Idiopathic pulmonary fibrosis is a chronic interstitial lung disease in which progressive scarring of the lungs leads to fibrosis and loss of lung function. People with IPF suffer from dry, persistent cough and progressive fatigue. Patients have a poor prognosis with an average survival of only 3 years after diagnosis without effective treatment. Thus, the prognosis for IPF is worse than for many cancers.

How many people have IPF?
About 50,000 new cases of idiopathic pulmonary fibrosis are diagnosed each year. Risk factors include age, gender, tobacco use, and a family history of pulmonary fibrosis, as some genes are associated with IPF.

How is IPF treated?
Currently, two antifibrotic agents are approved by the FDA and EMA for the treatment of idiopathic pulmonary fibrosis, namely pirfenidone and nintedanib. However, neither of these are able to stop the disease but only slow its progression. In addition, both agents are associated with significant side effects and not all patients show a good effect of the treatment.

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

The medical need for biomarkers in idiopathic pulmonary fibrosis is to improve both clinical trials and treatment of IPF patients by identifying distinct subpopulations. Biomarkers that can identify patient subgroups based on the underlying mechanism of disease, thereby enabling personalized medicine, and monitoring or predicting response to antifibrotic treatment in clinical trials are highly desirable.

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Changes in extracellular matrix (ECM) composition leading to chronic scar tissue (fibrosis) are a hallmark of IPF. A normal repair response results in a balance of ECM proteins that are degraded and rebuilt to maintain tissue structure and function.

In idiopathic pulmonary fibrosis, there is both accumulation of disrupted extracellular matrix proteins and destruction of lung tissue, resulting in excessive tissue remodeling. Tissue-derived Protein Fingerprint biomarkers can be measured serologically and used to accurately quantify changes in tissue turnover in the individual patient's lungs.

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In the field of IPF, a variety of serological biomarkers have been studied, but none of them are used in the clinic. It is becoming increasingly clear that idiopathic pulmonary fibrosis is not a single entity, but rather a term that encompasses patients with different disease subtypes.

In some patients, epithelial involvement predominates, whereas in others, interstitial changes or a genetic predisposition are present. The different subtypes may respond differently to treatment. Therefore, there remains a need in medicine and drug development for biomarkers that can characterize and quantify structural tissue changes and predict disease progression and response to treatment.

Tissue-derived Protein Fingerprint biomarkers, as well as neo-epitope biomarkers of wound healing, are important for evaluating pathological changes in the lungs of IPF patients. Our biomarkers can be used individually or in combination to detect abnormal lung tissue remodeling, wound healing, and inflammation.

Protein Fingerprint biomarkers measured in serum are generally elevated in IPF patients compared with healthy individuals.

Extracellular matrix 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 progression of idiopathic pulmonary fibrosis, with levels significantly higher in patients with declining FVC than in patients with stable disease.

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

Extracellular matrix 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

Extracellular matrix 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 rapidly modulated 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

Extracellular matrix 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.

The applications presented here are for research use only.

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