Cystic Lung Research Articles

Sclerotic Fibroma in a Patient with Birt-Hogg-Dubé Syndrome

Birt-Hogg-Dubé syndrome (BHD) is a rare genetic disorder characterized by cutaneous lesions and systemic findings such as renal neoplasms, pulmonary cysts, and spontaneous pneumothoraces. Fibrofolliculomas, trichodiscomas, and acrochordons are the most common cutaneous lesions associated with BHD. Some cases with mucocutaneous lesions have also been reported. We report a sclerotic fibroma that developed over a previous thoracotomy scar of a patient with BHD.

Enhanced Gαq Signaling in TSC2-deficient Cells Is Required for Their Neoplastic Behavior.

Inherited or sporadic loss of the TSC2 gene can lead to pulmonary lymphangioleiomyomatosis (LAM), a rare cystic lung disease caused by protease-secreting interstitial tumor nodules. The nodules arise by metastasis of cells that exhibit features of neural crest and smooth muscle lineage ('LAM cells'). Their aberrant growth is attributed to increased activity of 'mechanistic target of rapamycin complex 1' (mTORC1), an anabolic protein kinase that is normally suppressed by the TSC1-TSC2 protein complex. The mTORC1 inhibitor rapamycin slows the progression of LAM, but fails to eradicate disease, indicating a role for mTORC1-independent mechanisms in LAM pathogenesis. Our previous studies revealed G-protein coupled urotensin-II receptor (UT) signaling as a candidate mechanism, but how it promotes oncogenic signaling in TSC2-deficient cells remained unknown. Using a human pluripotent stem cell-derived in vitro model of LAM, we now show hyperactivation of UT, which was required for their enhanced migration and pro-neoplastic signaling in a rapamycin-insensitive mechanism that required heterotrimeric Gαq/11 (Gαq). Bioluminescence resonance energy transfer assays in HEK 293T cells lacking TSC2 demonstrated selective and enhanced activation of Gαq and its RhoA-associated effectors compared to wild-type control cells. By immunoprecipitation, recombinant UT was physically associated with Gαq and TSC2. The augmented Gαq signaling in TSC2-deleted cells was independent of mTOR activity, and associated with increased endosomal targeting of p63RhoGEF, a known RhoA-activating effector of Gαq. These studies identify potential mTORC1-independent pro-neoplastic mechanisms that can be targeted for prevention or eradication of pulmonary and extrapulmonary LAM tumors.

Successful use of ultrasound guided pecto-intercostal fascial plane block to provide intraoperative analgesia in four dogs undergoing median sternotomy.

To evaluate the impact of pecto-intercostal fascial plane block on providing intraoperative analgesia in dogs undergoing median sternotomy. 4 dogs. The dogs were presented with a history of inappetence, lethargy and respiratory distress. Thoracic radiographs, point of care ultrasound, thoracocentesis, bronchoscopy and computed tomography was performed to characterize the disease. 4 male castrated, 5.3 ± 3 years old dogs weighing 19.7 ± 13.5 kg and belonging to Dalmatian, Beagle, Siberian Husky and Rottweiler breeds were included. Three dogs were diagnosed with suppurative pleural effusions because of pulmonary abscesses and one dog with spontaneous pneumothorax due to the presence of pulmonary bullae. All dogs underwent median sternotomy under general anesthesia to explore the thorax. A pecto-intercostal fascial plane block was performed by injecting local anesthetic bupivacaine in the parasternal fascial plane between the deep pectoral and external intercostal muscles to provide antinociception by anesthetizing ventral cutaneous branches of intercostal nerves second through sixth. Analgesia from the block resulted in reduced requirement of inhalant anesthesia and minimal requirement for opioid to augment analgesia intraoperatively. Median sternotomy is required to perform thoracic surgery in dogs with various thoracic pathologies. Pecto-intercostal fascial plane block is a locoregional technique that can blunt nociception arising from the ventral thorax and can significantly improve perioperative patient care in dogs undergoing median sternotomy by providing effective intraoperative and potentially postoperative analgesia.

