Changes in body weight reflect disease progression in pleuroparenchymal fibroelastosis

Kinoshita, Yoshiaki; Utsunomiya, Takuhide; Koide, Yohei; Wada, Kenji; Ueda, Yusuke; Yoshida, Yūji; Kushima, Hisako; Ishii, Hiroshi

doi:10.1016/j.resmer.2022.100980

Cited by 3 publications

(8 citation statements)

References 15 publications

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“…Because the annual % change in body weight was found to correlate with the annual change in FVC, changes in body weight may help estimate the decline in FVC in PPFE patients [51]. Decreased BMI was related to disease progression [16], and we also reported body weight loss to be a simple and useful prognostic indicator of nintedanib therapy in ILD patients [53].…”

Section: Disease Behavior and Monitoring Markersmentioning

confidence: 69%

“…However, it is difficult to perform pulmonary function tests on patients with advanced PPFE because of their severely impaired pulmonary function and increased risk for pneumothorax [50]. Therefore, a non-invasive and simple method of estimating the longitudinal decline in FVC would be highly important in the management of PPFE patients [51]. In other words, unlike with IPF, FVC is not an appropriate-enough surrogate marker for monitoring the disease course in PPFE [3,19].…”

Section: Disease Behavior and Monitoring Markersmentioning

confidence: 99%

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Proposed Clinical Algorithm for Pleuroparenchymal Fibroelastosis (PPFE)

Yamakawa,

Oda,

Sugino

et al. 2024

JCM

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Pleuroparenchymal fibroelastosis (PPFE) is characterized by fibrosis involving the pleura and subpleural lung parenchyma, predominantly in the upper lobes. As PPFE appears to occur in patients with heterogeneous etiologies, the disease course is thus also heterogenous, with some patients showing rapid progression while others have slow progression. Therefore, it is very difficult to predict prognosis with PPFE. Needless to say, this problematic matter has influenced the treatment strategy of PPFE patients. In fact, until now no evidence has been shown for use in creating an appropriate management algorithm for PPFE. We speculate that “uncoordinated breathing” is the most important reason for dyspnea in PPFE patients. Because monitoring of physique and not just pulmonary function and radiological evaluation is also very important, particularly in PPFE patients, this review focused on the characteristics of PPFE through an overview of previous studies in this field, and we proposed an algorithm as precision medicine based on the current evidence. Multiple views by the pulmonologist are needed to standardize a clinical algorithm that is necessary to correctly assess PPFE patients under the premise of maintenance of physique by providing appropriate nutritional care and pulmonary rehabilitation.

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Section: Disease Behavior and Monitoring Markersmentioning

confidence: 69%

Section: Disease Behavior and Monitoring Markersmentioning

confidence: 99%

Proposed Clinical Algorithm for Pleuroparenchymal Fibroelastosis (PPFE)

Yamakawa,

Oda,

Sugino

et al. 2024

JCM

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“…These data have highlighted the use of nintedanib for PPFE (19 out of 1120, 1.7%). Both PPFE and nintedanib use are associated with weight loss and the nintedanib rate of adverse events of in this population is unknown [ 14 , 15 ]. Evidence for the use of nintedanib in PPFE is limited to conflicting retrospective reports, highlighting the need for prospective, controlled studies [ 16 , 17 ].…”

Section: Discussionmentioning

confidence: 99%

Real-world experience of nintedanib for progressive fibrosing interstitial lung disease in the UK

Dixon,

Hague,

Mulholland

et al. 2024

ERJ Open Res

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BackgroundNintedanib slows progression of lung function decline in patients with progressive fibrosing (PF) interstitial lung disease (ILD) and was recommended for this indication within the United Kingdom (UK) National Health Service in Scotland in June 2021 and in England, Wales and Northern Ireland in November 2021. To date, there has been no national evaluation of the use of nintedanib for PF-ILD in a real-world setting.Methods26 UK centres were invited to take part in a national service evaluation between 17 November 2021 and 30 September 2022. Summary data regarding underlying diagnosis, pulmonary function tests, diagnostic criteria, radiological appearance, concurrent immunosuppressive therapy and drug tolerability were collectedviaelectronic survey.Results24 UK prescribing centres responded to the service evaluation invitation. Between 17 November 2021 and 30 September 2022, 1120 patients received a multidisciplinary team recommendation to commence nintedanib for PF-ILD. The most common underlying diagnoses were hypersensitivity pneumonitis (298 out of 1120, 26.6%), connective tissue disease associated ILD (197 out of 1120, 17.6%), rheumatoid arthritis associated ILD (180 out of 1120, 16.0%), idiopathic nonspecific interstitial pneumonia (125 out of 1120, 11.1%) and unclassifiable ILD (100 out of 1120, 8.9%). Of these, 54.4% (609 out of 1120) were receiving concomitant corticosteroids, 355 (31.7%) out of 1120 were receiving concomitant mycophenolate mofetil and 340 (30.3%) out of 1120 were receiving another immunosuppressive/modulatory therapy. Radiological progression of ILD combined with worsening respiratory symptoms was the most common reason for the diagnosis of PF-ILD.ConclusionWe have demonstrated the use of nintedanib for the treatment of PF-ILD across a broad range of underlying conditions. Nintedanib is frequently co-prescribed alongside immunosuppressive and immunomodulatory therapy. The use of nintedanib for the treatment of PF-ILD has demonstrated acceptable tolerability in a real-world setting.

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“…[2,3] Pulmonary physiology in patients with iPPFE is notable due to the markedly decreased VC and increased RV/TLC ratio. [1,[4][5][6] This deviation from the typical pattern observed in most patients with restrictive lung diseases raises questions regarding the mechanism underlying increased RV/TLC ratio in patients with iPPFE.…”

Section: To the Editormentioning

confidence: 97%

“…We conducted an observational retrospective study including patients diagnosed with iPPFE between 2011 and 2022 at the Fukuoka University Chikushi Hospital (Japan). The inclusion criteria were patients aged 20 years or older who were diagnosed according to the proposed criteria for iPPFE [5] and who underwent simultaneous chest computed tomography (CT) and pulmonary function tests at diagnosis. Patients with concurrent pneumonia, pneumothorax, pneumomediastinum, or lung cancer detected on CT examination at diagnosis were excluded.…”

Section: To the Editormentioning

confidence: 99%