authors’ presented data. COVID-19 patients tend to have a higher incidence of barotrauma with IMV.[23] In this context, a comparison of PIE incidence in patients treated with NIV and IMV will help decide the mode of ventilation. Further, the airway pressures and ventilatory pressure setup in PIE and non-PIE patients receiving NIV will also help us optimise settings.[4] Further, we could not find precise mechanisms and possibilities that determine the evolution of different forms of PIE. In the authors’ cohort, variations in PIE were revealed in 25 patients and associated with pneumomediastinum, subcutaneous emphysema, and pneumothorax in 25 (100%), 16 (64%), and 7 (28%), respectively.[1] However, the determining factors that explain this variation allowing the framework of lung-protective strategies, prevention, and early diagnosis in high-risk patients are missing. It will be helpful for the readers if the authors present any such data from the cohort. It will help us plan future studies to evaluate the best tool for prevention and treatment. Author contribution HMRK, AME–observation, literature search, manuscript preparation, and editing. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Clinical characteristics and outcomes of MPO-ANCA-associated glomerulonephritis with bronchiectasis: A retrospective case-control study

BackgroundThe association of bronchiectasis with myeloperoxidase (MPO) antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (MPO-AAV) has been widely described in recent studies. However, the clinical features and outcomes of MPO-ANCA-associated glomerulonephritis (MPO-ANCA GN) patients with bronchiectasis remain enigmatic. MethodsMPO-ANCA GN patients with bronchiectasis were compared to MPO-ANCA GN patients alone. Clinical imaging, pathological tests, and follow-up examination data of patients were collected retrospectively. Progression to end-stage renal disease (ESRD) and death was treated as endpoint events. Results153 cases (52 patients with bronchiectasis) were included in this study. Compared to MPO-ANCA GN patients alone, MPO-ANCA GN patients with bronchiectasis exhibited a lower level of proteinuria (p = 0.019) and relatively higher eGFR level. MPO-ANCA GN patients with bronchiectasis had less frequent incidences of interstitial lung disease (ILD) and emphysema (p<0.001, p = 0.016, respectively) but with higher rates of pulmonary infection (p<0.001). Bronchiectasis severity (the modified Reiff score) was positively correlated with MPO antibody titers (ρ=0.480, p<0.001), but not with shorter renal survival. A relatively higher remission rate was been seen in MPO-ANCA GN patients with bronchiectasis, who showed reduced susceptibility in progressing to ESRD in multivariate analysis (p = 0.043, HR=0.542, 95% CI 0.299–0.982). One-and three-year overall survival rates were 88.2% and 77.3% for MPO-ANCA GN with bronchiectasis cases versus 83.7% and 67.2% for MPO-ANCA GN patients alone (p = 0.431, p = 0.241, respectively). ConclusionThe severity of bronchiectasis was correlated with anti-MPO antibody titers in MPO-ANCA GN patients. For MPO-ANCA GN patients, bronchiectasis associated with good renal prognosis, but it did not improve overall survival.

Syndrome of Combined Pulmonary Fibrosis and Emphysema: An Official ATS/ERS/JRS/ALAT Research Statement.

Background: The presence of emphysema is relatively common in patients with fibrotic interstitial lung disease. This has been designated combined pulmonary fibrosis and emphysema (CPFE). The lack of consensus over definitions and diagnostic criteria has limited CPFE research. Goals: The objectives of this task force were to review the terminology, definition, characteristics, pathophysiology, and research priorities of CPFE and to explore whether CPFE is a syndrome. Methods: This research statement was developed by a committee including 19 pulmonologists, 5 radiologists, 3 pathologists, 2 methodologists, and 2 patient representatives. The final document was supported by a focused systematic review that identified and summarized all recent publications related to CPFE. Results: This task force identified that patients with CPFE are predominantly male, with a history of smoking, severe dyspnea, relatively preserved airflow rates and lung volumes on spirometry, severely impaired DlCO, exertional hypoxemia, frequent pulmonary hypertension, and a dismal prognosis. The committee proposes to identify CPFE as a syndrome, given the clustering of pulmonary fibrosis and emphysema, shared pathogenetic pathways, unique considerations related to disease progression, increased risk of complications (pulmonary hypertension, lung cancer, and/or mortality), and implications for clinical trial design. There are varying features of interstitial lung disease and emphysema in CPFE. The committee offers a research definition and classification criteria and proposes that studies on CPFE include a comprehensive description of radiologic and, when available, pathological patterns, including some recently described patterns such as smoking-related interstitial fibrosis. Conclusions: This statement delineates the syndrome of CPFE and highlights research priorities.