Coinfection of pulmonary lophomoniasis, tuberculosis, and hydatid cyst in a pediatric autism patient: a case report and literature review

IntroductionAutism Spectrum Disorder (ASD) is a neurodevelopmental condition marked by difficulties in social interaction, communication, and repetitive behaviors or restricted interests. Although research on the link between ASD and parasitic diseases is limited, immune deficiency and inflammation may contribute to the development of parasitic infections.Case reportWe admitted a 14-year-old boy to the hospital who had a known history of ASD because he was presenting with respiratory symptoms, including cough and hemoptysis. During his time in the hospital, after conducting a series of tests and evaluations, we made a critical diagnosis of co-infection of lophomonas, tuberculosis, and hydatid cyst. In response to this diagnosis, we initiated a treatment plan that involved administering appropriate antibiotics as well as preparing for surgical intervention.ConclusionThis case report highlights the complexities and challenges of managing such a rare combination of co-infections with TB, pulmonary hydatid disease and lophomonas in a pediatric patient with underlying developmental considerations such as ASD.

Correlation between elderly patients with COPD and the impact on immunity in tuberculosis patients: A retrospective study.

The prevalence of chronic obstructive pulmonary disease (COPD) and tuberculosis (TB) is increasing globally, yet their comprehensive impact on the immune system remains underexplored. This study aimed to provide a thorough assessment of the immune status of patients with COPD and tuberculosis (TB-COPD), including their pulmonary conditions, immune cell responses, and changes in lymphocyte subpopulations. A total of 151 patients with TB-COPD patients were included, and clinical data were compared between the TB-COPD group and a group of TB patients without COPD (TB-NCOPD). Lung imaging findings and peripheral blood immune cell levels were compared between the 2 groups. Flow cytometry was used to analyze the absolute counts of lymphocyte subpopulations. The incidence of pulmonary lobe lesions and cavitation in the TB-COPD group aged 70 years or older was significantly higher than that in the control group. At the immune cell level, patients with TB-COPD showed a significant reduction in total lymphocytes, CD4+ T lymphocytes and CD4+/CD8+ ratio. Regardless of COPD status, the CD4+ T cell count in the CMV-infected group was significantly lower than that in the uninfected group (P < .05). Additionally, the CD4+/CD8+ ratio in the COPD + TB CMV + group was significantly lower than that in the uninfected group. Analysis of lymphocyte subpopulations revealed a decrease in the counts of CD4+ T lymphocytes in patients with TB-COPD, potentially associated with the chronic inflammatory state induced by COPD. The one-month treatment outcomes showed that the improvement rate in the control group was 70.58%, which was significantly higher than the 38.92% in the COPD + TB group (P < .001). We observed a significant increase in the number of pulmonary cavity patients in the TB-COPD group, suggesting that COPD may be a potential risk factor for the formation of pulmonary cavities in patients with TB. At the immune cell level, TB-COPD patients showed a notable decrease in lymphocytes and CD4+ T lymphocytes, implying that COPD combined with pulmonary TB may significantly affect the immune system, leading to a reduction in the counts of key immune cells.

Analysis of risk factors for pneumothorax after particle implantation in the treatment of advanced lung cancer after surgery and establishment of a nomogram prediction model.

To analyze the risk factors for pneumothorax after particle implantation in the treatment of advanced lung cancer and to construct and validate a nomogram prediction model. A retrospective analysis was conducted on 148 patients who underwent 125I particle implantation for advanced lung cancer at the *** from December 2022 to December 2023. Potential risk factors were identified using univariate logistic regression analysis, followed by a multivariate logistic regression analysis to evaluate the predictive factors for pneumothorax. Interaction effects between variables were studied and incorporated into the model construction. ROC curves and nomograms were generated for visualization. Calibration analysis was performed, and the corresponding net benefit was calculated to adjust the predictive model. Among the 148 patients, 58 (39.19%) experienced pneumothorax, with a mean age of 62.5 (55.25, 70) years. Multivariate analysis showed that the angle between the puncture needle and the pleura < 50° (P = 0.002, OR: 3.908, CI: 1.621-9.422), preoperative CT suggesting emphysema (P = 0.002, OR: 3.798, CI: 1.600-9.016), atelectasis (P = 0.009, OR: 3.156, CI: 1.331-7.481), and lesion located in the left lung fissure (P = 0.008, OR: 4.675, CI: 14.683) were independent risk factors for pneumothorax after particle implantation in the treatment of advanced lung cancer. Preoperative CT suggesting lesions in the left lung fissure or suggesting emphysema had a significant impact in the nomogram, with probabilities of pneumothorax occurrence at 40% and 38%, respectively. The predictive AUC for the above four risk factors for pneumothorax after particle implantation in the treatment of lung adenocarcinoma was 0.837 (95% CI: 0.767-0.908). When the Youden index was 0.59, the sensitivity was 85.56%, specificity was 74.13%, accuracy was 81.01%, positive predictive value was 83.69%, and negative predictive value was 76.78%. The angle between the puncture needle and the pleura < 50°, preoperative CT suggesting emphysema, atelectasis, and lesion located in the left lung fissure are independent risk factors for pneumothorax after particle implantation in the treatment of advanced lung cancer. Preoperative planning of the puncture path should avoid lung bullae, interlobar fissures, areas of severe emphysema, and atelectasis.

