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Sarcoidosis Vasc Diffuse Lung Dis 2019 1;36(4):294-301. Epub 2019 May 1.

Health Science University Dr.Suat Seren Chest Diseases and Surgery Research and Training Hospital.

Background And Aim: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive interstitial lung disease (ILD) with unknown etiology that occurs primarily in older adults with a median survival time of 2.5±3.5 years. Read More

Sarcoidosis Vasc Diffuse Lung Dis 2019 1;36(2):148-156. Epub 2019 May 1.

Department of Respiratory Medicine.

Background: When a clinical context is indeterminate for idiopathic pulmonary fibrosis (IPF), or a chest high-resolution computed tomography (HRCT) pattern is not indicative of typical or probable usual interstitial pneumonia (UIP) in patients with interstitial lung disease (ILD), surgical lung biopsy should be considered to make a confident diagnosis on the basis of multidisciplinary diagnosis (MDD).

Aim: The aim of this study was to evaluate the role and safety of video-assisted thoracoscopic surgery (VATS) in patients with ILD.

Methods: A total of 143 patients with ILD underwent VATS at Toho University Medical Center Omori Hospital between March 2004 and April 2017. Read More

Sarcoidosis Vasc Diffuse Lung Dis 2019 1;36(2):116-123. Epub 2019 May 1.

Department of Clinical Epidemiology and Health Economics, School of Public Health, The University of Tokyo, Tokyo, Japan.

Purpose: Mortality of acute exacerbation of idiopathic pulmonary fibrosis is high, and it remains unknown whether cyclophosphamide is an effective treatment for this condition.

Objectives: This study compared the effects of cyclophosphamide combined with systemic glucocorticoids with those of systemic glucocorticoids alone.

Methods: Using the Diagnosis Procedure Combination database in Japan, adult patients with idiopathic pulmonary fibrosis who had received high-dose methylprednisolone and mechanical ventilation at admission from July 1, 2010, to March 31, 2014, were identified. Read More

Sarcoidosis Vasc Diffuse Lung Dis 2019 1;36(2):108-115. Epub 2019 May 1.

Pneumology department, Cliniques universitaires Saint-Luc, Bruxelles, Belgium.

Background: The guidelines on idiopathic pulmonary fibrosis (IPF) diagnosis established the crucial role of multidisciplinary discussion (MDD) in the diagnosis of interstitial lung diseases (ILD). However, real-life evaluation of MDD remains scarce. Our aim was to study the impact of a well-structured MDD on etiological assessment, diagnosis, and management of ILD. Read More

Sarcoidosis Vasc Diffuse Lung Dis 2019 1;36(2):92-107. Epub 2019 May 1.

Department of Medicine, Section of Pulmonary and Critical Care Medicine, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin, United States.

Although relatively few patients with pulmonary sarcoidosis develop advanced disease that progresses to respiratory insufficiency despite receiving best practice pharmacologic interventions, lung transplantation may be the only therapeutic option for such patients to both prolong survival and provide improved quality of life. Lung transplant can be successfully performed for patients with end-stage pulmonary sarcoidosis, and post-transplant survival is similar to that for other transplant indications such as idiopathic pulmonary fibrosis. However, appropriate timing of referral, comprehensive assessment of potential candidates for lung transplant, placement of patients on the lung transplant waiting list when within the transplant window as appropriate, choosing the best procedure (bilateral versus single lung transplant), and optimal peri-operative and post-transplant management are key to successful lung transplant outcomes for patients with sarcoidosis. Read More

Sarcoidosis Vasc Diffuse Lung Dis 2019 1;36(1):74-78. Epub 2019 May 1.

Department of Pulmonary Medicine, Sendai Kousei Hospital, 4-15, Hirosemachi, Aoba-ku, Sendai, Miyagi 980-0873, Japan.

Background: We have often encountered adverse events requiring dose reduction and/or discontinuation of nintedanib in patients with idiopathic pulmonary fibrosis.

Objectives: The objectives of this study were to clarify the incidence of dose reduction and/or discontinuation following the commercialization of nintedanib and to investigate predictors of dose reduction and/or discontinuation of nintedanib at our hospital.

Methods: We retrospectively identified 25 patients who had received nintedanib 150 mg twice daily at Sendai Kousei Hospital and categorized them into two groups according to whether they had or had not required dose reduction and/or discontinuation and sought to identify predictors of dose reduction and/or discontinuation. Read More

Sarcoidosis Vasc Diffuse Lung Dis 2019 1;36(1):39-46. Epub 2019 May 1.

Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center.

Background: In 2016, the diagnostic criteria for the acute exacerbation (AE) of idiopathic pulmonary fibrosis (IPF) were revised. However, there have been published few clinical reports on AE-IPF published using the new criteria. The aim of this study was to investigate the incidence of, risk factors for, and mortality due to newly defined AE. Read More

Sarcoidosis Vasc Diffuse Lung Dis 2018 9;35(4):363-370. Epub 2020 Mar 9.

Department of Pulmonary Medicine, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.

Acute respiratory failure (ARF) due to diffuse parenchymal lung diseases (DPLDs) is associated with high mortality. Whether ARF due to acute interstitial pneumonia (AIP), idiopathic pulmonary fibrosis (IPF) and non-IPF DPLDs behaves differently remains unclear. A retrospective analysis of consecutive DPLD subjects with ARF admitted to respiratory intensive care unit (RICU). Read More

Sarcoidosis Vasc Diffuse Lung Dis 2018 9;35(4):317-326. Epub 2020 Mar 9.

Interstitial Lung Diseases Center of Excellence, Department of Pulmonology, St Antonius Hospital, Nieuwegein, The Netherlands.

Patients with pulmonary fibrosis (PF) have a clear exercise intolerance. The 4-meter-gait-speed (4MGS) test and the 5-repetitions-sit-to-stand (5STS) test are easy, inexpensive and reliable measures of functional performance. Both tests have been validated in healthy adults and patients with chronic obstructive pulmonary disease. Read More

Sarcoidosis Vasc Diffuse Lung Dis 2018 9;35(4):299-307. Epub 2020 Mar 9.

Dr. Suat Seren Chest Diseases and Surgery Training and Research Hospital, Izmir, Turkey.

Exercise training have been shown to be the effective approach for functional outcomes in interstitial lung diseases (ILD). In many studies, the duration of exercise programs (EPs) varies between 8-12 weeks. However, the optimal duration of EPs is still unknown. Read More

Sarcoidosis Vasc Diffuse Lung Dis 2018 28;35(3):268-275. Epub 2018 Apr 28.

Institute of Immunology, Comenius University Faculty of Medicine, Bratislava, Slovakia.

Analysis of new markers in bronchoalveolar lavage fluid (BALF) provides new insights into the immunopathogenesis and may be helpful in differential diagnosis of lung diseases. High mobility group box 1 protein (HMGB1) is a non-histone nuclear protein and its release into the extracellular environment may be associated with the inflammatory response. The aim of the study is the analysis of HMGB1 in BALF, correlations with other markers of inflammation and differences in extracellular HMGB1 levels in various lung diagnoses. Read More

Sarcoidosis Vasc Diffuse Lung Dis 2018 28;35(2):139-142. Epub 2018 Apr 28.

Department of Respiratory Medicine, Tokyo Medical and Dental University, Tokyo, Japan.

Pirfenidone is an antifibrotic drug used for the treatment of idiopathic pulmonary fibrosis. The aim of this study was to evaluate the efficacy of pirfenidone in patients with chronic hypersensitivity pneumonitis (HP). Twenty-three patients with chronic HP treated with pirfenidone were enrolled in this study based on a retrospective medical record review. Read More

Sarcoidosis Vasc Diffuse Lung Dis 2018 28;35(2):109-114. Epub 2018 Apr 28.

Division of Rheumatology, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA.

Recent studies have suggested that patients with idiopathic pulmonary fibrosis (IPF) may have a higher risk of venous thromboembolism (VTE) compared to general population even though the results were inconsistent. To investigate the risk of VTE among patients with IPF. Comprehensive literature review using MEDLINE and EMBASE database were performed to identify studies that compared the risk of VTE among patients with IPF to general population. Read More

Sarcoidosis Vasc Diffuse Lung Dis 2018 28;35(1):95-96. Epub 2018 Apr 28.

Department of Respiratory Medicine, Shanghai Pulmonary Hospital, Tongji University, School of Medicine, Shanghai, China.

Sarcoidosis Vasc Diffuse Lung Dis 2018 28;35(1):35-43. Epub 2018 Apr 28.

Allergy and Immunology Unit, Istituti Clinici Scientifici Maugeri IRCCS, Pavia, Italy.

