Is it possible to predict survival time based on laboratory or radiological findings in patients with IPF?
High-resolution computed tomography (HRCT) imaging of the lungs is a fundamental examination in diagnosing idiopathic pulmonary fibrosis (IPF). According to experts from the Mayo Clinic, it can also serve as a tool for more accurate prediction of survival time in IPF patients.
The definition of IPF includes histopathological and radiological changes
According to the current recommended guidelines, IPF is defined as a specific form of chronic progressive fibrosing interstitial pneumonia of unknown etiology. IPF is associated with a histopathological and/or radiological pattern of UIP (usual interstitial pneumonia).
Prognosis comparable to bronchogenic carcinoma
IPF is a lethal lung disease with a prognosis similar to that of bronchogenic carcinoma. The estimated median survival time at the diagnosis of IPF is 2.5–3 years. The 5-year survival rate for IPF patients is 20–40%; the current 5-year survival rate for non-small cell bronchogenic carcinoma is 18%.
Can the prognosis be predicted based on laboratory or radiological signs?
Biomarkers
For predicting prognosis and monitoring the risk of acute exacerbation, some centers use the following biomarkers. However, these markers cannot be routinely recommended for monitoring and therapeutic decisions.
- Krebs von den Lungen 6 (KL-6): Among mucins, it is produced by regenerating type II pneumocytes and is elevated in the serum and bronchoalveolar lavage fluid (BALF) of IPF patients, correlating with the mortality of IPF patients.
- Surfactant proteins A and C (SP-A, C), chemokine CCL-18, BNP, and matrix metalloproteinase 7 (MMP-7): These may have predictive value in IPF patients.
- Circulating fibrocytes in peripheral blood: Representing a negative prognostic factor for short-term survival in IPF patients.
Radiological changes
Experts at the Mayo Clinic developed a software tool for use in the CALIPER program that automatically evaluates findings. This tool analyzes and subsequently quantifies abnormalities in the lung parenchyma as displayed on HRCT scans. The study was conducted on 55 patients with idiopathic pulmonary fibrosis, and their radiological results obtained by this method were correlated with clinical outcomes. Reticular changes and overall interstitial abnormalities were evaluated. These data proved to be independent predictors of survival and mortality in IPF patients.
Accurate prognosis assessment in IPF patients is complex
Accurately assessing the prognosis in IPF patients remains complex due to significant individual radiological and clinical variability.
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Sources:
1. Vašáková M. Idiopathic pulmonary fibrosis − changes in diagnosis and treatment. Cardiology Review 2019; 21 (3): 135−141.
2. Maldonado F., Moua T., Rajagopalan S. et al. Automated quantification of radiological patterns predicts survival in idiopathic pulmonary fibrosis. Eur Respir J 2014; 43 (1): 204−212, doi: 10.1183/09031936.00071812.
3. Vašáková M. Treatment of idiopathic pulmonary fibrosis. Remedia 2012; 22 (6): 398−402.
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