Clinical and Functional Outcomes Associated with Quality of Life in Patients with Lymphangioleiomyomatosis: A Cross-Sectional Study.

Lymphangioleiomyomatosis (LAM) is a rare (twenty-one per million female inhabitants) neoplastic cystic lung disease that impairs health-related quality of life (HRQoL). However, the factors associated with impaired quality of life in patients with LAM are poorly understood. To assess the clinical, psychosocial, and functional characteristics associated with impaired quality of life in patients with LAM. This was a cross-sectional study performed on two nonconsecutive days. HRQoL (SF-36 and CRQ), lung function tests, anxiety and depression symptoms (HADS), maximal (CPET and ISWT), and submaximal exercise capacity (6MWT) were assessed. Linear associations among outcomes were assessed using Pearson's correlation and multivariate tests. Forty-five women with LAM (46 ± 10.years; FEV1,74%pred) were evaluated. The lowest SF-36 scores were observed for general health and vitality and the highest for the physical and social domains. The lowest CRQ scores were observed for dyspnea and fatigue, and the highest were for the emotional function and self-control domains. Sixteen (35%) women had anxiety, and 8 (17%) had depression symptoms. Most of the SF-36 and CRQ domains were associated with anxiety and depression symptoms (from r = 0.4 to r = 0.7; p < 0.05) and exercise capacity (from r = 0.3 to r = 0.5; p < 0.05). Lung function parameters were weakly or not associated with quality of life domains. After multiple linear regression, HRQoL was independently associated with depression symptoms and physical capacity but not with lung function. Our results show that aerobic capacity and depression symptoms are the main factors, rather than lung function, related to quality of life in patients with LAM.

Application and clinical effects in lung bullae resection of CT 3-dimensional reconstruction.

To investigate the value and clinical effect of computed tomography (CT) 3-dimensional reconstruction technology in lung bullae resection. Ninety-eight patients who underwent thoracoscopic surgical resection of lung bullae in our hospital from February 2019 to February 2023 were selected and divided into 2 groups according to the principle of voluntary participation in the clinical trial, of which 43 cases underwent preoperative spiral CT for thin-layer enhancement or planar CT scanning of lung bullae (control group), and 43 cases were imaged with MimicsMedical 21 software for 3-dimensional reconstruction to develop a surgical plan (observation group). To compare the surgery-related indexes, postoperative complications, pain, and analgesic use between the 2 groups, we measured serum creatine phosphokinase and myoglobin levels. The amount of surgical bleeding, drainage volume, hospital stay, and postoperative visual analog scale score of the research group were lower than those of the control group (P < .05), and the duration of analgesic medication was shorter than that of the control group (P < .05), and the levels of serum creatine phosphokinase and myoglobin were lower than those of the control group at 1 and 3 days postoperatively, and the differences were statistically significant (P < .05). Preoperative CT 3-dimensional reconstruction technique can clearly show the anatomical structures around the lung bullae and has its application value for precise lung bullae.

