nintedanib and Dyspnea

To present an overview of the impact of idiopathic pulmonary fibrosis (IPF) on patients' emotional well being and quality of life (QoL).. IPF is an interstitial lung disease which causes irreversible, progressive lung scarring in a pathological pattern of usual interstitial pneumonia. The incidence of IPF is increasing at a global level, subjecting an increasing number of people to its high morbidity and risk of mortality. Diagnosis is based on a multidisciplinary team approach and the exclusion of other interstitial lung diseases. Two novel antifibrotic treatments, pirfenidone and nintedanib, were recently approved by regulatory agencies around the globe, thus providing many IPF patients with treatment options for the first time. Several other drugs have entered the investigational pipeline, including many in early-phase or late-phase clinical trials. Given the incurable and progressive nature of IPF, even with antifibrotic therapy, depression and anxiety are common among patients; these and burdensome symptoms of breathlessness, cough and fatigue are factors that impact patients' emotional well being and QoL. In addition to even more effective drugs, there is a need for psychosocial interventions and mental health support strategies focused on improving patients' QoL so they are better equipped to live with this devastating condition.. The current article highlights the effects of IPF on patients' emotional well being and QoL and offers suggestions for strategies to help patients with IPF live as well as possible in their daily lives.

Topics: Anti-Inflammatory Agents, Non-Steroidal; Cough; Dyspnea; Fatigue; Humans; Idiopathic Pulmonary Fibrosis; Indoles; Lung; Mental Health; Protein Kinase Inhibitors; Pyridones; Quality of Life

Idiopathic Pulmonary Fibrosis (IPF) is a most common progressive interstitial lung disease (ILD) of unknown etiology, although majority of patients are elderly male smokers. The main pathogenesis is aberrant recovery of epithelial injury and collagen deposition. Fibrotic nonspecific interstitial pneumonia, connective tissue disease (CTD) especially rheumatoid arthritis (RA) associated ILD, and chronic hypersensitivity pneumonia(CHP) are important differential diagnosis. Main symptoms are non-productive cough and progressive exertional dyspnea. Crucial physical findings are scalene muscle hypertrophy, bibasilar fine crackles, and finger clubbing. The serum markers such as lactate dehydrogenase (LDH) and Krebs von den Lungen-6 (KL-6) are sensitive for ILD detection and activity. Both pulmonary function test (PFT) and the 6-minute walk test (6MWT) are useful tool for evaluation of disease progression of IPF. Serial changes of forced vital capacity (FVC) and 6MWT distance predict mortality in IPF effectively. Recently published international IPF guidelines highlight the importance of chest high resolution computed tomography (HRCT) findings such as honeycombing, traction bronchiectasis (TBE), and sub-pleural reticular opacity. IPF is chronic and progressive; therefore, tracking disease behavior is crucial. Unifying clinical, physiological, and imaging information over time is useful. With regard to its management, two anti-fibrotic drugs such as pirfenidone and nintedanib have been available. These drugs can slow the decline of FVC and prevent acute exacerbation (AE). In this review, I outline the clinical characteristics of IPF, physiological, imaging, pathological findings and review diagnosis process and management.

Topics: Biomarkers; Chronic Disease; Cough; Diagnosis, Differential; Disease Progression; Dyspnea; Humans; Idiopathic Pulmonary Fibrosis; Indoles; L-Lactate Dehydrogenase; Mucin-1; Pyridones; Respiratory Function Tests; Tomography, X-Ray Computed; Vital Capacity

Topics: Diagnosis, Differential; Dyspnea; Enzyme Inhibitors; Humans; Idiopathic Pulmonary Fibrosis; Indoles; Lung; Oxygen Inhalation Therapy; Prognosis; Pyridones; Risk Factors; Tomography, X-Ray Computed; Transforming Growth Factor beta

Nintedanib is an approved treatment for idiopathic pulmonary fibrosis (IPF). A subgroup analysis of a previously published trial suggested that sildenafil may provide benefits regarding oxygenation, gas exchange as measured by the diffusion capacity of the lungs for carbon monoxide (Dl. We randomly assigned, in a 1:1 ratio, patients with IPF and a Dl. A total of 274 patients underwent randomization. There was no significant difference in the adjusted mean change from baseline in the SGRQ total score at week 12 between the nintedanib-plus-sildenafil group and the nintedanib group (-1.28 points and -0.77 points, respectively; P=0.72). A benefit from sildenafil treatment was not observed with regard to dyspnea as measured with the use of the University of California, San Diego, Shortness of Breath Questionnaire. No new safety signals were observed, as compared with previous trials.. In patients with IPF and a Dl

