Idiopathic pulmonary fibrosis (IPF) is a chronic interstitial lung disease in which progressive scarring of the lung causes fibrosis and loss of lung function. People with IPF experience dry, persistent cough and progressive fatigue. Patients have a poor prognosis with median survival after diagnosis of only 3 years without effective treatment. Thus, the prognosis for IPF is worse than many types of cancer.

How many have IPF?
Approximately 50,000 new cases of IPF are diagnosed each year. Risk factors include age, gender, tobacco smoking, and family history of pulmonary fibrosis since some genes have been associated with IPF.

How is IPF treated?
Currently, two antifibrotic agents are approved by the FDA and EMA for the treatment of IPF, namely pirfenidone and nintedanib. However, neither of these are able to halt the disease, but merely slow down disease progression. Additionally, both compounds are associated with significant side effects and not all patients show good effect of the treatment.

How is IPF diagnosed?
IPF is diagnosed by lung function tests, a chest X-ray and a high-resolution CT scan to identify the fibrotic pattern associated with IPF. A lung biopsy may also be needed in order to make a diagnosis.

The medical need for biomarkers in IPF is to improve both clinical trials and the treatment of IPF patients by identifying distinct subpopulations. Biomarkers that can identify patient subgroups based on the underlying disease mechanism enabling personalized medicine, as well as monitoring or prediction of anti-fibrotic treatment response in clinical trials are highly desirable.

Changes to the extracellular matrix (ECM) composition leading to chronic scar tissue (fibrosis) is a hallmark of IPF. A normal repair response yields a balance between ECM proteins that are broken down and rebuild to uphold tissue structure and function. Both accumulation of disordered ECM proteins and destructions to the lung tissue occurs in IPF and lead to excessive tissue remodeling. The tissue-derived Protein Fingerprint biomarkers can be measured serologically and used to accurately quantify changes in tissue turnover in the lung in the individual patient.

In the IPF field, a wide range of serological biomarkers have been evaluated but none are used in the clinic. It is becoming more and more evident that IPF is not a common entity but rather a term covering patients with different disease subtypes. Some patients have predominant epithelial involvement where others have interstitial alterations or a genetic predisposition. Different subtypes may respond differently to treatment. Thus, there is still a medical- and drug development need for biomarkers that may characterize and quantify structural tissue change and predict disease progression and treatment response. Tissue derived Protein Fingerprint biomarkers as well as neo-epitope biomarkers of wound healing are highly relevant for assessing the pathological changes occurring in the lungs of IPF patients. Our biomarkers can be used separately or in combination to reflect aberrant lung tissue remodeling, wound healing and inflammation.  

Protein Fingerprint biomarkers measured in serum are generally increased in IPF patients compared to healthy individuals.

ECM alterations quantified by MMP degraded type I, III, and VI collagen and C-reactive protein (C1M, C3M, C6M, CRPM) and type VI collagen formation (PRO-C6) are all increased in IPF patients compared to healthy controls.

References: Jenkins G et al, Lancet Resp Med 2015; Organ L et al, Respir Res. 2019

Protein Fingerprint biomarkers measured in serum are associated with IPF progression, with significantly higher levels in patients that experience FVC decline as compared to those with stable disease. 

References: Jenkins G et al, Lancet Resp Med 2015; Organ L et al, Respir Res. 2019

ECM alterations quantified by MMP degraded type I, III, and VI collagen and C-reactive protein (C1M, C3M, C6M, CRPM) and type III and VI collagen formation (PRO-C3, PRO-C6) are all increased in patients with progressive IPF as compared to stable disease.

Rising concentrations of Protein Fingerprint biomarkers over a three months period are associated with higher risk of mortality in IPF. These biomarkers are dynamic and reflect tissue changes associated with a poor outcome.

Reference: Jenkins RG et al, Lancet Respir Med. 2015

ECM alterations quantified by MMP degraded type I, III, V, and VI collagen, biglycan and C-reactive protein (C1M, C3M, C5M, C6M, BGM, CRPM) are all predictive of all-cause mortality in IPF.

Protein Fingerprint biomarkers can be modulated rapidly with different types of treatment in IPF patients.

Examples: mTOR/PI3Kinase inhibitor by: Nanthakumar CB et al, presented at ATS 2019; Nintedanib+Sildenafil : Eric White presented at ERS 2019

ECM alterations can be dose-dependently modulated by antifibrotic treatments in patients with IPF.

Protein Fingerprint biomarkers are elevated in progressive IPF patients and can be modulated with treatment both in patients and in model systems.

References: Organ L et al, Respir Res. 2019; Nanthakumar CB et al, presented at ATS 2019; Rønnow SR et al, Respir Res 2020; Leeming DJ et al, presented at ERS 2018

Formation of type VI collagen, quantified by PRO-C6, is dose-dependently modulated by antifibrotic treatment across model systems and in IPF patients. Additionally, this biomarker may also identify the patients in most need of treatment.

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