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Given the recently expanded approval of antifibrotics for various fibrotic interstitial lung diseases (ILDs), early and correct recognition of these diseases is imperative for physicians. Because high-resolution chest CT scan forms the backbone of diagnosis for ILD, this review will discuss evidence-based imaging findings of key fibrotic ILDs and an approach for differentiating these diseases. (1) Imaging findings of nonspecific interstitial pneumonia may evolve over time and become indistinguishable from usual interstitial pneumonia. Therefore, if remote imaging can be reviewed, this would increase the likelihood of an accurate imaging diagnosis, particularly if findings appear to represent a usual interstitial pneumonia pattern on the recent examination. (2) Given the difficulty and lack of objectivity in classifying patients with hypersensitivity pneumonitis into acute, subacute, and chronic categories and that prognosis depends primarily on presence or absence of fibrosis, the new set of guidelines released in 2020 categorizes patients with hypersensitivity pneumonitis as either nonfibrotic (purely inflammatory) or fibrotic (either purely fibrotic or mixed fibrotic/inflammatory) based on imaging and/or histologic findings, and the prior temporal terms are no longer used. (3) Interstitial lung abnormalities are incidental CT scan findings that may suggest early ILD in patients without clinical suspicion for ILD. Patients with high-risk features should undergo clinical evaluation for ILD and be actively monitored for disease progression. Fibrotic ILD on high-resolution chest CT scan is a complex topic, but with use of an evidence-based analysis and algorithm as provided in this article, the probability of a correct imaging diagnosis increases. [ABSTRACT FROM AUTHOR]

Over the years, ensuring the necessary resources to meet the performance standards imposed by international organizations to which Romania belongs, namely NATO, has held a very important place among the main objectives and activities of the state. The extensive process of transformation and continuous adaptation undergone by various bodies and structures of the country has led to a harmonization of resources, with studies conducted on the unfolding phenomena and measures implemented to adapt to the requirements of today's society regarding their management. The objective of this study is to outline a general overview of how NATO investment projects are carried out in Romania through public institutions under the Ministry of National Defense, using financial and accounting information, in compliance with national and international legislation, to ensure the continuous fulfillment of objectives and standards set by the North Atlantic Alliance.

Background. The diffusing capacity of the lungs for carbon monoxide (DLCO) represents the most sensitive independent factor for highlighting the interstitial lung disease associated with rheumatoid arthritis (RA-ILD). Many studies have analyzed this association between RA and lung modifications because it correlates with a decrease in the patients’ quality of life and a higher mortality. Objective. This study aimed to highlight the importance of DLCO as a predictor of ILD progression in RA, beside the pattern on high-resolution chest CT (HRCT) and the double seropositivity of the disease. Patients and method. This prospective cohort study, carried out between august 2022 and may 2023, included 48 RA patients with ILD, fulfilling the 1987 ACR or 2010 ACR/EULAR classification criteria. Important attention was paid to respiratory functional tests, immunological changes, and specific lung pattern on HRCT. Patients with current malignancies, active infections or Overlap syndrome were excluded from the study. Results. 48 RA patients, mostly women (81%) with a mean age of 65.54±10.58 years old and a median age of RA onset of 52.58±11.36 years, were included. The proportion of patients with a decreased DLCO was 64.6%. Using statistical tests, we found significant correlations between DLCO and age, double seropositivity and a specific pulmonary pattern on HRCT. Conclusion. ILD, an underrecognized disease, may be considered one of the most serious of all extra-articular manifestations in RA, the risk for mortality being triple in these cases. Our results are in correlation with the published data and support the key role of DLCO in the diagnosis and follow-up of these patients.

Background. The diffusing capacity of the lungs for carbon monoxide (DLCO) represents the most sensitive independent factor for highlighting the interstitial lung disease associated with rheumatoid arthritis (RA-ILD). Many studies have analyzed this association between RA and lung modifications because it correlates with a decrease in the patients’ quality of life and a higher mortality. Objective. This study aimed to highlight the importance of DLCO as a predictor of ILD progression in RA, beside the pattern on high-resolution chest CT (HRCT) and the double seropositivity of the disease. Patients and method. This prospective cohort study, carried out between august 2022 and may 2023, included 48 RA patients with ILD, fulfilling the 1987 ACR or 2010 ACR/EULAR classification criteria. Important attention was paid to respiratory functional tests, immunological changes, and specific lung pattern on HRCT. Patients with current malignancies, active infections or Overlap syndrome were excluded from the study. Results. 48 RA patients, mostly women (81%) with a mean age of 65.54±10.58 years old and a median age of RA onset of 52.58±11.36 years, were included. The proportion of patients with a decreased DLCO was 64.6%. Using statistical tests, we found significant corelations between DLCO and age, double seropositivity and a specific pulmonary pattern on HRCT. Conclusion. ILD, an underrecognized disease, may be considered one of the most serious of all extra-articular manifestations in RA, the risk for mortality being triple in these cases. Our results are in correlation with the published data and support the key role of DLCO in the diagnosis and follow-up of these patients. [ABSTRACT FROM AUTHOR]

