RSV and Heart Health
What Is Nonspecific Interstitial Pneumonia, A Rare Disorder?
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Nintedanib Slows Symptom Worsening In Progressive Pulmonary Fibrosis - Healio
February 13, 2024
3 min read
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Key takeaways:Among patients with progressive pulmonary fibrosis, taking nintedanib for 52 weeks lowered the degree of worsened dyspnea, fatigue and cough compared with placebo, according to results published in European Respiratory Journal.
"Changes in [Living with Pulmonary Fibrosis (L-PF)] questionnaire scores over 52 weeks suggested that, in patients with [progressive pulmonary fibrosis], nintedanib slowed the worsening of dyspnea, fatigue and cough and the impacts of the disease on patients' lives," Marlies Wijsenbeek, MD, PhD, head of the Center of Excellence for Interstitial Lung Diseases and Sarcoidosis at Erasmus Medical Center-University Medical Center Rotterdam, and colleagues wrote.
Data were derived from Wijsenbeek M, et al. Euro Respir J. 2024;doi:10.1183/13993003.00752-2023.
Using data from the INBUILD trial, Wijsenbeek and colleagues assessed 663 patients (mean age, 65.8 years; 53.7% men) with progressive pulmonary fibrosis to determine the impact of nintedanib on L-PF questionnaire scores after 52 weeks of treatment.
On high-resolution CTs, over half of the cohort (62.1%) had a usual interstitial pneumonia-like fibrotic pattern. Researchers further observed several ILD diagnoses in the total cohort, including high prevalences of hypersensitivity pneumonitis (26.1%) and autoimmune disease-related ILDs (25.6%), followed by diagnoses of idiopathic non-specific interstitial pneumonia (NSIP; 18.9%), unclassifiable idiopathic interstitial pneumonia (IIP; 17.2%) and other ILDs (12.2%).
Within the study population, 332 patients received nintedanib and 331 received placebo.
When evaluating changes in the L-PF total score from baseline to week 52, in which increases signal worsening, researchers found that patients receiving placebo had larger score increases vs. Patients receiving nintedanib (adjusted mean, 5.1 points vs. 0.5 points) in mixed models.
This outcome was further observed when evaluating changes in adjusted mean L-PF sub-scores, with a lower degree of worsening in those receiving nintedanib compared with placebo in the L-PF symptoms score (1.3 points vs. 5.3 points), dyspnea score (4.3 points vs. 7.8 points) and fatigue score (0.7 points vs. 4 points).
For the L-PF cough score, researchers found that the nintedanib group achieved a reduction of 1.8 points between baseline and week 52, whereas this score rose by 4.3 points in the placebo group. A similar pattern was observed in the L-PF impacts score (–0.2 points vs. 4.6 points).
When the total cohort was divided into two groups — those with usual interstitial pneumonia-like fibrotic pattern on high-resolution CT and those with other fibrotic patterns on high-resolution CT — patients receiving nintedanib in each group continued to show less worsening in L-PF scores vs. Placebo.
Across all ILD diagnoses groups, patients receiving placebo had poorer changes in the L-PF total score vs. Patients receiving nintedanib:
As a final assessment, researchers compared the proportion of patients from the nintedanib group who met thresholds signaling worsening in terms of dyspnea and cough L-PF scores with the proportion of patients from the placebo group.
An increase in the L-PF dyspnea score of 6 points or higher occurred less frequently in the nintedanib vs. Placebo group (36.8% vs. 46.4%), as did an increase of 7 points or higher (34.7% vs. 43%).
Similarly, an increase in the L-PF cough score of 4 points or higher was less prevalent in the nintedanib vs. Placebo group (41.6% vs. 50.6%), as was an increase of 5 points or higher (34.6% vs. 46.3%).
"The meaningful change thresholds for deterioration in L-PF questionnaire dyspnea and cough scores need validation in other cohorts," Wijsenbeek and colleagues wrote. "While these data are encouraging, research in other cohorts is needed into the impact of nintedanib on dyspnea, cough and fatigue in patients with pulmonary fibrosis."