Pediatric pleuropulmonary blastoma: analysis of four cases

BackgroundPleuropulmonary Blastoma (PPB) is an extremely uncommon, highly aggressive tumor that arises from either the lungs or pleura. According to Dehner, PPB was classified into three groups: type I (cystic), type II (mixed), and type III (solid). Type I tends to occur more commonly in infants and has a more favorable prognosis compared to types II and III. This tumor is very rare in pediatric age group; hence, there is no consensus on the optimal treatment regimen for it to date. Type I tumors, which resemble congenital lung cysts, can eventually progress to more aggressive type II and type III tumors. This article aims to increase general awareness of this pathology, clinical presentation, and differential diagnosis in order to identify this rare entity early in its course. By presenting 4 such cases, we highlight that PPB can be missed early in diagnosis and it is important to be alert when putting this rare tumor in differential diagnosis of cystic lung lesions.MethodsA retrospective study was conducted between 2015 and 2020 involving patients who had a definitive diagnosis of PPB with emphasis on clinical presentation, preoperative imaging studies, intra-operative findings, pathological reports, ancillary treatment, and outcomes. All patients were followed up every 6 months to monitor local recurrence and distant metastasis by undergoing physical exam and non-contrast enhanced CT of the chest. The primary outcome is to identify the mortality and morbidity (recurrence and distant metastasis) of PPB for cases admitted in our institute.ResultsFour children were diagnosed with PPB during the study period. Clinically, patients presented with manifestations ranging from respiratory distress, fever to obstructive shock and radiologically, 2 cases were presented with mediastinal mass and the other 2 presented with pneumothorax. Regrettably, none of the cases were diagnosed pre-operatively. One lesion proved to be type I, 2 were type II and one was type III. All cases underwent chemotherapy using the combination of vincristine, Adriamycin and cyclophosphamide (VAC regimen). Recurrence was detected in a type II case, around 2 years after operation, and the other type II case developed brain metastasis that was discovered 3 years after operation. Type I case showed no local or distant metastasis.ConclusionA prompt preoperative diagnosis and workup of cases of PPB is crucial to enable optimal intervention intraoperatively and early postoperative treatment. Though it is uncommon, PPB should be considered in the differential diagnosis of cystic lung lesions.

Pityriasis Rubra Pilaris and underlying malignancy.

A 59-year-old man presented with a five-month history of widespread, salmon-colored, scaly plaques. Skin examination revealed salmon-colored plaques with "islands of sparing" on the trunk and waxy keratoderma on the palms and soles. Histopathology of the lesion showed alternating orthokeratosis and parakeratosis in vertical and horizontal directions, consistent with a diagnosis of pityriasis rubra pilaris (PRP). The patient was unresponsive to methotrexate and Secukinumab. Further evaluation through a computerized tomography scan revealed a presence of air-filled multicystic changes in the upper left lung, accompanied by diffuse pulmonary bullae. Elevated serum tumor markers, including CEA, NSE, and SCC, indicated an underlying acinar-predominant adenocarcinoma, suggesting a paraneoplastic PRP. He was subsequently referred to the oncology department for further management. This report underscores the importance of identifying an underlying malignancy in recalcitrant PRP cases.

7908 GPNMB Promotes Tumor Growth And Is A Biomarker For Lymphangioleiomyomatosis (LAM)

Abstract Disclosure: E. Gibbons: None. M. Taya: None. H. Wu: None. S. Lopa: None. J. Moss: None. E.P. Henske: None. F.X. McCormack: None. S.R. Hammes: None. Lymphangioleiomyomatosis (LAM) is a rare, progressive cystic lung disease affecting almost exclusively female-sexed individuals. The cysts represent regions of lung destruction caused by smooth muscle tumors containing mutations in one of the two tuberous sclerosis (TSC) genes. mTORC1 inhibition slows but does not stop LAM advancement. Furthermore, monitoring disease progression is hindered by insufficient biomarkers. Therefore, new treatment options and biomarkers are needed. LAM cells express melanocytic markers, including Glycoprotein Non-Metastatic Melanoma Protein B (GPNMB). The function of GPNMB in LAM is currently unknown; however, GPNMB’s unique cell surface expression on tumor versus benign cells makes GPNMB a potential therapeutic target, and persistent release of its extracellular ectodomain suggests potential as a serum biomarker. Here we establish that GPNMB regulates TSC2-null tumor cell invasion as well as TSC2-null xenograft tumor growth, and that GPNMB expression is dependent on mTORC1 signaling. Further, we show that GPNMB’s ectodomain is released from the cell surface of TSC2-null cells by ADAM10 and 17 proteases, and we identify the protease target sequence on GPNMB. In fact, mutating this cleavage sequence so that the GPNMB ectodomain can no longer be shed results in cessation of TSC2-null tumor growth. Finally, we demonstrate that GPNMB’s ectodomain is present at higher levels in LAM patient serum compared to healthy controls, and that ectodomain levels decrease with mTORC1 inhibition, making it a potential LAM biomarker. Presentation: 6/3/2024