Topics: Adult; Aged; Double-Blind Method; Drug Therapy, Combination; Dyspnea; Enzyme Inhibitors; Female; Humans; Idiopathic Pulmonary Fibrosis; Indoles; Male; Middle Aged; Phosphodiesterase 5 Inhibitors; Protein-Tyrosine Kinases; Pulmonary Gas Exchange; Quality of Life; Sildenafil Citrate; Treatment Outcome

Pulmonary rehabilitation improves dyspnea and exercise capacity in idiopathic pulmonary fibrosis (IPF); however, it is unknown whether breathing high amounts of oxygen during exercise training leads to further benefits.. Herein, we describe the design of the High Oxygen Delivery to Preserve Exercise Capacity in IPF Patients Treated with Nintedanib study (the HOPE-IPF study). The primary objective of this study is to determine the physiological and perceptual impact of breathing high levels of oxygen during exercise training in patients with IPF who are receiving antifibrotic therapy.. HOPE-IPF is a two-arm double-blind multicenter randomized placebo-controlled trial of 88 patients with IPF treated with nintedanib. Patients will undergo 8 weeks of three times weekly aerobic cycle exercise training, breathing a hyperoxic gas mixture with a constant fraction of 60% inhaled oxygen, or breathing up to 40% oxygen as required to maintain an oxygen saturation level of at least 88%.. End points will be assessed at baseline, postintervention (Week 8), and follow-up (Week 26). The primary analysis will compare the between-group baseline with post-training change in endurance time during constant work rate cycle exercise tests. Additional analyses will evaluate the impact of training with high oxygen delivery on 6-minute walk distance, dyspnea, physical activity, and quality of life.. The HOPE-IPF study will lead to a comprehensive understanding of IPF exercise physiology, with the potential to change clinical practice by indicating the need for increased delivery of supplemental oxygen during pulmonary rehabilitation in patients with IPF. Clinical trial registered with www.clinicaltrials.gov (NCT02551068).

Topics: Adult; Aged; Canada; Double-Blind Method; Dyspnea; Exercise Test; Exercise Therapy; Exercise Tolerance; Female; Humans; Idiopathic Pulmonary Fibrosis; Indoles; Male; Middle Aged; Oxygen; Quality of Life; Research Design; Treatment Outcome; Vital Capacity; Young Adult

Nintedanib has been shown to significantly reduce the annual rate of decline in the forced vital capacity (FVC) in patients with idiopathic pulmonary fibrosis (IPF) in previous randomized trials. A 71-year-old man developed exertional dyspnea and was diagnosed with IPF. Four months after treatment with nintedanib, high-resolution computed tomography findings revealed reduced areas of ground-glass opacity and consolidation; 13 months after treatment, the FVC showed improvement from 3.07 to 3.43 L, and the serum Krebs von den Lungen (KL)-6 concentration showed a decline to normal levels. We herein report a patient with IPF who was considered a super responder to nintedanib.

Topics: Aged; Antineoplastic Agents; Dyspnea; Humans; Idiopathic Pulmonary Fibrosis; Indoles; Male; Tomography, X-Ray Computed; Treatment Outcome; Vital Capacity

Topics: Aged; Alveolitis, Extrinsic Allergic; Biopsy; Diagnosis, Differential; Dyspnea; Humans; Idiopathic Pulmonary Fibrosis; Indoles; Lung; Male; Pyridones; Radiography, Thoracic; Sarcoidosis; Tomography, X-Ray Computed

In the Phase III INPULSIS(®) trials, 52 weeks' treatment with nintedanib reduced decline in forced vital capacity (FVC) versus placebo in patients with idiopathic pulmonary fibrosis (IPF). Patients who completed the INPULSIS(®) trials could receive nintedanib in an open-label extension trial (INPULSIS(®)-ON). Patients with FVC <50 % predicted were excluded from INPULSIS(®), but could participate in INPULSIS(®)-ON. In patients with baseline FVC ≤50 % and >50 % predicted at the start of INPULSIS(®)-ON, the absolute mean change in FVC from baseline to week 48 of INPULSIS(®)-ON was -62.3 and -87.9 mL, respectively (n = 24 and n = 558, respectively). No new safety signals were identified in INPULSIS(®)-ON compared with INPULSIS(®). The decline in FVC in INPULSIS(®)-ON in both subgroups by baseline FVC % predicted was similar to that in INPULSIS(®), suggesting that nintedanib may have a similar effect on disease progression in patients with advanced disease as in less advanced disease.

Topics: Aged; Diarrhea; Disease Progression; Dyspnea; Enzyme Inhibitors; Female; Humans; Idiopathic Pulmonary Fibrosis; Indoles; Male; Middle Aged; Vital Capacity