Over the years, ensuring the necessary resources to meet the performance standards imposed by international organizations to which Romania belongs, namely NATO, has held a very important place among the main objectives and activities of the state. The extensive process of transformation and continuous adaptation undergone by various bodies and structures of the country has led to a harmonization of resources, with studies conducted on the unfolding phenomena and measures implemented to adapt to the requirements of today's society regarding their management. The objective of this study is to outline a general overview of how NATO investment projects are carried out in Romania through public institutions under the Ministry of National Defense, using financial and accounting information, in compliance with national and international legislation, to ensure the continuous fulfillment of objectives and standards set by the North Atlantic Alliance. [ABSTRACT FROM AUTHOR]

Background: Pulmonary lung involvement is the most common extra-glandular manifestation in patients with primary Sjögren's syndrome (pSS), leading to a worsening of the patient's prognosis. To date, different studies have assessed the prevalence of pulmonary involvement and interstitial lung disease (ILD) in pSS patients with different results. Methods: We performed a systematic literature review and meta-analysis on ILD pooled prevalence in pSS according to the PRISMA and MOOSE guidelines. Furthermore, we explored the pooled prevalence of the two main presentations of pSS-ILD, nonspecific interstitial pneumonia (NSIP) and usual interstitial pneumonia (UIP). Results: We analysed the pSS-ILD prevalence in 30 studies including 8255 pSS patients. The pSS-ILD pooled prevalence was 23% (95% CI: 16–30). For NSIP, we found a pooled prevalence of 52% (CI 41–64), and for UIP we found a pooled prevalence of 44% (CI: 32–55). Regarding the meta-regression analysis, male gender, DLco value, country, and HRCT seem to contribute to the ILD presence. Conclusions: At least 20% of pSS patients have a comorbid ILD, usually NSIP. Male gender and alteration in DLco value may be considered the most important independent factors supporting an active search of lung complications during the clinical history of pSS patients. [ABSTRACT FROM AUTHOR]

No abstract available. Article truncated after 150 words. History of Present Illness A 32-year-old woman with no known past medical history presented with progressive shortness of breath for 2 weeks. She denied having a cough, fever, or chills, but she did have a one-month history of fatigue, weakness, and painful rashes on her hands. PMH, SH, and FH • No known past medical history • Former tobacco user (quit 2 years prior to admission) • No drug use • Worked as an office assistant • Has two pet dogs and four pet macaws • No family history of lung disease • Not taking any prescription medications Physical Exam • BP: 116/65, Pulse: 105, T: 37°C, RR: 28, SpO2: 89% on HHFNC (60L; 100%) • Pulmonary: Tachypneic, in respiratory distress, crackles throughout • Cardiovascular: Tachycardic but regular, no murmurs • Extremities: No edema • Skin: Palms with purplish discoloration and erythematous papules Which of the following should be done next? 1. CT Chest 2. COVID-19 testing 3. Sputum gram stain and …

Abstract Background Heme oxygenase-1 (HMOX1) catalyzes the metabolism of heme into carbon monoxide, ferrous iron, and biliverdin. Through biliverdin reductase, biliverdin becomes bilirubin. HMOX1-deficiency is a rare autosomal recessive disorder with hallmark features of direct antibody negative hemolytic anemia with normal bilirubin, hyperinflammation and features similar to macrophage activation syndrome. Clinical findings have included asplenia, nephritis, hepatitis, and vasculitis. Pulmonary features and evaluation of the immune response have been limited. Case presentation We present a young boy who presented with chronic respiratory failure due to nonspecific interstitial pneumonia following a chronic history of infection-triggered recurrent hyperinflammatory flares. Episodes included hemolysis without hyperbilirubinemia, immunodeficiency, hepatomegaly with mild transaminitis, asplenia, leukocytosis, thrombocytosis, joint pain and features of macrophage activation with negative autoimmune serologies. Lung biopsy revealed cholesterol granulomas. He was found post-mortem by whole exome sequencing to have a compound heterozygous paternal frame shift a paternal frame shift HMOX1 c.264_269delCTGG (p.L89Sfs*24) and maternal splice donor HMOX1 (c.636 + 2 T > A) consistent with HMOX1 deficiency. Western blot analysis confirmed lack of HMOX1 protein upon oxidant stimulation of the patient cells. Conclusions Here, we describe a phenotype expansion for HMOX1-deficiency to include not only asplenia and hepatomegaly, but also interstitial lung disease with cholesterol granulomas and inflammatory flares with hemophagocytosis present in the bone marrow.