7908 GPNMB Promotes Tumor Growth and is a Biomarker for Lymphangioleiomyomatosis (LAM)

Abstract Disclosure: E. Gibbons: None. M. Taya: None. H. Wu: None. S. Lopa: None. J. Moss: None. E.P. Henske: None. F.X. McCormack: None. S.R. Hammes: None. Lymphangioleiomyomatosis (LAM) is a rare, progressive cystic lung disease affecting almost exclusively female-sexed individuals. The cysts represent regions of lung destruction caused by smooth muscle tumors containing mutations in one of the two tuberous sclerosis (TSC) genes. mTORC1 inhibition slows but does not stop LAM advancement. Furthermore, monitoring disease progression is hindered by insufficient biomarkers. Therefore, new treatment options and biomarkers are needed. LAM cells express melanocytic markers, including Glycoprotein Non-Metastatic Melanoma Protein B (GPNMB). The function of GPNMB in LAM is currently unknown; however, GPNMB’s unique cell surface expression on tumor versus benign cells makes GPNMB a potential therapeutic target, and persistent release of its extracellular ectodomain suggests potential as a serum biomarker. Here we establish that GPNMB regulates TSC2-null tumor cell invasion as well as TSC2-null xenograft tumor growth, and that GPNMB expression is dependent on mTORC1 signaling. Further, we show that GPNMB’s ectodomain is released from the cell surface of TSC2-null cells by ADAM10 and 17 proteases, and we identify the protease target sequence on GPNMB. In fact, mutating this cleavage sequence so that the GPNMB ectodomain can no longer be shed results in cessation of TSC2-null tumor growth. Finally, we demonstrate that GPNMB’s ectodomain is present at higher levels in LAM patient serum compared to healthy controls, and that ectodomain levels decrease with mTORC1 inhibition, making it a potential LAM biomarker. Presentation: 6/3/2024

9308 GPNMB Promotes Tumor Growth And Is A Biomarker For Lymphangioleiomyomatosis (LAM)

Abstract Disclosure: E. Gibbons: None. M. Taya: None. H. Wu: None. S. Lopa: None. J. Moss: None. E.P. Henske: None. F.X. McCormack: None. S.R. Hammes: None. Lymphangioleiomyomatosis (LAM) is a rare, progressive cystic lung disease affecting almost exclusively female-sexed individuals. The cysts represent regions of lung destruction caused by smooth muscle tumors containing mutations in one of the two tuberous sclerosis (TSC) genes. mTORC1 inhibition slows but does not stop LAM advancement. Furthermore, monitoring disease progression is hindered by insufficient biomarkers. Therefore, new treatment options and biomarkers are needed. LAM cells express melanocytic markers, including Glycoprotein Non-Metastatic Melanoma Protein B (GPNMB). The function of GPNMB in LAM is currently unknown; however, GPNMB’s unique cell surface expression on tumor versus benign cells makes GPNMB a potential therapeutic target, and persistent release of its extracellular ectodomain suggests potential as a serum biomarker. Here we establish that GPNMB regulates TSC2-null tumor cell invasion as well as TSC2-null xenograft tumor growth, and that GPNMB expression is dependent on mTORC1 signaling. Further, we show that GPNMB’s ectodomain is released from the cell surface of TSC2-null cells by ADAM10 and 17 proteases, and we identify the protease target sequence on GPNMB. In fact, mutating this cleavage sequence so that the GPNMB ectodomain can no longer be shed results in cessation of TSC2-null tumor growth. Finally, we demonstrate that GPNMB’s ectodomain is present at higher levels in LAM patient serum compared to healthy controls, and that ectodomain levels decrease with mTORC1 inhibition, making it a potential LAM biomarker. Presentation: 6/3/2024

Rare genetic interstitial lung diseases: a pictorial essay.