Idiopathic pulmonary fibrosis (IPF) is a chronic and irreversible interstitial lung disease whose diagnosis often requires surgical lung biopsies (SLB) in cases without consistent radiological findings. We previously published that the expression of the chemokine receptors CXCR3 and CCR4 on T cells is significantly different in bronchoalveolar lavage (BAL) of IPF patients from other interstitial lung diseases. The aim of the study was to evaluate cut-off values of CXCR3 and CCR4 receptors expressed on bronchoalveolar lavage (BAL) and peripheral blood (PB) T cells useful for a differential diagnosis. Read More

Sarcoidosis Vasc Diffuse Lung Dis 2018 28;35(1):16-20. Epub 2018 Apr 28.

Center for Thoracic Surgery, Department of Medicine and Surgery, University of Insubria, Ospedale di Circolo, Varese, Italy.

The Quantitative chest CT (QCT) is emerging as a promising tool in the assessment of interstitial lung disease (ILD). However, the precise relationship between QCT parameters and the fibrosis detectable in lung tissue, remains to be established. The aim of this study was to compare QCT and histopathological features in patients with ILD. Read More

Sarcoidosis Vasc Diffuse Lung Dis 2017 28;34(4):326-335. Epub 2017 Apr 28.

Department of Pneumology, University Hospitals Leuven, Leuven, Belgium.

Interstitial lung disease (ILD) can be either idiopathic, the result of exposure or may be associated with extrapulmonary diseases. Among the latter, connective tissue diseases (CTDs) make up the largest part. The identification, follow-up and treatment of CTD-associated ILD (CTD-ILD) are a challenge for every physician as ILD can occur before, during and after the diagnosis of CTD. Read More

Sarcoidosis Vasc Diffuse Lung Dis 2017 28;34(4):300-306. Epub 2017 Apr 28.

Department of Diagnostic Imaging, Molecular Imaging, Interventional Radiology and Radiotherapy, University of Rome "Tor Vergata", Rome, Italy.

Fibrotic interstitial lung diseases (ILDs) include a number of pulmonary disorders characterized by infiltration of inflammatory cells in lung parenchyma and fibrosis resulting in decreased lung compliance. Idiopathic pulmonary fibrosis (IPF) represents the most common ILD. ILDs can be divided in two anatomo-pathological and radiographic patterns: usual interstitial pneumonitis (UIP) and non-specific interstitial pneumonitis (NSIP). Read More

Sarcoidosis Vasc Diffuse Lung Dis 2017 28;34(4):290-299. Epub 2017 Apr 28.

Chiba University, Graduate School of Medicine, Department of Respirology, Inohana, Chuo, Chiba, Chiba, Japan.

Pirfenidone is one of the anti-fibrotic drugs used for patients with idiopathic pulmonary fibrosis. Pirfenidone exerts anti-inflammatory effects by inhibiting the influx of inflammatory cells. The purpose of this study was to clarify the differences in the baseline parameters in responsive and unresponsive patients, and to assess the clinical and radiological changes after pirfenidone therapy. Read More

Sarcoidosis Vasc Diffuse Lung Dis 2017 28;34(4):283-289. Epub 2017 Apr 28.

Department of Cardiopulmonary Rehabilitation Science, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Nagasaki, Japan.

While the efficacy of pulmonary rehabilitation (PR) in chronic obstructive pulmonary disease (COPD) has been well established, emerging evidence also suggests its benefit in idiopathic pulmonary fibrosis (IPF). However, the differences and similarities between how PR affects diseases with different physiologies remain unknown. This study aimed to compare the efficacy of PR in COPD and IPF patients by performing multifactorial evaluation with various exercise capacity measurements, and dyspnea and health-related quality of life (QoL) assessment. Read More

Sarcoidosis Vasc Diffuse Lung Dis 2017 9;34(3):251-256. Epub 2020 Mar 9.

University Hospitals Leuven, Respiratory Medicine, Leuven, BE.

Idiopathic dendriform diffuse pulmonary ossification (DPO) is a rare disorder. High resolution CT (HRCT) with appropriate osteoporosis window setting reveals the diagnosis. We report the features of eight patients, of whom two brothers, with HRCT findings compatible with predominant DPO in a bibasal subpleural distribution (usual interstitial pneumonia (UIP)-like distribution) and review the literature for DPO in this UIP-like distribution. Read More

Sarcoidosis Vasc Diffuse Lung Dis 2017 9;34(3):226-235. Epub 2020 Mar 9.