Within the Interstitial Lung Diseases (ILD), patients with idiopathic pulmonary fibrosis (IPF) and a subset of those with non-IPF fibrotic ILD have a distinct clinical phenotype of progression despite management. This group of patients has been collectively termed the progressive fibrotic phenotype (PFP). Their early recognition may facilitate access to antifibrotic therapies to prevent or slow progression. Macrophages/monocytes within the lung orchestrate the progression and maintenance of fibrosis. A novel role for monocyte-derived macrophages during tissue damage and wound healing is the expression of collagens. We examined Collagen 1a1 expression in airway macrophages from ILD patients at diagnosis. COL1A1 mRNA levels from BAL cells were elevated in IPF and Non-IPF patients. The presence of a UIP pattern and a subsequent progressive phenotype were significantly associated with the higher BAL COL1A1 levels. In Non-IPF patients, higher COL1A1 levels were associated with a more than twofold increase in mortality. The intracellular localisation of COL1A1 in airway macrophages was demonstrated by confocal microscopy in CD45 and CD163 co-staining assays. Additionally, airway macrophages co-expressed COL1A1 with the profibrotic SPP1 gene product osteopontin. The levels of SPP1 mRNA and OPN in the BAL were significantly higher in IPF and Non-IPF patients relative to healthy. Our results suggest that profibrotic airway macrophages are increased in the BAL of patients with IPF and other ILDs and co-express COL1A1 and OPN. Importantly, COL1A1 expression by pro-fibrotic airway macrophages could be a marker of disease progression and poor survival in ILDs. [ABSTRACT FROM AUTHOR]

Within the Interstitial Lung Diseases (ILD), patients with idiopathic pulmonary fibrosis (IPF) and a subset of those with non-IPF fibrotic ILD have a distinct clinical phenotype of progression despite management. This group of patients has been collectively termed the progressive fibrotic phenotype (PFP). Their early recognition may facilitate access to antifibrotic therapies to prevent or slow progression. Macrophages/monocytes within the lung orchestrate the progression and maintenance of fibrosis. A novel role for monocyte-derived macrophages during tissue damage and wound healing is the expression of collagens. We examined Collagen 1a1 expression in airway macrophages from ILD patients at diagnosis. COL1A1 mRNA levels from BAL cells were elevated in IPF and Non-IPF patients. The presence of a UIP pattern and a subsequent progressive phenotype were significantly associated with the higher BAL COL1A1 levels. In Non-IPF patients, higher COL1A1 levels were associated with a more than twofold increase in mortality. The intracellular localisation of COL1A1 in airway macrophages was demonstrated by confocal microscopy in CD45 and CD163 co-staining assays. Additionally, airway macrophages co-expressed COL1A1 with the profibrotic SPP1 gene product osteopontin. The levels of SPP1 mRNA and OPN in the BAL were significantly higher in IPF and Non-IPF patients relative to healthy. Our results suggest that profibrotic airway macrophages are increased in the BAL of patients with IPF and other ILDs and co-express COL1A1 and OPN. Importantly, COL1A1 expression by pro-fibrotic airway macrophages could be a marker of disease progression and poor survival in ILDs.

