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  • 1
    Language: English
    In: Respiration; international review of thoracic diseases, 2014, Vol.87(1), pp.1-2
    Description: No abstract available [PUBLICATION ]
    Keywords: Bronchoscopy -- Methods ; Pulmonary Medicine -- Methods ; Ultrasonography -- Methods
    ISSN: 00257931
    E-ISSN: 1423-0356
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  • 2
    Language: English
    In: Respiration; international review of thoracic diseases, 2011, Vol.82(4), pp.307-16
    Description: Supportive and palliative care is an interdisciplinary challenge with the aims of symptom relief and improvement of quality of life in end-stage patients. Main complaints of patients with advanced nonmalignant lung disease are depression and anxiety, dyspnea, pain, and coughing. The discomfort of many physicians, caregivers, and family members with discussions about end-of-life care is one obstacle for the timely initiation of palliative care and the uncertainty of the short-term prognosis in most advanced nonmalignant respiratory diseases. Early dialog about supportive care already at the onset of the patient's first symptoms and contemporaneous to life-prolonging therapy may overcome these barriers. Furthermore, continuing education for health professionals in palliative care ensures adequate palliative support. Here, we review insights into symptom control and palliative care in patients with advanced nonmalignant respiratory disease.
    Keywords: Palliative Care ; Quality of Life ; Dyspnea -- Therapy ; Lung Diseases -- Therapy ; Pain -- Drug Therapy
    ISSN: 00257931
    E-ISSN: 1423-0356
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  • 3
    Language: English
    In: Respiration; international review of thoracic diseases, 2018, Vol.96(2), pp.117-126
    Description: Detection of surfactant proteins A and D (SP-A and SP-D) in the serum of patients with pulmonary diseases is thought to reflect an injury of the alveolar epithelial barrier and as such serve as a biomarker for these diseases. However, the data for SP-B are limited. The aim of this feasibility study was to assess whether immature SP-B pre-proteins might have value as a possible biomarker for pulmonary diseases. In serum samples from patients with different chronic lung diseases (interstitial lung diseases [ILDs], chronic obstructive pulmonary disease, asthma, lung cancer, pulmonary hypertension, inflammation, patients on ventilator support; total n = 283), C-proSP-B was measured using an electrochemiluminescence immunoassay based on mouse monoclonal anti-C-proSP-B antibodies. Levels were correlated to lung functional and clinical parameters. The highest C-proSP-B levels were detected in the serum of idiopathic pulmonary fibrosis (IPF) patients. In a multivariate analysis, C-proSP-B levels were able to discriminate IPF patients from patients with all other pulmonary diseases (p 〈 0.0001). No significant correlations were found between C-proSP-B levels and lung function, smoking history, or disease extent. SP-B pre-proteins might serve as a biomarker in pulmonary diseases with alveolar or interstitial damage such as ILDs, especially in IPF. Their role in the long-term monitoring of such diseases has to be clarified further.
    Keywords: Biomarker ; C-Prosp-B ; Interstitial Lung Diseases ; Pulmonary Diseases ; Lung Diseases -- Blood ; Pulmonary Surfactant-Associated Protein B -- Blood
    ISSN: 00257931
    E-ISSN: 1423-0356
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  • 4
    Language: English
    In: Respiration, September 2016, Vol.92(2), pp.98-106
    Description: Background: Nintedanib is approved for the treatment of idiopathic pulmonary fibrosis (IPF) and has been shown to slow disease progression by reducing annual lung function decline. Objective: To evaluate the results of a large cohort of IPF patients treated with nintedanib within a compassionate use program (CUP) in Germany (9 centers). Methods: Patients (≥40 years) were required to have a confirmed diagnosis of IPF, a forced vital capacity (FVC) ≥50% predicted (pred.) and a carbon monoxide diffusing capacity (DLCO) 30-79% pred. and not to be eligible for pirfenidone treatment. Clinical data, pulmonary function tests and adverse events were recorded up to July 2015. Results: Sixty-two patients (48 male/14 female) with moderate IPF (FVC 64 ± 17% pred. and DLCO 40 ± 10% pred.) were treated with nintedanib. 77% of patients switched from pirfenidone (mean treatment duration 14 ± 2 months) mostly due to disease progression (mean decline in FVC 7.4 ± 3% pred. in the 6 months prior to nintedanib intake). Initiation of nintedanib treatment occurred 69 ± 29 months after IPF diagnosis, and mean treatment duration was 8 ± 4 months. Most patients (63%) stabilized 6 months after treatment start (mean FVC decline 3 ± 1 vs. -17 ± 2% in patients with disease progression; p 〈 0.01). The most common adverse events were diarrhea (63%) and weight loss (50%). Dose reduction occurred in 34% of cases and treatment discontinuation in 10%. Conclusion: Nintedanib treatment was generally well tolerated and was associated with FVC stabilization in the majority of IPF patients in this CUP setting where most patients were not treatment naïve. Our data are in agreement with the previously published data.
