Prognostic prediction score: A method to predict Pleuroparenchymal fibroelastosis

Soumya Shraddhya Paul, Amity University, Noida

Pleuroparenchymal fibroelastosis (PPFE) is an uncommon form of interstitial lung disease (ILD) that is characterised by elastofibrosis in the upper lobes. Patients with PPFE have several distinct clinical characteristics not seen in other ILDs. These are gradual weight loss and restrictive ventilatory dysfunction with increasing residual volume (RV). PPEE patients have a 5-year survival rate ranging from 23.3 to 58.9% and a great majority of patients are observed dying of chronic respiratory failure. Even though the clinical course of PPFE varies greatly from patient to patient, no proven models for predicting the prognosis of PPFE exist.

Pleuroparenchymal fibroelastosis (PPFE) has a wide range of clinical manifestations however, there is no clear prognostic prediction model for the disease. Therefore, to develop a proper prognosis about PPEE a model was developed by Yoshiaki Kinoshita from the Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, along with other researchers (Kinoshita et al.). The main purpose of this model was to find links and help in prognostics along with the overall treatments of the patients. 

Brief about the study:

The prediction model was built using data from two cohorts: the single-center cohort and a nationwide multicenter cohort that included 21 institutions. Prognostic variables were discovered using Cox regression analysis. The weighted sum of the values for the specified variables was used to get the overall score. Harrell’s concordance index (C-index)was used to assess the prediction models’ performance. The gender-age-physiology (GAP) model was also tested for its ability to predict the prognosis of PPFE patients.

Results of this study:

A total of 104 individuals with PPEE were evaluated (52 cases from each cohort). For designing, the following factors were used in the new prognostic prediction model (PPFE Prognosis Score) for PPFE patients: 

• FVC, 
• history of pneumothorax, 
• ILD in the lower lobes, and 
• serum KL-6 level. 

This model outperformed the GAP model in terms of forecasting the death of PPFE patients. 

When can the model be used in prognosis?

There were several limitations to this investigation. Despite the fact that this study included a large number of cases given the rarity of PPFE, the sample size was insufficient to undertake an external validation examination of the model. Second, the model did not account for therapy. For PPFE, no therapies have been demonstrated to be helpful. 

Even with limited data the model still has hope and can be used in prognosis after further development. 

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Reference:

1. Kinoshita, Y., Ikeda, T., Miyamura, T., Ueda, Y., Yoshida, Y., Kushima, H., Fujita, M., Ogura, T., Watanabe, K., & Ishii, H. (2021). A proposed prognostic prediction score for pleuroparenchymal fibroelastosis. Respiratory Research, 22(1), 215. https://doi.org/10.1186/s12931-021-01810-z

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About Author:

Soumya Shraddhya Paul is an undergrad biotechnology student who worked in building 3D prosthetics in Base Hospital Delhi Cantt and holds a key interest in nutraceuticals and enzymology. 

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Social Media Info: www.linkedin.com/in/soumya-shraddhya-paul-858229203