Introduction: The inflammation and fibrosis in diffuse parenchymal lung diseases (DPLDs) in varied proportions give rise to different patterns in radiology and histopathology. The radiological pattern on CT of the thorax most often allows us to make a diagnosis with varying levels of confidence, to optimize management. With a multidisciplinary team bringing the strengths of their individual domains of knowledge, clinical, radiological, histopathological, and in many cases rheumatological, the level of confidence in making this diagnosis increases, often to the stage where the diagnosis is most often right, is concordant with the diagnosis achieved at histopathology and therefore obviates the need for lung biopsy which carries its own costs and risks of complications. Our study emphasizes the role of the multidisciplinary team (MDT) in the management of DPLDs at a tertiary care referral center. Materials and Methods: Every case of DPLD presenting to our pulmonology department was discussed in an MDT meeting before subjecting them to any diagnostic intervention or therapy. A clinico‐radiological diagnosis was made according to the 2002 ATS/ERS guidelines initially. Later an official ATS/ERS/JRS/ALAT statement on idiopathic pulmonary fibrosis and a 2013 ATS/ERS consensus for the classification and diagnosis of idiopathic interstitial pneumonia was used. The concordance in our study was defined as the percentage of histopathological diagnoses that were identical to the clinico‐radiological MDT diagnosis prior to the biopsy. Results: A total of 434 patients with DPLDs were evaluated. The MDT suggested biopsy for only 38.7% (168/434) patients since the pattern was very clear in 266 (61.3%) cases. As not all patients consented to undergo the biopsy procedure when recommended, histopathology was obtained in 102 patients. The histological diagnosis was concordant with the initial MDT diagnosis in 80.3% (82/102) of samples. On an individual basis, connective tissue disease‐interstitial lung disease and sarcoidosis showed the best concordance (87%). In idiopathic non‐specific interstitial pneumonitis (NSIP) cases, the histopathological diagnosis concurred in only 53.3% (8/15), out of which 8 were NSIP, 4 were usual interstitial pneumonia, and 3 were reported as inadequate sampling on histopathology. Conclusion: The MDT plays a crucial role in the diagnosis of DPLDs. Not every pattern requires biopsy confirmation. However, an idiopathic non‐specific interstitial pneumonitis diagnosis by the MDT should probably be better confirmed by biopsy. [ABSTRACT FROM AUTHOR]

No abstract available. Article truncated after 150 words. History of Present Illness: The patient is a 53-year-old man who presented in January 2018 for a second opinion on interstitial lung disease first diagnosed in 2011. He lives in Los Angeles and had one year of increasing dyspnea on exertion prior to diagnosis. He had an outside surgical lung biopsy and was treated with prednisone, then started on azathioprine and the prednisone tapered. He was followed regularly and had limited progression over next 7 years. However, recently he had increasing shortness of breath. Past Medical History, Social History, Family History: He has no significant past medical history. He is a nonsmoker and denies any significant occupational exposures. Physical Examination: Physical examination was unremarkable without rales or clubbing. Which of the following should be obtained at this time? 1. Prior chest x-rays, CT scans, pulmonary function testing and lung biopsy/2. Repeat CT scan, pulmonary function testing. 3. Rheumatological serologies …

The UK is currently facing unprecedented times as Covid-19 has forced the country into lockdown. However, the recent development consent application from EDF Energy for the Sizewell C Nuclear Power Station provides an opportunity for the planning sector to begin to return to normal. This opinion considers whether it is possible to achieve full public consultation on the Sizewell C Nuclear Power Station application, given the current circumstances that the UK faces due to Covid-19. It is argued that the Planning Inspectorate has not currently taken sufficient action to ensure that members of the public who do not have internet access and those who require library access to make representations are not left out of the process. It is also argued that businesses and non-governmental organisations may also struggle to make representations at a time when they are suffering from limited resources. In conclusion, it is essential that the Planning Inspectorate takes immediate action to ensure that the public consultation process is preserved for applications of this kind while Covid-19 restrictions are in place. [ABSTRACT FROM AUTHOR]

Background: Interstitial lung diseases (ILD) encompass various disorders characterized by inflammation and/or fibrosis in the lung interstitium. These conditions produce distinct patterns in High-Resolution Computed Tomography (HRCT)., Objective: We employ a deep learning method to diagnose the most commonly encountered patterns in ILD differentially., Materials and Methods: Patients were categorized into usual interstitial pneumonia (UIP), nonspecific interstitial pneumonia (NSIP), and normal lung parenchyma groups. VGG16 and VGG19 deep learning architectures were utilized. 85% of each pattern was used as training data for the artificial intelligence model. The models were then tasked with diagnosing the patterns in the test dataset without human intervention. Accuracy rates were calculated for both models., Results: 1 The success of the VGG16 model in the test phase was 95.02% accuracy. 2 Using the same data, 98.05% accuracy results were obtained in the test phase of the VGG19 model., Conclusion: Deep Learning models showed high accuracy in distinguishing the two most common patterns of ILD., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

Non-specific interstitial pneumonia (NSIP) is a form of idiopathic interstitial pneumonia with a specific histological pattern involving varying degrees of alveolar wall inflammation or fibrosis, and with a temporal uniformity of lesions. The organized component can be found at different degrees, and the presence of a histologically organized component does not exclude a diagnosis of NSIP. Acute respiratory failure is rare in NSIP. A female patient who presented to our clinic with radiological findings of widespread consolidation and acute respiratory failure was diagnosed with NSIP with organized pneumonia through a surgical lung biopsy. The patient responded rapidly to steroid therapy, and the clinical findings improved dramatically. We present our case in order to emphasize the importance of treatment in the event of respiratory failure in idiopathic NSIP with organized pneumonia, and recommend rapid diagnosis and treatment. [ABSTRACT FROM AUTHOR]

