From Classification to disease activity
By Dr. Sandra Langer, Fujirebio
Previous posts in this series:
- KL-6 in Lung Diseases: Current classification of interstitial lung diseases
- ILD diagnosis – current professional practice
- Use of serum biomarkers in progressive fibrosing ILDs
- KL-6 in disease prognosis
Download the complete guidance document by following this link (requires a Premium eServices account).
- You will find more resources about ILDs and the KL-6 biomarker in our dedicated microsite at www.ay05.cn/kl-6 (opens in a new window).
Emergence of a new paradigm for assessing disease activity in chronic pulmonary fibrosis
- Focus on disease activity instead of classification.
- KL-6 can help to get a clearer picture of the pathophysiological proceedings in the lung and the disease activity that lead to different courses of the disease.
While IPF is the classic fibrosing ILD, clinical data suggest that there is a larger group of patients with differing clinical ILD diagnoses who develop a progressive fibrosing phenotype during the course of their disease. These patients demonstrate a number of similarities to IPF, with their disease being defined by the presence of progressive pulmonary fibrosis, worsening respiratory symptoms, declining lung function, resistance to immunomodulatory therapies and, ultimately, early mortality. These patients can be described as patients with progressive fibrosing interstitial lung disease.1
Pulmonary function parameters at a single time point do not reliably predict disease behavior and, despite multiple attempts, HRCT-quantified disease extent on sequential imaging has not been established as a reliable marker of disease progression.2 Serum biomarkers, such as KL-6 can help to get a clearer picture of the pathophysiological proceedings in the lung and the disease activity that lead to different courses of the disease.
With KL-6 we don’t focus on different classes within ILD but on the severity and activity of the disease.
- Flaherty, Kevin R., et al. "Design of the PF-ILD trial: a double-blind, randomised, placebo-controlled phase III trial of nintedanib in patients with progressive fibrosing interstitial lung disease." BMJ open respiratory research 4.1 (2017): e000212.
- Robbie, Hasti, et al. "Evaluating disease severity in idiopathic pulmonary fibrosis." European Respiratory Review 26.145 (2017): 170051.