Collagen Fingerprinting for Stratification of Pulmonary Hypertension (PH) Patients

Chronic lung diseases such as pulmonary fibrosis and chronic obstructive pulmonary disease (COPD) can lead to pulmonary hypertension. This serious complication involves increased pressure in the lung vessels, which strains the heart and worsens outcomes. Since the early symptoms are unclear, diagnosis often occurs too late, underscoring the need for simple, noninvasive methods of early detection.

A key driver of the disease is vascular remodeling, which involves the narrowing and stiffening of blood vessels. This process involves changes in the extracellular matrix, particularly in the understudied basement membrane. Our project examines how specific components, especially non-classical collagens, change during disease progression. As vessels remodel, detectable fragments enter the bloodstream, potentially creating a molecular fingerprint of the disease.

By analyzing lung tissue and blood samples, the investigators aim to identify non-invasive biomarkers for earlier diagnosis, better patient classification, and more personalized treatment.

Study Overview

• Status: Recruiting
• Conditions: Pulmonary Hypertension Due to Lung Diseases

Detailed Description

Pulmonary hypertension (PH) is a severe complication of chronic lung diseases (CLDs) that significantly worsens patient outcomes. Although extracellular matrix (ECM) remodeling is central to PH pathogenesis, the basement membrane (BM)-a specialized ECM-remains understudied. The BM's dynamic role in lung tissue organization and signaling may provide insight into disease progression and phenotypic heterogeneity.

The investigators hypothesize that BM remodeling and the release of its bioactive components generate a measurable molecular fingerprint reflecting PH onset and progression in CLD. This study aims to prove the concept of using BM-derived fingerprints for disease detection and patient stratification.

• Study Type: Observational
• Enrollment (Estimated): 80

Contacts and Locations

• Study Contact: Name: Grazyna Kwapiszewska-Marsh, PhD; Phone Number: 004331638572918; Email: grazyna.kwapiszewska-marsh@medunigraz.at
• Study Contact Backup: Name: Johannes Lorenz Berg, PhD; Phone Number: 004331638572917; Email: johannes.berg@medunigraz.at

Study Locations

• Austria
  • Styria
    • Graz, Styria, Austria, 8010
      • Recruiting
      • Medical University of Graz
      • Contact: Nikolaus Kneidinger, MD; Phone Number: 004331638513305; Email: nikolaus.kneidinger@medunigraz.at

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Adult; Older Adult
• Accepts Healthy Volunteers: Yes

• Sampling Method: Non-Probability Sample

Study Population

Patients undergoing lung transplantation with COPD or PF, with or without associated PH or PAH.

Outpatient cohort with COPD or PF, with or without associated PH or PAH.

Description

Inclusion criteria:

Patients undergoing lung transplantation with COPD or PF, with or without associated PH and PAH.

- Outpatient cohort with COPD or PF, with or without associated PH or PAH.

Exclusion criteria:

• Presence of other lung diseases
• Signs of infection, such as pneumonia, pulmonary tuberculosis, or pleural effusions.

Study Plan

How is the study designed?

Number of groups / cohorts

4

Cohorts and Interventions

Group / Cohort
Patients with confirmed COPD with or without pulmonary hypertension (COPD/PH group); Tissue Sampling Blood is collected via venipuncture into citrate- or ethylenediaminetetraacetic acid (EDTA)-containing Vacutainer tubes, as well as into serum tubes. Obtain explant lung tissue from patients who have undergone lung transplantation.
Patients with confirmed pulmonary fibrosis with or without pulmonary hypertension (PF/PH group); Tissue Sampling Blood is collected via venipuncture into citrate- or ethylenediaminetetraacetic acid (EDTA)-containing Vacutainer tubes, as well as into serum tubes. Obtain explant lung tissue from patients who have undergone lung transplantation.
Patients with confirmed pulmonary arterial hypertension (PAH group); Tissue Sampling Blood is collected via venipuncture into citrate- or ethylenediaminetetraacetic acid (EDTA)-containing Vacutainer tubes, as well as into serum tubes. Obtain explant lung tissue from patients who have undergone lung transplantation.
Control group without diagnosed COPD, PF or pulmonary hypertension (Control group); Tissue Sampling Blood is collected via venipuncture into citrate- or ethylenediaminetetraacetic acid (EDTA)-containing Vacutainer tubes, as well as into serum tubes. Explant lung tissue is obtained from patients who have undergone lung transplantation. Non-transplanted donor lungs that were harvested for transplantation but not implanted due to size reduction serve as controls.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Analysis of collagen fragments	The fragments will be measured in the plasma, serum, or tissue using an enzyme-linked immunosorbent assay (ELISA), mass spectrometry, or single-cell analysis.	within 48 months

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Disease severity assessment	The assessment of disease severity will be based on the associated clinical data and compared to the generated collagen fingerprint, as determined by the primary outcome.	within 48 months

Collaborators and Investigators

• Sponsor: Medical University of Graz
• Collaborators: Medical University of Vienna
• Investigators: Study Chair: Nikolaus Kneidinger, MD, Medical University of Graz

Study record dates

Study Major Dates

• Study Start (Estimated): July 1, 2026
• Primary Completion (Estimated): June 30, 2030
• Study Completion (Estimated): June 30, 2030

Study Registration Dates

• First Submitted: March 23, 2026
• First Submitted That Met QC Criteria: March 23, 2026
• First Posted (Actual): March 27, 2026

Study Record Updates

• Last Update Posted (Actual): April 3, 2026
• Last Update Submitted That Met QC Criteria: March 30, 2026
• Last Verified: March 1, 2026

More Information

Terms related to this study

• Keywords: Collagens
• Other Study ID Numbers: 10.55776/KLP3022825

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: UNDECIDED

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No