No abstract available. Article truncated at 150 words. Chief Complaint: Difficulty breathing History of Present Illness A 49-year-old gentleman with history of hepatitis C cirrhosis complicated by ascites presented to the emergency room of Olive View Medical Center in San Fernando Valley, California complaining of worsening shortness of breath. The patient reports that he occasionally has shortness of breath, usually about 2-3 times a year. However for the past 2 months, he has had worsening dyspnea on exertion and cannot walk further than 5 minutes. He also reports orthopnea and paroxysmal nocturnal dyspnea. He has been having a dry cough for the past 3-4 weeks. He has a history of chronic ascites that has required multiple taps. He has been taking his prescribed diuretics however instead of taking these medications daily he takes them about every other day due to financial constraints. However, his abdominal distention and his lower extremity swelling are stable. He reports some nausea with …

A non-invasive test to facilitate the diagnosis of non-small cell lung cancer (NSCLC) and idiopathic pulmonary fibrosis (IPF) is still not available and represents an important goal. Forty-eight patients with stage I NSCLC, 45 with IPF, 30 with other idiopathic interstitial pneumonias (IIPs) including idiopathic non-specific interstitial pneumonia (NSIP) and chronic hypersensitivity pneumonitis (HP), 35 with diffuse non-malignant disease and 30 healthy donors were enrolled onto the study. Free circulating (fc)DNA and MMP-7 levels were evaluated by Real Time PCR and ELISA, respectively. Median fcDNA levels were similar in NSCLC (127 ng/mL, range 23.6–345 ng/mL) and IPF (106 ng/mL, range 22–224 ng/mL) patients, and significantly lower in IIPs patients, in individuals with other diseases and in healthy donors (p < 0.05). Conversely, median MMP-7 values were significantly higher in IPF patients (9.10 ng/mL, range 3.88–19.72 ng/mL) than in those with NSCLC (6.31 ng/mL, range 3.38–16.36 ng/mL; p < 0.0001), NSIP (6.50 ng/mL, range 1.50–22.47 ng/mL; p = 0.007), other diseases (5.41 ng/mL, range 1.78–15.91, p < 0.0001) or healthy donors (4.35 ng/mL, range 2.45–7.23; p < 0.0001). Serum MMP-7 levels seem to be capable of distinguishing IPF patients from those with any other lung disease. fcDNA levels were similar in NSCLC and IPF patients, confirming its potential role as a biomarker, albeit non-specific, for the differential diagnosis of NSCLC.

Background Interstitial lung diseases (ILDs) are a heterogeneous group of disorders characterized by inflammation and/or fibrosis of the lungs, varying from idiopathic interstitial pneumonias to secondary variants, including the ILDs associated to connective tissue diseases (CTDs). In addition, a number of patients are recognized as unclassifiable ILD (U-ILD), because of the inability to reach a definite diagnosis; some of them show autoimmune manifestations not fulfilling the classification criteria of a given CTD. The term interstitial pneumonia with autoimmune features (IPAF) has been recently proposed for this particular ILD subset. Methods Here, we report our experience resulting from the integrated — pneumology/rheumatology — approach to patients with suspected ILDs or CTDs referred to our university-based Center for the Rare Pulmonary Diseases and Rheumatology Unit, from January 2009 to June 2015, with particular attention to the above-mentioned U-ILD, IPAF, and undifferentiated connective tissue disease (UCTD). The comparative analysis of these clinical variants was carried out; moreover, the observed findings were compared with the results of the updated review of the literature. Results After the first clinical assessment, the U-ILD were identified in 50 patients; afterwards, on the basis of clinico-serological and radiological findings U-ILD group was subdivided into 2 subgroups, namely U-ILD without any clinical extra-thoracic manifestations and/or immunological alterations (15 pts) and IPAF according to the above-mentioned classification criteria (35 pts). Patients with either IPAF or U-ILD were compared with a series of 52 stable UCTD (disease duration ≥ 3 years), followed at our Rheumatology Unit. Some important differences were evidenced among the 3 series of U-ILD, IPAF, and UCTD: firstly, female gender was more frequent in patients with UCTD (86%) or IPAF (69%) compared with U-ILD (60%) or idiopathic pulmonary fibrosis (24%; p = 0.001). In addition, UCTD patients were younger and showed longer disease duration. More interestingly, both UCTD and IPAF series show a comparable prevalence of various clinical manifestations, with the exception of the interstitial lung involvement detectable in a very small percentage of UCTD patients. Concordantly, the review of the literature evidenced two main subsets of U-ILD, one is characterized by isolated unclassifiable interstitial pneumonia and another one composed by subjects with clinically prevalent lung involvement in the setting of not definite CTD, the recently proposed IPAF. Conclusion We hypothesize that IPAF and UCTD might represent two clinical variants of the same systemic autoimmune disorders. The marked difference regarding the prevalence of ILD, which is the clinical hallmark of IPAF but very rare in UCTD, may at least in part reflect a selection bias of patients generally referred to different specialist centers, i.e. pneumology or rheumatology, according to the presence/absence of clinically dominant ILD, respectively. Well-integrated, interdisciplinary teams are recommended for the assessment and management of these patients in the clinical practice. Finally, the cooperation between multidisciplinary groups with different experiences may be advisable for a validation study of the proposed nomenclature and classification criteria of these indefinable ILD/CTD variants. [ABSTRACT FROM AUTHOR]

