Pirfenidone is one of two approved therapies for the treatment of idiopathic pulmonary fibrosis (IPF). Randomised controlled clinical trials and subsequent
analyses have demonstrated that pirfenidone ...reduces lung function decline, decreases mortality and improves progression-free survival. Long-term extension trials, registries and real-world studies have also shown similar treatment effects with pirfenidone. However, for patients with IPF to obtain the maximum benefits of pirfenidone treatment, the potential adverse events (AEs) associated with pirfenidone need to be managed. This review highlights the well-known and established safety profile of pirfenidone based on randomised controlled clinical trials and real-world data. Key strategies for preventing and managing the most common pirfenidone-related AEs are described, with the goal of maximising adherence to pirfenidone with minimal AEs.
Acute exacerbation of interstitial lung disease (ILD) is associated with a poor prognosis and high mortality. Numerous studies have documented acute exacerbation in idiopathic pulmonary fibrosis ...(IPF), but less is known about these events in other ILDs that may present a progressive-fibrosing phenotype. We propose defining acute exacerbation as an acute, clinically significant respiratory deterioration, typically less than 1 month in duration, together with computerised tomography imaging showing new bilateral glass opacity and/or consolidation superimposed on a background pattern consistent with fibrosing ILDs. Drawing on observations in IPF, it is suspected that epithelial injury or proliferation and autoimmunity are risk factors for acute exacerbation in ILDs that may present a progressive-fibrosing phenotype, but further studies are required. Current acute exacerbation management strategies are based on recommendations in IPF, but no randomised controlled trials of acute exacerbation management have been performed. Although there are no formal strategies to prevent the development of acute exacerbation, possible approaches include antifibrotic drugs (such as nintedanib and pirfenidone), and minimising exposure to infection, airborne irritants and pollutants. This review discusses the current knowledge of acute exacerbation of ILDs that may present a progressive-fibrosing phenotype and acknowledges limitations of the data available.
Drug-Induced Interstitial Lung Diseases Ng, Nicole; Padilla, Maria L; Camus, Philippe
Immunology and allergy clinics of North America,
05/2023, Letnik:
43, Številka:
2
Journal Article
Recenzirano
Drug-induced interstitial lung disease (DI-ILD) is an increasingly common cause of morbidity and mortality as the list of culprit drugs continues to grow. Unfortunately, DI-ILD is difficult to study, ...diagnose, prove, and manage. This article attempts to raise awareness of the challenges in DI-ILD and discusses the current clinical landscape.
Many health professions schools host anatomy outreach sessions for high school students that utilize anatomical donors. However, teaching with anatomical donors for younger learners is uncommon. This ...study aimed to assess the comfort levels and experiences of students who attended the anatomy sessions as part of summer programs at Rutgers New Jersey Medical School. Younger learners (students entering grades 7–10; n = 25) and older learners (students entering grades 11–12; n = 33) completed pre‐ and post‐session surveys about their comfort using a 5‐point Likert scale. Before the sessions, most students felt comfortable or very comfortable learning from isolated organs (μ = 2.7, SD = 1.3) or full‐body donors (μ = 2.4, SD = 1.4), even though most have never been to an anatomical donor lab before. After the sessions, the comfort level significantly increased for both isolated organs (μ = 3.3, SD = 1.1; p = 0.02) and full‐body donors (μ = 3.1, SD = 1.2; p = 0.004). For isolated organs, there was no significant difference in the comfort level between younger and older learners before (p = 0.50) or after (p = 0.56) the sessions. Similarly, for full‐body donors, there was no significant difference in the comfort level between younger and older learners before (p = 0.95) or after (p = 0.75) the sessions. Most students expressed that the experience was unique and positive. In conclusion, most students entering grades 7–12 felt comfortable learning from isolated organs and full‐body anatomical donors prior to the sessions and felt more comfortable after the sessions. With this, anatomy outreach programs that utilize anatomical donors could be expanded to include younger learners to provide more dynamic teaching experiences for students of various ages.
To study the role of whole-body 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET) scans in the identification of occult biopsy sites and reversible granulomatous disease in patients ...with sarcoidosis.
A retrospective review was undertaken of 188 FDG PET scans performed in 137 patients with proven sarcoidosis. All patients had given a complete medical history and undergone a physical examination, standard chest radiograph, spirometry, diffusing capacity determination, and measurement of serum angiotensin-converting enzymes levels.
One hundred thirty-nine whole-body scans had positive findings. The most common positive sites were mediastinal lymph nodes (54 scans), extrathoracic lymph nodes (30 scans), and lung (24 scans). The standardized uptake value (SUV) ranged from 2.0 to 15.8. Twenty occult disease sites were identified. Eleven repeat scans exhibited decreased SUV with corticosteroid therapy. The positive pulmonary FDG PET scan findings occurred in two thirds of patients with radiographic stage II and III sarcoidosis. Negative pulmonary FDG PET scan findings were common in patients with radiographic stage 0, I, and IV sarcoidosis.
Whole-body FDG PET scans are of value in identifying occult and reversible granulomas in patients with sarcoidosis. However, a positive FDG PET scan finding, by itself, is not an indication for treatment.