Respiratory Medicine, Graduate School of Medicine, Kyoto University, Kyoto, Japan.

Generally, a disease-specific health-related quality of life (HRQOL) measurement is more useful than generic measures in assessing perceived physical and mental health characteristic of a particular disease. The idiopathic pulmonary fibrosis (IPF)-specific version of St. George's Respiratory Questionnaire (SGRQ-I) has been recently developed for patients with IPF. Read More

Sarcoidosis Vasc Diffuse Lung Dis 2017 9;34(3):217-225. Epub 2020 Mar 9.

ild care foundation research team, The Netherlands.

Health care management has to be based on the local prevalence of diseases and the local diagnostic and therapeutic needs. So far, no systematic registration system for various lung diseases exists on Aruba. Questions that need to be answered are: what specific lung disorders occur on Aruba, and what are the specific needs there? The aim of this study was to assess the prevalence of lung disorders and the diversity of the patient population. Read More

Sarcoidosis Vasc Diffuse Lung Dis 2017 28;34(2):149-155. Epub 2017 Apr 28.

Clinica Pneumologica, Azienda Ospedaliera San Gerardo, School of Medicine and Surgery, Università degli Studi Milano-Bicocca, Monza, Italy.

Chronic hypersensitivity pneumonitis (HP), in its progressive fibrotic form, is difficult to distinguish from other fibrosing interstitial lung diseases (ILD), particularly idiopathic pulmonary fibrosis (IPF) and non-specific interstitial pneumonia (NSIP). The role of serum precipitating antibodies in the diagnosis of fibrosing ILD has not been discussed in recent clinical practice guidelines. The aim of this study is to assess the role of precipitins in the diagnosis of non pre-selected cases of fibrosing ILD. Read More

Sarcoidosis Vasc Diffuse Lung Dis 2017 28;34(1):35-40. Epub 2017 Apr 28.

Department of Respiratory Medicine, Nippon Medical School.

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) was recently proposed as an entity to be included among rare idiopathic interstitial pneumonias (IIPs). However, the cause, clinical features and prognosis of this rare entity have not been elucidated. We aimed to examine the clinical features, outcomes and prognostic factors for IPPFE in comparison to those of idiopathic pulmonary fibrosis (IPF). Read More

Sarcoidosis Vasc Diffuse Lung Dis 2020 1;36(3):195-201. Epub 2020 May 1.

Division of Pulmonary and Critical Care Medicine.

Background: The association between interstitial lung disease (ILD) and myeloperoxidase (MPO)-antineutrophil cytoplasmic antibodies (ANCAs) has been described, but pathologic characteristics are not well characterized.

Objectives: We assessed the radiologic and pathologic characteristics of ILD in MPO-ANCA-positive patients and the association between ILD and vasculitis, particularly microscopic polyangiitis (MPA).

Methods: We retrospectively searched electronic health records to identify MPO-ANCA-positive patients with ILD who underwent surgical lung biopsy at our institution from January 1997 through August 2017. Read More

Sarcoidosis Vasc Diffuse Lung Dis 2020 1;36(3):176-184. Epub 2020 May 1.

Division of Respiratory Medicine, Toho University Omori Medical Center.

Background: Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is often fatal. Although pharmacological treatments have been studied, outcomes remain poor. This study evaluated the effectiveness of pharmacological treatments for AE-IPF. Read More

BMJ Open 2020 May 30;10(5):e034428. Epub 2020 May 30.

Observational and Pragmatic Research Institute Pte Ltd, Singapore

Objective: To explore the clinical pathways, including signs and symptoms, and symptom progression patterns preceding idiopathic pulmonary fibrosis (IPF) diagnosis.

Design And Setting: A historical cohort study was conducted using primary care patient records from the Optimum Patient Care Research Database.

Participants: Patients included were at least 30 years, had IPF diagnosis, identified via clinical-coding and free-text records and had a consultation with a chest specialist prior to IPF diagnosis. Read More

Transplant Proc 2020 May 27. Epub 2020 May 27.

Silesian Center for Heart Diseases, Zabrze, Poland.

Background: Lung transplant remains the only viable treatment for certain patients with end-stage lung diseases. Such patients can become either single or double lung recipients. The 2 procedures are associated with specific risks and benefits. Read More

Biochem Biophys Res Commun 2020 May 27. Epub 2020 May 27.