The creation of a biobank for Interstitial Lung Diseases (ILDs), according to the standards set by the European Idiopathic Pulmonary Fibrosis registry (eurIPFreg) and Biobanking and Biomolecular Research Infrastructure (BBMRI) is a major step in the understanding of these highly complex diseases. Particular emphasis is placed on Idiopathic Interstitial Pneumonias and especially Idiopathic Pulmonary Fibrosis (IPF), a chronic and progressive fibrotic interstitial pneumonia, with an average life span of 3 years. Our aim is to present a methodological guide for the development of a biobank for ILDs. [ABSTRACT FROM AUTHOR]

A non-invasive test to facilitate the diagnosis of non-small cell lung cancer (NSCLC) and idiopathic pulmonary fibrosis (IPF) is still not available and represents an important goal. Forty-eight patients with stage I NSCLC, 45 with IPF, 30 with other idiopathic interstitial pneumonias (IIPs) including idiopathic non-specific interstitial pneumonia (NSIP) and chronic hypersensitivity pneumonitis (HP), 35 with diffuse non-malignant disease and 30 healthy donors were enrolled onto the study. Free circulating (fc)DNA and MMP-7 levels were evaluated by Real Time PCR and ELISA, respectively. Median fcDNA levels were similar in NSCLC (127 ng/mL, range 23.6-345 ng/mL) and IPF (106 ng/mL, range 22-224 ng/mL) patients, and significantly lower in IIPs patients, in individuals with other diseases and in healthy donors (p < 0.05). Conversely, median MMP-7 values were significantly higher in IPF patients (9.10 ng/mL, range 3.88-19.72 ng/mL) than in those with NSCLC (6.31 ng/mL, range 3.38-16.36 ng/mL; p < 0.0001), NSIP (6.50 ng/mL, range 1.50-22.47 ng/mL; p = 0.007), other diseases (5.41 ng/mL, range 1.78-15.91, p < 0.0001) or healthy donors (4.35 ng/mL, range 2.45-7.23; p < 0.0001). Serum MMP-7 levels seem to be capable of distinguishing IPF patients from those with any other lung disease fcDNA levels were similar in NSCLC and IPF patients, confirming its potential role as a biomarker, albeit non-specific, for the differential diagnosis of NSCLC. [ABSTRACT FROM AUTHOR]

Objective: To report the case of a morbidly obese 49-year-old woman with nonspecific interstitial pneumonia who underwent bariatric surgery. Because of inadequate weight loss after sleeve gastrectomy, duodenal switch as component of a stepwise treatment was performed and led to unexplained progressive weight loss and malnutrition. Method: Case report. Results: After duodenal switch surgery, the patient presented with late postsurgical symptoms of malabsorption. Postsurgical psychological evaluation revealed a persistent binge eating disorder. Along with exocrine pancreatic insufficiency, binge eating had led to progressive weight loss of ≈100 kg from a body mass index of 50.3 kg/m2 presurgery to 17.3 kg/m2 postsurgery. Discussion: Recent research has focused on eating patterns after bariatric surgery and the risks of exacerbating eating disorders after surgery. This case study illustrates the need for auxiliary prepsychotherapeutic and postpsychotherapeutic evaluation and subsequent support for patients with eating disorders preparing for bariatric surgery. © 2012 by Wiley Periodicals, Inc. (Int J Eat Disord 2013) [ABSTRACT FROM AUTHOR]