The main monogenic causes of pulmonary fibrosis in adults are mutations in telomere-related genes. These mutations may be associated with extrapulmonary signs (hepatic, haematological and dermatological) and typically present radiologically as usual interstitial pneumonia or unclassifiable fibrosis. In children, the monogenic causes of pulmonary fibrosis are dominated by mutations in surfactant-related genes. These mutations are not associated with extrapulmonary signs and often manifest radiologically as unclassifiable fibrosis with cysts that can lead to chest wall deformities in adults. This review discusses these mutations, along with most of the monogenic causes of interstitial lung disease, including interferon-related genes, mutations in genes causing cystic lung disease, Hermansky-Pudlak syndrome, pulmonary alveolar proteinosis, lysinuric protein intolerance and lysosomal storage disorders, and their pulmonary and extrapulmonary manifestations.

Assessment of intercostal nerve block analgesia and local anesthetic infiltration for thoracoscopic pulmonary bullae resection: a comparative study

ObjectiveThe purpose of this study was to compare the analgesic effects of intercostal nerve block (ICNB) and local anesthetic infiltration (LAI) on postoperative pain and recovery following thoracoscopic resection of pulmonary bullae.MethodsA total of 160 patients undergoing thoracoscopic pulmonary bullae resection were randomly assigned to receive either ICNB (n = 80) or LAI (n = 80). An experienced anesthesiologist administered ultrasound guided ICNB at the T4 and T7 levels with 5 mL of 0.375% ropivacaine hydrochloride for the ICNB group. Instead, the LAI group received 10 mL of the same concentration of ropivacaine hydrochloride at the same concentration used for ICNB for infiltration anesthesia at the incision sites. Out of the initial cohort, 146 patients completed the study (ICNB group, n = 71; LAI group, n = 75). The collected data included preoperative clinical characteristics, visual analog scale (VAS) scores for pain at various time points post-surgery (6, 12, 24, 48, and 72 h). Additionally, the Quality of Recovery-15 (QoR-15) questionnaire was administered 24 h after surgery, and sleep quality was evaluated using the Pittsburgh Sleep Quality Index (PSQI).ResultsNo significant differences were found in drainage volume, use of additional analgesics, duration of chest tube placement, or hospital stay between the two groups. However, the ICNB group had significantly lower VAS scores and QoR-15 scores 24 h postoperatively (p < 0.05), indicating better pain management and recovery. The ICNB group also reported better sleep quality, as reflected by lower PSQI scores.ConclusionICNB provides superior analgesia compared to LAI after thoracoscopic resection of pulmonary bullae, significantly improving postoperative recovery.

A case of cytomegalovirus cystic lung disease and review of literature.

A 31-week gestation male infant with respiratory distress since day of delivery had lobectomy at 8 weeks of age for symptomatic, suspected congenital pulmonary airway malformation (CPAM). Histology of resected lung showed cytomegalovirus (CMV) inclusion bodies and emphysematous changes. The infant was treated with antiviral therapy with improvement in symptoms. CMV infection of the lung should be considered in any neonate presenting with lung cysts.

Silence of the lungs: comparing measures of slow and noncommunicating lung units from pulmonary function tests with computed tomography.