Abstract Background Early recognition of patients with interstitial lung disease (ILD) who have an increased risk of developing acute exacerbation (AE) or preclinical AE may be clinically useful, ...since AE is associated with poor outcome and preventive measures would be of interest to ILD researchers. This study evaluated the relationship between elevated serum D-dimer level (≥0.4 mcg/mL) and subsequent AE or preclinical AE in patients with ILD. Methods This single-center, retrospective study was performed from October 2009 through September 2015 in patients with ILD who were ≥18 years old and had idiopathic pulmonary fibrosis, other idiopathic interstitial pneumonias, chronic hypersensitivity pneumonitis, ILD related to collagen tissue disease, or combined pulmonary fibrosis/emphysema. The primary outcome measure was AE development within three months from each D-dimer measurement. The secondary outcome measures were respiratory-related hospitalization, all-cause hospitalization, venous thromboembolism (VTE), and all-cause mortality within three months. Results A total of 263 patients (mean age, 64.1 years) with 374 D-dimer measurements (median, 0.44 mcg/mL) were included. The risk of developing AE was significantly higher in patients with elevated serum D-dimer level (adjusted odds ratio: 10.46; 95% CI: 1.24–88.11; p = 0.03). Patients with elevated serum D-dimer level had increased risk for respiratory-related hospitalization, all-cause hospitalization, VTE, and all-cause mortality. The other factors predictive for AE were home oxygen therapy, increased serum lactate dehydrogenase, decreased FVC, and decreased FEV1.0. Conclusions Elevated serum D-dimer is associated with the risk of developing AE. Serum D-dimer may be used as a prognostic marker to predict AE or recognize preclinical AE.
Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial pneumonia with distinct clinicopathologic features. It has been associated with exposure to hematopoietic stem cell transplantation ...(HSCT) and classical alkylating agents. Here, we highlight PPFE as a late complication of childhood cancer therapy by describing the cases of four survivors of childhood cancer with a diagnosis of treatment‐related PPFE. All patients received high‐dose alkylating agents. PPFE should be considered in the differential diagnosis of restrictive lung disease in patients with history of exposure to alkylating agents or HSCT. Development of PPFE‐specific, noninvasive diagnostic tools and disease‐modifying therapies will clinically benefit these patients.
Noninvasive ventilation (NIV) is an effective form of treatment in obesity hypoventilation syndrome (OHS) with severe OSA. However, there is paucity of evidence in patients with OHS without severe ...OSA phenotype.
Is NIV effective in OHS without severe OSA phenotype?
In this multicenter, open-label parallel group clinical trial performed at 16 sites in Spain, we randomly assigned 98 stable ambulatory patients with untreated OHS and apnea-hypopnea index < 30 events/h (ie, no severe OSA) to NIV or lifestyle modification (control group) using simple randomization through an electronic database. The primary end point was hospitalization days per year. Secondary end points included other hospital resource utilization, incident cardiovascular events, mortality, respiratory functional tests, BP, quality of life, sleepiness, and other clinical symptoms. Both investigators and patients were aware of the treatment allocation; however, treating physicians from the routine care team were not aware of patients' enrollment in the clinical trial. The study was stopped early in its eighth year because of difficulty identifying patients with OHS without severe OSA. The analysis was performed according to intention-to-treat and per-protocol principles and by adherence subgroups.
Forty-nine patients in the NIV group and 49 in the control group were randomized, and 48 patients in each group were analyzed. During a median follow-up of 4.98 years (interquartile range, 2.98-6.62), the mean hospitalization days per year ± SD was 2.60 ± 5.31 in the control group and 2.71 ± 4.52 in the NIV group (adjusted rate ratio, 1.07; 95% CI, 0.44-2.59; P = .882). NIV therapy, in contrast with the control group, produced significant longitudinal improvement in Paco
, pH, bicarbonate, quality of life (Medical Outcome Survey Short Form 36 physical component), and daytime sleepiness. Moreover, per-protocol analysis showed a statistically significant difference for the time until the first ED visit favoring NIV. In the subgroup with high NIV adherence, the time until the first event of hospital admission, ED visit, and mortality was longer than in the low adherence subgroup. Adverse events were similar between arms.
In stable ambulatory patients with OHS without severe OSA, NIV and lifestyle modification had similar long-term hospitalization days per year. A more intensive program aimed at improving NIV adherence may lead to better outcomes. Larger studies are necessary to better determine the long-term benefit of NIV in this subgroup of OHS.
ClinicalTrials.gov; No.: NCT01405976; URL: www.clinicaltrials.gov.
Idiopathic pleuroparenchymal fibroelastosis is a rare recently described entity likely to be under- and misdiagnosed, as awareness of this entity is not yet widespread. We report two cases that show ...the need to include this disease in the differential diagnosis of patients with predominantly pleural and subpleural fibrotic processes. The condition is a fibrotic thickening of the pleura and subpleural parenchyma due to elastic fiber proliferation predominantly in the upper lobes. Performing elastic fiber stains routinely in patients with fibrosis of this distribution may, therefore, aid in establishing the diagnosis and differentiating it from usual interstitial pneumonia/idiopathic pulmonary fibrosis. These patients may be prone to the development of secondary spontaneous pneumothoraces and persistent postoperative bronchopleural fistulae. Continued study of newly diagnosed cases may uncover shared characteristics or features helpful in generating an etiologic hypothesis. Only with better understanding of this disease can we hope in the future to be able to offer treatments other than supportive care and ultimately lung transplantation, which are the only therapeutic options available today.
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