Department of Molecular Medicine, Research Institute for Frontier Medicine, Sapporo Medical University School of Medicine, Sapporo, Japan. Electronic address:

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive interstitial lung disease that includes fibroblastic foci (FF). It has been increasingly appreciated that the origin of collagen-overproducing cells such as pathological myofibroblasts in FF is pericytes. However, neither pericytes derived from the lung nor FF in the IPF lung have not been fully characterized. Read More

Med J Malaysia 2020 May;75(Suppl 1):10-13

Universitas Gadjah Mada, Public Health, and Nursing, Faculty of Medicine, Department of Pharmacology and Therapy, Jl. Farmako Sekip Utara, Yogyakarta, Indonesia.

Introduction: Diabetes mellitus is known as one of the risk factors for Idiopathic Pulmonary Fibrosis (IPF) development. Recently, metformin, the commonly used antidiabetic medication, is reported to have a therapeutic effect in IPF. However, the benefit of metformin therapy in IPF is still controversial. Read More

Respir Res 2020 May 29;21(1):132. Epub 2020 May 29.

Department of Physiological Sciences, Eastern Virginia Medical School, Norfolk, Virginia, USA.

Background: Chronic tissue injury was shown to induce progressive scarring in fibrotic diseases such as idiopathic pulmonary fibrosis (IPF), while an array of repair/regeneration and stress responses come to equilibrium to determine the outcome of injury at the organ level. In the lung, type I alveolar epithelial (ATI) cells constitute the epithelial barrier, while type II alveolar epithelial (ATII) cells play a pivotal role in regenerating the injured distal lungs. It had been demonstrated that eukaryotic cells possess repair machinery that can quickly patch the damaged plasma membrane after injury, and our previous studies discovered the membrane-mending role of Tripartite motif containing 72 (TRIM72) that expresses in a limited number of tissues including the lung. Read More

J Heart Lung Transplant 2020 Apr 30;39(4S):S387. Epub 2020 Mar 30.

Lewis Katz School of Medicine, Philadelphia, PA.

Purpose: Due to the high mortality of idiopathic pulmonary fibrosis (IPF) patients on the waiting list, potential older donors are considered frequently for lung transplantation. We investigated the survival outcome of IPF patients receiving lungs from donors aged <50 and ≥50 years.

Methods: We retrospectively studied IPF transplant recipients that underwent either a single or double lung transplant at our center (Mar-2012 to Jun-2019) and divided patients into two groups (donors age <50 vs. Read More

J Heart Lung Transplant 2020 Apr 30;39(4S):S382-S383. Epub 2020 Mar 30.

University of Pittsburgh, Pittsburgh, PA.

Purpose: Epstein-Barr virus (EBV)-associated post-transplant lymphoproliferative disorder (PTLD) is a serious complication in lung transplant recipients (LTRs) associated with significant mortality. We performed a single-center retrospective study to evaluate the risks for PTLD in LTRs over a seven-year period.

Methods: All LTRs transplanted from 2010 to 2016 were included. Read More

J Heart Lung Transplant 2020 Apr 30;39(4S):S326-S327. Epub 2020 Mar 30.

Pulmonary Medicine, The University of Florida, Gainesville, FL.

Purpose: Acute exacerbations of interstitial lung disease (AE-ILD) have a poor prognosis, and lung transplantation can be lifesaving. Previous studies have reported significantly higher mortality in patients transplanted with acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF) compared to stable IPF. These results have yet to be corroborated, and whether they apply to the wide spectrum of AE-ILD beyond IPF is unknown. Read More

J Heart Lung Transplant 2020 Apr 30;39(4S):S313-S314. Epub 2020 Mar 30.

Department of Thoracic Surgery, The University Tokyo Graduate School of Medicine, Tokyo, Japan.

Purpose: Idiopathic pleurparenchymal fibroelastosis (IPPFE) is a relatively new disease entity of interstitial pneumonia characterized by upper lobe dominant fibrosis and flat chest. The purpose of this study is to evaluate the outcome of lung transplantation for IPPFE in comparison with idiopathic pulmonary fibrosis (IPF).

Methods: We retrospectively reviewed patients with IPPFE and those with IPF aged 20 or over who underwent lung transplantation between 1998 and 2018. Read More

J Heart Lung Transplant 2020 Apr 30;39(4S):S305-S306. Epub 2020 Mar 30.

Pneumology, Ruhrlandklinik, University Hospital, University Duisburg-Essen, Essen, Germany.