Multiple breath washout (MBW) has successfully assessed the silent lung zone particularly in cystic fibrosis lung disease, however, it is limited to the communicating lung only. There are a number of different pulmonary function methods that can assess what is commonly referred to as trapped air, with varying approaches and sensitivity. Twenty-five people with cystic fibrosis (pwCF) underwent MBW, spirometry, body plethysmography, and spirometry-controlled computed tomography (spiro-CT) on the same day. PwCF also performed extensions to MBW that evaluate air trapping, including our novel extension (MBWShX), which reveals the extent of underventilated lung units (UVLU). In addition, we used two previously established 5-breath methods that provide a volume of trapped gas (VTG). We used trapped air % from spiro-CT as the gold standard for comparison. UVLU derived from MBWShX showed the best agreement with trapped air %, both in terms of correlation (RS 0.89, P < 0.0001) and sensitivity (79%). Bland-Altman analysis demonstrated a significant underestimation of the VTG by both 5-breath methods (-249 mL [95% CI -10,796; 580 mL] and -203 mL [95% CI -997; 591 mL], respectively). Parameters from both spirometry and body plethysmography were suboptimal at assessing this pathophysiology. The parameters from MBWShX demonstrated the best relationship with spiro-CT and had the best sensitivity compared with the other pulmonary function methods assessed in this study. MBWShX shows promise to assess and monitor this critical pathophysiological feature, which has been shown to be a driver of lung disease progression in pwCF.NEW & NOTEWORTHY We consider the term "trapped air" either in the use of imaging or pulmonary function testing, something of a misnomer that can lead to an inaccurate assessment of an important physiological feature. Instead, we propose the term underventilated lung units (UVLU). Of the many pulmonary function methods we used in this study, we found that the use of multiple breath washout with short extension (MBWShX) to be the best nonimaging method.

Variability of radiological and clinical features in cases with usual interstitial pneumonia without honeycombing

BackgroundUsual interstitial pneumonia (UIP) cases without honeycombing (possible UIP) included various CT features and was often difficult to diagnose. PurposeThis study aimed to classify the cases with possible UIP on CT features using cluster analysis and evaluate the features of subsets of participants and the correlation of prognosis. Materials and MethodsThe study included 85 patients with possible UIP in the 2011 idiopathic pulmonary fibrosis (IPF) guideline with radiological diagnosis. All cases underwent surgical biopsies and were diagnosed by multidisciplinary discussion (MDD) from the nationwide registry in Japan. The readers evaluated pulmonary opacity, nodules, cysts, and predominant distribution which were reclassified by IPF guidelines in 2018. Additionally, cases were classified into four groups by cluster analysis based on CT findings. The differences in survival among IPF classification and the clusters were evaluated. ResultsCases were diagnosed as IPF (n = 55), NSIP (n = 4), unclassifiable (n = 23), and others (n = 3) by MDD. Cluster analysis revealed 4 clusters by CT features (n = 47, 16, 19 and 3, respectively). Cluster 1 had fewer lesions overall. Cluster 2 have many pure ground-glass opacities and ground-glass opacities with reticulation. Cluster 3 had many reticular opacities and nodules with few lower predominant distributions. Cluster 4 was characterized by peribronchovascular consolidation.The mean survival time of cluster 1 (4518 days) was significantly better than cluster 2, 3, and 4 (1843, 2196, and 1814 days, respectively) (p = 0.03). ConclusionIn conclusion, UIP without honeycombing included various CT patterns and MDD diagnoses. Significangly differences in prognosis were observed among clusters classified by CT findings.

Dépôts tissulaires non amyloïdes d’immunoglobuline monoclonale (hors atteinte rénale)

Monoclonal immunoglobulins can form deposits other than amyloidosis in various tissues. Immunofluorescence is the key analysis for the identification of monoclonal immunoglobulin deposits. Pulmonary light chain deposition disease is a diagnosis to be considered when dealing with diffuse cystic lung disease.

TNF-α inhibitors in the management of refractory granulomatosis with polyangiitis: A report of two cases

Granulomatosis with polyangiitis (GPA) is a type of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) that causes granulomatous necrotizing inflammation of small blood vessels. The goal of AAV treatment is to create a long-term remission. For inducing remission, the European League Against Rheumatism recommends using glucocorticoids (GCs) in combination with either cyclophosphamide (CYC) or rituximab (RTX). Despite this, in 10-40% the disease is resistant to initial treatment. In this report, we present two cases of refractory GPA that successfully achieved long-term remission using tumor necrosis factor-α inhibitors. The first case is a 27-year-old woman with GPA with involvement of upper airway, breast and central nervous system with active disease despite treatment with high dose GCs, CYC and RTX, successfully treated with infliximab and the disease was in complete remission for 54 months. The second case is a 35-year-old man with GPA with arthritis, lung cavitary lesions and nephritis resistant to treatment with high dose GCs, CYC and RTX, treated with adalimumab and the disease was in complete remission for 40 months.