Interstitial lung disease (ILD) is the most frequent organ involvement (found in nearly half) of myositis patients, but it reveals various clinical courses and therapeutic responsiveness according to clinical and serological subsets. Autoantibodies, as well as imaging and histopathological studies, are useful for the classification of ILD in myositis and provide useful information for predicting prognosis and determining treatment. Antisynthetase antibodies are correlated with chronic and recurrent ILD, whereas anti-CADM-140 (MDA5/IFIH1) antibodies are a marker of acute progressive ILD in clinically amyopathic dermatomyositis. Serum KL-6, SP-D, and ferritin are useful biomarkers for monitoring the activity and severity of ILD. Regarding treatment, glucocorticoids are the first-line drug, but additional immunomodulating drugs are also used in refractory patients. Cyclophosphamide and calcineurin inhibitors (cyclosporine and tacrolimus) appear to be the key drugs in the treatment of refractory myositis-ILD. Rituximab may become another candidate if these drugs are not effective. [ABSTRACT FROM AUTHOR]

Abstract: ATS/ERS evaluation of ILD’s has recently considered NSIP as a single entity and it has historically been considered a provisional diagnosis. As more cases are reviewed, pathologic characteristics may become more precise with less overlap and help in diagnosis of complex cases. Here, we present a series of cases of HP and NSIP recently admitted to Masih Daneshvari Hospital with hope to characterize them better and eventually have less ambiguity about nature of NSIP. [Copyright &y& Elsevier]

Mazur W, Lindholm P, Vuorinen K, Myllärniemi M, Salmenkivi K, Kinnula VL. Cell-specific elevation of NRF2 and sulfiredoxin-1 as markers of oxidative stress in the lungs of idiopathic pulmonary fibrosis and non-specific interstitial pneumonia. APMIS 2010; 118: 703–12. Human idiopathic pulmonary fibrosis (IPF) and non-specific interstitial pneumonia (NSIP) have been proposed to be attributable to oxidative stress. The nuclear factor, erythroid derived 2, like protein (NRF2)–sulfiredoxin-1 (SRX1) pathway was hypothesized to be associated with the pathogenesis of human pulmonary fibrosis. Several methods including digital morphometry were used in the assessment of the cell-specific localization and expression of NRF2 and SRX1 and selected proteins linked to their activation/stability in human IPF/usual interstitial pneumonia (UIP) and NSIP lung. The proteins of the NRF2 pathway were localized in the hyperplastic alveolar epithelium and inflammatory cells in IPF and NSIP, but were absent in the fibroblastic foci characteristic of IPF. Morphometric evaluation revealed NRF2 and KEAP1 to be significantly elevated in the hyperplastic alveolar epithelium compared with the normal alveolar epithelium, and NRF2 was remarkably expressed in the nuclear compartment of the hyperplastic cells. SRX1 was expressed mainly in alveolar macrophages, and the number of SRX1-positive macrophages/surface area was elevated in NSIP, a disease which contains more marked inflammatory reaction compared with the IPF/UIP lung. The expression of the NRF2 pathway in human IPF and NSIP is further evidence that the pathogenesis of human fibrotic lung diseases is oxidant-mediated and originates from the alveolar epithelium. [ABSTRACT FROM AUTHOR]

Summary: Usual interstitial pneumonia is an almost uniformly fatal form of fibrosing interstitial lung disease. It is the most common idiopathic interstitial pneumonia, and currently, there is no effective therapy. Lung biopsy is often needed for diagnosis, and pathologists must be able to recognize its features and distinguish it from other interstitial lung diseases that have a better prognosis and a more favorable response to therapy. This review is an attempt to clarify the diagnostic pathologic features of usual interstitial pneumonia and to provide guidelines for its distinction from other interstitial lung diseases that enter the differential diagnosis. [Copyright &y& Elsevier]

Summary: Usual interstitial pneumonia is an almost uniformly fatal form of fibrosing interstitial lung disease. It is the most common idiopathic interstitial pneumonia, and currently, there is no effective therapy. Lung biopsy is often needed for diagnosis, and pathologists must be able to recognize its features and distinguish it from other interstitial lung diseases that have a better prognosis and a more favorable response to therapy. This review is an attempt to clarify the diagnostic pathologic features of usual interstitial pneumonia and to provide guidelines for its distinction from other interstitial lung diseases that enter the differential diagnosis. [Copyright &y& Elsevier]