Purpose: The long-term survival of lung transplant recipients is significantly limited by the occurrence of chronic lung allograft dysfunction (CLAD). A single nucleotide polymorphism (SNP) in the promoter region of the MUC5B-gene (rs35705950) has been identified as an independent risk factor for the development of idiopathic pulmonary fibrosis (IPF). Moreover, MUC5B genotype is associated with survival in IPF patients. Read More

J Heart Lung Transplant 2020 Apr 30;39(4S):S20. Epub 2020 Mar 30.

Division of Infectious Disease, Duke University Medical Center, Durham, NC.

Introduction: In light of a national shortage of thoracic organs, patients with end stage lung disease need an expanded donor pool. There are approximately 847,000 persons with chronic hepatitis B virus (HBV) in the U.S. Read More

J Heart Lung Transplant 2020 Apr 30;39(4S):S169. Epub 2020 Mar 30.

Biochemistry and Molecular Biology, UTHealth - McGovern Medical School, Houston, TX.

Purpose: Pulmonary hypertension is a common complication of idiopathic pulmonary fibrosis (IPF), which leads to cor pulmonale and right-heart failure. β-blockers have been used extensively for the treatment of left heart disease, yet their use for right-heart failure is controversial. Recent studies have shown that β-blockers may be beneficial for the treatment of pulmonary arterial hypertension. Read More

J Heart Lung Transplant 2020 Apr 30;39(4S):S104. Epub 2020 Mar 30.

Department of Cardiothoracic, Transplant and Vascular Surgery, Hannover Medical School, Hannover, Germany.

Purpose: In lung transplantation, intraoperative extracorporeal membrane oxygenation (ECMO) allows transplanting high-risk patients, such as those with idiopathic pulmonary arterial hypertension (iPAH). However, early postoperative course is more complicated in patients with than without ECMO, with higher prevalence of severe primary graft dysfunction (PGD), a risk factor for chronic lung allograft dysfunction (CLAD). Aim of this retrospective study was to test the hypothesis if the worse early postoperative course in ECMO patients translates in a worse long-term graft function too. Read More

Clin Respir J 2020 May 27. Epub 2020 May 27.

Department of Respiratory and Critical Care Medicine, Beijing Engineering Research Center of Respiratory and Critical Care Medicine, Beijing Institute of Respiratory Medicine, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, China.

Introduction: Relatively little is known about the effects of mechanical ventilation (MV; including invasive MV [IMV] and noninvasive ventilation) on clinical outcomes of patients with idiopathic pulmonary fibrosis (IPF) and connective tissue disease-associated interstitial lung disease (CTD-ILD) in the intensive care unit (ICU), and risk factors for ICU death remain to be determined.

Objectives: Our objective was to determine and compare mortality rates between IPF and CTD-ILD patients receiving MV and to identify risk factors for ICU death in these patients.

Methods: We conducted a retrospective cohort study in respiratory ICUs of three university hospitals in China during a 7-year period. Read More

J Clin Med 2020 May 21;9(5). Epub 2020 May 21.

Institute for Pulmonary Rehabilitation Research, Schoen Klinik Berchtesgadener Land, Malterhoeh 1, 83471 Schoenau am Koenigssee, Germany.

The recommendation for pulmonary rehabilitation (PR) in idiopathic pulmonary fibrosis (IPF) is weak with low-quality evidence. Therefore, the aim of this study is to investigate short-term PR effects and their maintenance after a 3-month follow-up. Fifty-four IPF patients were randomized into a group receiving a 3-week comprehensive, inpatient PR ( = 34, FVC: 74 ± 19% pred. Read More

Curr Opin Pulm Med 2020 May 20. Epub 2020 May 20.

Campus Venlo, Maastricht University, Maastricht, The Netherlands.

Purpose Of The Review: Idiopathic pulmonary fibrosis (IPF) is a terminal lung disease of largely unknown cause. Gastroesophageal reflux disease (GERD) was recently discovered to be a trigger for the development of IPF. The current pharmaceutical approach to IPF falls short and there is a pressing need for improved therapeutic options. Read More

Adv Ther 2020 May 21. Epub 2020 May 21.

Decision Resources Group, Burlington, USA.

Introduction: Idiopathic pulmonary fibrosis (IPF) is the classic progressive fibrosing interstitial lung disease (ILD), but some patients with ILDs other than IPF also develop a progressive fibrosing phenotype (PF-ILD). Information on use and cost of healthcare resources in patients with PF-ILD is limited.