    Keywords: Clinical Investigations ; Idiopathic Pulmonary Fibrosis ; Nintedanib ; Compassionate Use ; Medicine
    ISSN: 0025-7931
    E-ISSN: 1423-0356
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  • 5
    Language: English
    In: Respiration; international review of thoracic diseases, 2018, Vol.96(6), pp.514-524
    Description: Antifibrotics are recommended for the treatment of individuals with idiopathic pulmonary fibrosis (IPF), but treatment use remains at ∼60%. To investigate the views of individuals with IPF and pulmonologists on the diagnosis and management of IPF to understand treatment patterns. Interviews and/or online surveys were completed by patients and pulmonologists from Canada, France, Germany, Italy, Spain, and the UK. Responses from physicians were analyzed by time between diagnosis and treatment initiation in the majority of patients with IPF (group A, 〉 4 months; group B, ≤4 months). Statistical comparisons between physicians were undertaken using z tests, with p 〈 0.05 considered statistically significant. The physicians in group A saw fewer patients, were less comfortable discussing the IPF prognosis with patients, and had less belief in the benefits of antifibrotic treatments than the physicians in group B. These physicians' attitudes contrasted with those of the patients, who wanted more information about the IPF prognosis and pharmacological treatment options at diagnosis and were more concerned about preventing disease progression than avoiding medication side effects. Differences between countries were found regarding physicians' comfort in discussing the prognosis at diagnosis and access to care. Several barriers to antifibrotic treatment, principally reflecting the differing views and values of patients and physicians, were identified in this study, suggesting a need for better patient-physician communication about pharmacological therapy for IPF.
    Keywords: Antifibrotic Treatment ; Barriers to Treatment ; Idiopathic Pulmonary Fibrosis ; Patient-Physician Communication ; Anti-Inflammatory Agents, Non-Steroidal -- Therapeutic Use ; Idiopathic Pulmonary Fibrosis -- Drug Therapy ; Indoles -- Therapeutic Use ; Pulmonologists -- Psychology ; Pyridones -- Therapeutic Use
    E-ISSN: 1423-0356
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  • 6
    Language: English
    In: Respiration; international review of thoracic diseases, 2014, Vol.87(3), pp.204-10
    Description: Pulmonary function may decline after induction chemotherapy and predict perioperative complications in non-small cell lung cancer (NSCLC). The influence of adjuvant chemotherapy is largely indeterminate. To assess whether adjuvant chemotherapy alters pulmonary function and impacts on treatment-related adverse events. In a trial on adjuvant chemotherapy (the TREAT trial), 132 patients with R0-resected NSCLC were randomised to 4 cycles of cisplatin-vinorelbine (CVb, n = 65) or cisplatin-pemetrexed (CPx, n = 67). Pulmonary function tests (forced expiratory volume in 1 s, FEV1, forced vital capacity, FVC, total lung capacity, TLC, diffusing capacity for carbon monoxide, DLCO, and blood gas analyses, BGA) were analysed before and 30 days after the last chemotherapy, and changes were calculated (Δ = mean differences). Overall, FVC increased significantly (Δ +290 ml, n = 76; p 〈 0.0001), while TLC did not change (Δ +220 ml, n = 41; p = 0.174). For CPx, FEV1 increased significantly (Δ +150 ml, n = 47; p = 0.0017), but not for CVb (Δ +30 ml, n = 30). DLCO decreased only for CVb (-8%, n = 6) but not for CPx (-0.39%, n = 17; p = 0.58). BGA did not change (p = 0.99). In a Cox regression analysis, baseline pulmonary function did not influence treatment failure. Adjuvant chemotherapy seems not to result in a decrease of pulmonary function parameters. A significant FVC increase was probably due to ongoing postoperative improvement. Decline of DLCO was noted with CVb but not with CPx. Pulmonary function does not impact on treatment failure.