Among idiopathic interstitial pneumonias (IIP), idiopathic pulmonary fibrosis (IPF) is the most common form with an incidence of more than 10 cases per 100,000 population, increasing with age. The prognosis is poor and comparable to many malignant diseases. The time course is characterized by progressive dyspnoea and a decline of pulmonary function, often complicated by acute exacerbations or the development of pulmonary hypertension. IPF is considered to be a result of an abnormal alveolar wound repair process after repeated epithelial microinjuries by so far unknown agents, rather than a primary inflammatory disorder. The diagnosis is based on clinical symptoms and findings like crackles over the basal areas of the lung, impairment of pulmonary function (restriction, reduced diffusion capacity, hypoxemia), and the absence of an alternative diagnosis. High resolution computed tomography (HRCT) has been established as a powerful tool that allows the diagnosis of IPF in experienced centres without the performance of a surgical lung biopsy. The characteristic histopathological pattern is usual interstitial pneumonia (UIP) showing a typical heterogenous pattern and fibroblast foci. Faced with a poor prognosis and the lack of an effective treatment, it is very important to distinguish IPF from other interstitial lung diseases like other IIP (in particular non-specific interstitial pneumonia NSIP), drug-induced pulmonary fibrosis, asbestosis, extrinsic allergic alveolitis, sarcoidosis or diffuse parenchymal lung diseases associated with connective tissue diseases. Currently, no effective therapy is available and anti-inflammatory treatment is of limited value. However, new insights into the pathogenesis of IPF opened up novel therapeutic options, which are currently investigated in clinical trials. [ABSTRACT FROM AUTHOR]

Summary: Little is known about the role of chemokines and their receptors interaction, which are essential for recruitment of selective lymphocyte subsets during inflammation, in the pathogenesis of idiopathic nonspecific interstitial pneumonia (NSIP). Recent studies have revealed Th1 and Th2 cells preferentially employ the chemokine receptors, CXCR3 and CCR4, respectively, in the process of accumulation into inflammatory sites. We evaluated the CXCR3 and CCR4 expression on infiltrated lymphocytes in lung tissues of 12 NSIP cases and 10 idiopathic pulmonary fibrosis (IPF) cases in our previous study. The number of CXCR3 positive lymphocytes of NSIP patients was significantly greater than that of IPF patients (261.1±145.1 vs. 64.9±27.0, P<0.01). The number of CCR4 positive lymphocytes of NSIP patients was significantly lower than that of IPF (9.5±8.3 vs.62.6±26.9, P<0.01). The CXCR3 to CCR4 ratio of NSIP patients was significantly greater than that of IPF patients (47.9±45.9 vs. 1.11±0.40, P<0.01). The differences of CXCR3 positive, CCR4 positive lymphocyte counts, and of CXCR3/CCR4 ratio between cellular and fibrosing NSIP were not significant. These results suggest that a Th1 pattern of chemokine receptor expression predominates in the lung interstitium of patients with NSIP but, in IPF patients, CCR4 might be relatively predominant, in contrast to the finding in NSIP patients, and that Th1/Th2 balance might be an important factor in the pathogenesis of NSIP. [Copyright &y& Elsevier]

Background: Usual interstitial pneumonia (UIP) and nonspecific interstitial pneumonia (NSIP) are common forms of interstitial lung disease (ILD). Recent studies have identified potential links between UIP and senescence which is a form of irreversible cell cycle arrest that can be caused by DNA damage, shortened telomeres or oncogene activation. Senescent fibroblasts express p16 and can develop a senescence-associated secretory phenotype (SASP) to produce profibrotic growth factors and matrix remodeling proteins. Design: Gene expression from explanted lungs of patients with IPF (n=22), NSIP (n=10), mixed IPF-NSIP (n=5), and normal controls (n=11) was assessed. Immunohistochemistry for p16, p53, Periostin and IDO1 was performed on surgical lung biopsies from 22 UIP, 9 NSIP and 5 mixed cases. Results: UIP cases showed a unique expression pattern with an increased expression of ACTA-2, MUC5B and IGFBP-5 compared to the NSIP cases. Immunohistochemistry identified p16 positive fibroblastic foci in 15 of 23 (65%) of UIP cases, 0 of 9 (0%) NSIP cases and 2 of 5 (40%) mixed cases. P16 fibroblastic foci were also positive for p53 by immunohistochemistry. No difference in Periostin or IDO1 was identified by immunohistochemistry. Conclusion: UIP cases expressed a distinct subset of growth factors and matrix remodeling genes. P16 positive fibroblastic foci were identified in the majority of UIP cases and highlights a potential important etiological basis for senescence in UIP and supports the ongoing development of senolytic therapies to disrupt this process. Further, there is a potential use for p16 as an adjunct marker to distinguish UIP from NSIP. [ABSTRACT FROM AUTHOR]