Perspective Back to Top An individual living with ILD experiences daily symptoms (eg, cough, dyspnea, exercise intolerance, fatigue) that uniquely affect their quality of life and overall well-being. While both approved and non-approved therapies modify the course of lung function in patients with ILD, few therapies are proven to improve these daily symptoms. Using a relatively new patient-reported outcome measure developed specifically for an ILD population (ie, L-PF), the authors of this study demonstrated that treatment with nintedanib was associated with smaller increases (worsening) in L-PF scores compared with placebo in patients with progressive forms of ILD. This study included 663 patients with ILDs other than idiopathic pulmonary fibrosis, including hypersensitivity pneumonitis, autoimmune disease-related ILD, idiopathic NSIP, as well as unclassifiable and other ILDs. With the exception of the cough domain, scores for the symptoms, dyspnea, fatigue and impact domains worsened in all patients in the study, which may reflect the fact that this study specifically enrolled patients with a progressive ILD phenotype. The degree of worsening, however, was notably and significantly less in patients who received nintedanib. These findings are important to disseminate to doctors, as they may guide benefit vs. Risk discussions with patients considering treatment with nintedanib. Another interesting observation was that patients with autoimmune ILDs (n = 165) had relatively minimal change in their total L-PF scores over the course of the 52-week study. Baseline L-PF scores were not provided for the autoimmune ILD cohort. However, this finding could suggest that this questionnaire requires further validation in an autoimmune ILD cohort, particularly because extra-pulmonary features of autoimmune diseases can affect symptoms associated with ILD (eg, cough, fatigue, exercise tolerance). In my experience, nintedanib is typically used in combination with other immunomodulatory therapies in patients with autoimmune ILD. However, the present study excluded patients taking commonly prescribed immunomodulatory therapies (eg, mycophenolate, rituximab). Future studies are needed to evaluate the performance of the L-PF in a real-world cohort of patients with autoimmune ILDs. Elizabeth Volkmann, MD, MS Associate Professor and Director, UCLA Scleroderma Program Co-Director, CTD-ILD Program at University of California, Los AngelesDisclosures: Volkmann reports consulting for Boehringer Ingelheim and GSK, as well as receiving grants from Boehringer Ingelheim, Horizon and Prometheus.
Sources/DisclosuresCollapse Disclosures: Wijsenbeek reports receiving grants to her institution from AstraZeneca/Daiichi Sankyo, BI, Hoffmann-La Roche, Sarcoidosis.Nl, The Dutch Lung Foundation, The Dutch Pulmonary Fibrosis Patients Association, The Netherlands Organisation for Health Research and Development and The Thorax Foundation; and consulting/speaker fees from AstraZeneca, BI, Bristol Myers Squibb, CSL Behring, Galapagos, Galecto, Hoffmann-La Roche, Horizon Therapeutics, Kinevant Sciences, Molecure, NeRRe, Novartis, PureTech, Thyron, Trevi and Vicore. Please see the study for all other authors' relevant financial disclosures.Add topic to email alerts
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Researchers Describe Concomitant Interstitial Lung Disease With Psoriasis - AJMC
A recent case series examined clinical and radiographic characteristics of patients with concomitant psoriasis and interstitial lung disease (ILD).
A recent case series examined clinical and radiographic characteristics of patients with concomitant psoriasis and interstitial lung disease (ILD).
ILD is defined as any lung disease occurring in the parenchymal interstitium (ie, the alveolar wall or alveolar septa) or loose-binding connective tissue. Type 17 helper T cells (Th17) are involved in both ILD and psoriasis and may share an underlying immunologic pathway. In ILD, they are reported to be one of the common pathways which contribute to alveolitis and enhance cytokine production in pulmonary fibroblasts, the authors wrote.
In this retrospective review of one institution's experiences of ILD concomitant with psoriasis, researchers extracted clinical, radiographic, pathological, and outcome data from medical records and identified 21 (4.7%) out of 447 patients with ILD who also had a previous or current diagnosis of psoriasis. Patients had a median age of 66 and most (66.7%) were male. A little more than 71% were current or former smokers. The most common symptom on presentation was "cough," followed by "dyspnea on exertion." Thirteen (61.9%) had not previously or concomitantly been exposed to immunosuppressive therapy directed against psoriasis; 2 eventually died.
The clinical diagnosis of ILD included:
The radiographic findings included:
The cases suggest a possible direct association between ILD and psoriasis, the researchers wrote. A link between psoriasis and chronic obstructive pulmonary disease has recently been seen, and the authors wrote that psoriasis-related immune dysfunction could lead to an abnormal immunologic response in the lung parenchyma, perhaps due to autoimmune-related pathogenesis where immune dysfunction in patients with psoriasis similarly causes inflammation and fibrotic process in the lung interstitium.
Discussing the radiographic findings, the authors noted that the UIP pattern was more common (42.9%) than the NSIP/OP pattern, which might suggest that immune dysfunction in the lungs triggered by psoriasis tends to cause fibrotic change rather than the conventional inflammatory process. They also said the findings resemble the patterns observed in rheumatoid arthritis, in which a radiographic UIP pattern is the most common; the 2 different diseases might have a common respiratory pathophysiology with shared clinical similarities.
In addition, compared with conventional IPF patients, the reduction of pulmonary function was mild, as shown by a modest change in the rate of predicted forced vital capacity (range, −5.7% to 18% per year); this implies that there is a distinct feature of IPF associated with psoriasis.
Further prospective studies are needed to determine the association between ILD and psoriasis, the researchers said.
Reference
Ishikawa G, Dua S, Mathur A, et al. Concomitant interstitial lung disease with psoriasis [published online August 25, 2019]. Can Respir J. Doi: 10.1155/2019/5919304.
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