    Keywords: Antineoplastic Combined Chemotherapy Protocols -- Therapeutic Use ; Carcinoma, Non-Small-Cell Lung -- Drug Therapy ; Lung -- Physiopathology ; Lung Neoplasms -- Drug Therapy
    ISSN: 00257931
    E-ISSN: 1423-0356
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  • 7
    Language: English
    In: Respiration; international review of thoracic diseases, 2018, Vol.96(4), pp.314-322
    Description: Diabetes mellitus is a possible risk factor for the development of idiopathic pulmonary fibrosis (IPF), yet the effect of antidiabetic therapy on the course of IPF is unknown. This post hoc analysis assessed the effect of metformin on clinically relevant outcomes in patients with IPF. For the primary analysis, patients randomized to placebo (n = 624) in 3 phase 3, double-blind, controlled trials of pirfenidone (CAPACITY [NCT00287716 and NCT00287729]; ASCEND [NCT01366209]) were categorized by baseline metformin use. The primary outcome was disease progression (forced vital capacity [FVC] decline ≥10%, 6-min walking distance [6MWD] decline ≥50 m, or death). Other outcomes included mortality, hospitalization, FVC decline (≥10 and ≥5%), and 6MWD decline. Outcomes were also assessed in patients with diabetes and/or hyperglycemia (impaired glucose tolerance [IGT] and diabetes population [IGT-diabetes population]) and all patients included in the 3 studies (intention-to-treat [ITT] population). Overall, 71 (11.4%) patients were metformin users and 553 (88.6%) were nonmetformin users. Baseline data were similar between groups, except for a higher percentage of males (84.5 vs. 73.2%) and a history of diabetes (98.6 vs. 11.6%) in metformin users versus nonmetformin users. The unadjusted 1-year analyses demonstrated no significant differences in disease progression or other outcomes. A higher proportion of metformin users compared with nonmetformin users had a relative FVC decline of ≥5% (63.4 vs. 50.6%, p = 0.043). Results were similar for the IGT-diabetes population and for the ITT population. Multivariable analyses yielded similar results. Metformin has no effect on clinically relevant outcomes in patients with IPF.
    Keywords: Diabetes Mellitus ; Hyperglycemia ; Idiopathic Pulmonary Fibrosis ; Metformin ; Post Hoc Analysis ; Diabetes Mellitus -- Drug Therapy ; Hypoglycemic Agents -- Therapeutic Use ; Idiopathic Pulmonary Fibrosis -- Complications ; Metformin -- Therapeutic Use ; Vital Capacity -- Drug Effects
    ISSN: 00257931
    E-ISSN: 1423-0356
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  • 8
    Language: English
    In: Respiration; international review of thoracic diseases, 2018, Vol.95(5), pp.301-309
    Description: Nintedanib, an oral tyrosine kinase inhibitor, has been shown to slow down the progression of idiopathic pulmonary fibrosis (IPF) in two randomised placebo-controlled trials by reducing the annual decline in forced vital capacity (FVC). However, real-world experience is limited. To assess the efficacy and safety of nintedanib in a large cohort of patients treated at a tertiary referral site for interstitial lung diseases. The records of patients with a confirmed diagnosis of IPF were reviewed. Full medical history, pulmonary function, and adverse events (AEs) were recorded from each clinic visit. Disease progression was defined as a reduction in FVC ≥5% and/or in diffusing capacity of the lung for carbon monoxide ≥15% according to recent publications. Only patients with a treatment duration ≥3 months were included in the efficacy evaluation. A total of 64 patients were treated. Mean ± standard deviation (SD) FVC was 71 ± 21% predicted, and the mean time from diagnosis to initiation of nintedanib treatment was 23.8 months. Nearly half of patients (n = 30, 47%) had received prior pirfenidone treatment. The mean duration of follow-up was 11 months. At 6 months following initiation of nintedanib, 67% of the patients were stable. The mean ± SD change in percent predicted FVC from baseline was 0.2 ± 7.8% at 3 months, -1.3 ± 7.9% at 6 months, and -2.1 ± 9% at 9 months. Diarrhoea was the most common AE experienced by 33% of patients and was generally manageable. The results from this real-world clinical setting support findings from previously conducted clinical trials and show that nintedanib is effective for the management of IPF and is associated with disease stabilisation. Nintedanib is generally well tolerated.