Methods: We used USA-based medical insurance claims (2014-2016) to assess use and cost of healthcare resources in PF-ILD. Read More

Intern Emerg Med 2020 May 25. Epub 2020 May 25.

Department of Medical and Surgical Sciences, University Hospital of Modena, Respiratory Diseases Unit and Centre for Rare Lung Diseases, University of Modena Reggio Emilia, Modena, Italy.

Data on the presence of subclinical fibrosis across multiple organs in patients with idiopathic lung fibrosis (IPF) are lacking. Our study aimed at investigating through hepatic transient elastography (HTE) the prevalence and clinical impact of subclinical liver fibrosis in a cohort of patients with IPF. Patients referred to the Centre for Rare Lung Disease of the University Hospital of Modena (Italy) from March 2012 to February 2013 with established diagnosis of IPF and without a documented history of liver diseases were consecutively enrolled and underwent HTE. Read More

Chest 2020 May 22. Epub 2020 May 22.

Department of Internal Medicine; Division of Pulmonary, Critical Care and Sleep Medicine; University of California at Davis, Sacramento, CA. Electronic address:

Background: A number of circulating plasma biomarkers have been shown to predict survival in patients with idiopathic pulmonary fibrosis (IPF), but most were identified prior to the use of anti-fibrotic therapy (AF) in this population. Because pirfenidone and nintedanib have been shown to slow IPF progression and may prolong survival, the role of such biomarkers in AF treated patients is unclear.

Research Question: To determine whether plasma concentration of CA-125, CXCL13, MMP7, SP-D, YKL-40, VCAM-1 and OPN is associated with differential transplant-free survival (TFS) in AF exposed and non-exposed patients with IPF. Read More

Int J Cardiovasc Imaging 2020 May 25. Epub 2020 May 25.

Semi-Intensive Care Unit, Department of Pneumology, Department of Respiratory Physiopathology and Pulmonary Hemodynamics, Ospedale San Giuseppe MultiMedica IRCCS, Via San Vittore 12, 20123, Milan, Italy.

No data are actually available regarding the left atrial (LA) functional assessment by two-dimensional speckle tracking echocardiography (2D-STE) in early-stage idiopathic pulmonary fibrosis (IPF). The primary end-point of our study was to assess whether global LA peak strain (GLAPS), measured by 2D-STE analysis, may detect early alterations in LA function in IPF patients without right heart failure (RHF). Between September 2017 and January 2019, 50 consecutive IPF patients (73. Read More

Expert Opin Investig Drugs 2020 May 23. Epub 2020 May 23.

Fondazione Policlinico Universitario "A. Gemelli" IRCCS , Rome, Italy.

Introduction: The two available therapies for idiopathic pulmonary fibrosis (IPF), pirfenidone and nintedanib, slow down but do not halt IPF progression. Hence, in the last few years, several agents with specific molecular targets have been investigated to find a cure for IPF. Pamrevlumab, a recombinant human antibody that binds to connective tissue growth factor (CTGF) has emerged as a potential therapy for IPF and has advanced to phase 3 clinical trials. Read More

Adv Ther 2020 May 22. Epub 2020 May 22.

Boehringer Ingelheim, Amsterdam, The Netherlands.

Introduction: The term progressive fibrosing interstitial lung disease (ILD) describes patients with fibrotic ILDs who, irrespective of the aetiology of the disease, show a progressive course of their disease despite current available (and non-licensed) treatment. Besides in idiopathic pulmonary fibrosis, little is known about management and the burden of patients with fibrotic ILD, particularly those with a progressive behaviour.

Methods: Using the Delphi method, 40 European experts in ILD management delivered information on management of (progressive) fibrosing ILD and on the impact of the disease on patients' quality of life (QoL) and healthcare resource utilisation (HCRU). Read More

Drug Healthc Patient Saf 2020 7;12:85-94. Epub 2020 May 7.

Division of Pulmonary, Allergy, and Critical Care Medicine, University of Alabama at Birmingham, Birmingham, AL, USA.

Idiopathic Pulmonary Fibrosis (IPF) is a chronic fibrotic disease characterized by a progressive decline in lung function with a median survival of 3-5 years after diagnosis. The course of disease is highly variable and unpredictable, often punctuated by episodes of acute respiratory failure, known as acute exacerbations. The incidence of IPF is on the rise due to the aging population, as age is the most important risk factor for this disease. Read More