    Keywords: Idiopathic Pulmonary Fibrosis ; Nintedanib ; Real-World Experience ; Antineoplastic Agents -- Therapeutic Use ; Idiopathic Pulmonary Fibrosis -- Drug Therapy ; Indoles -- Therapeutic Use
    ISSN: 00257931
    E-ISSN: 1423-0356
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  • 9
    Language: English
    In: Respiration; international review of thoracic diseases, 2014, Vol.88(3), pp.199-207
    Description: Pirfenidone is a novel antifibrotic drug for the treatment of mild-to-moderate idiopathic pulmonary fibrosis (IPF). However, adverse events may offset treatment benefits and compliance. To assess recent course of disease, adverse events and compliance in patients who started pirfenidone. In an observational cohort study, 63 patients with mild-to-moderate IPF who started pirfenidone between May 2011 and June 2013 were reviewed. Pulmonary function, adverse events and treatment compliance were recorded at each clinic visit. Disease progression was defined as a reduction of vital capacity ≥10% and/or diffusion capacity (DLCO) ≥15%. Follow-up time on pirfenidone treatment was 11 (±7) months. Sixty-six percent of the patients continued with pirfenidone monotherapy and 34% of the patients received pirfenidone combined with corticosteroids (CCS) and/or N-acetylcysteine (NAC). There was a nonsignificant reduction in mean decline of percent predicted forced vital capacity after treatment start (0.7 ± 10.9%) compared to the pretreatment period (6.6 ± 6.7%, p = 0.098). Sixty-two percent of the patients had stable disease on pirfenidone treatment. Adverse events affected 85% of the patients, leading to discontinuation of pirfenidone in 20%. Adverse events and treatment discontinuation were seen more frequently in patients with concomitant CCS and/or NAC treatment. Adverse events affect the majority of patients treated with pirfenidone, but are mostly manageable with supportive measures. In this heterogeneous patient group, a nonsignificant effect of pirfenidone treatment on pulmonary function was seen, underlining the need for more data on patient selection criteria and efficacy of pirfenidone, particularly in patients with coexistent emphysema and concomitant NAC/CCS treatment.
    Keywords: Anti-Inflammatory Agents, Non-Steroidal -- Therapeutic Use ; Idiopathic Pulmonary Fibrosis -- Drug Therapy ; Pyridones -- Therapeutic Use
    ISSN: 00257931
    E-ISSN: 1423-0356
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  • 10
    Language: English
    In: Respiration; international review of thoracic diseases, 2016, Vol.91(1), pp.3-8
    Description: Increasing evidence suggests a role of gastro-oesophageal reflux (GER) in idiopathic pulmonary fibrosis (IPF) pathogenesis. Recently, an association between serum Helicobacter pylori (HP) antibody positivity and more severe disease was described, but HP has not been directly analysed in lung tissue so far. To investigate the presence of HP in the lung tissue of IPF patients. Two tertiary interstitial lung disease care centre databases were screened for available lung biopsy material from IPF patients. Clinical and radiological data, including presence of GER and antiacid medication, were evaluated. HP-specific PCR was carried out on the IPF lung biopsy specimens. A total of 39 IPF patients were included, of whom 85% were male. The patients' median age was 66 years, their vital capacity was 79% predicted, and their diffusing capacity for carbon monoxide was 53% predicted. In all, 82% of the lung biopsies were surgical and 18% transbronchial. Comorbidities were GER disease in 23% (n = 9), sleep apnoea in 13% (n = 5) and hiatal hernia in 38% of the cases (n = 15). Proton pump inhibitors were prescribed at the time of biopsy in 21% of the cases (n = 9). After a median follow-up of 25 months (range 6-69), there were 1 death, 1 lung transplantation and 8 acute exacerbations without relevant differences between the GER and non-GER subgroups. HP DNA was not detected in any of the lung tissue samples. The fact that no HP DNA was detected in the lung tissues calls into question the proposed relevance of HP to the direct pathogenesis of IPF.
    Keywords: DNA, Bacterial -- Isolation & Purification ; Gastroesophageal Reflux -- Epidemiology ; Helicobacter Infections -- Epidemiology ; Helicobacter Pylori -- Isolation & Purification ; Hernia, Hiatal -- Epidemiology ; Idiopathic Pulmonary Fibrosis -- Epidemiology ; Lung -- Chemistry
    ISSN: 00257931
    E-ISSN: 1423-0356
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