Lung Clearance Index to Identify Scleroderma Patients at Risk for ILD

Lung Clearance Measurement to Determine Pre-clinical Airway Involvement in Scleroderma Patients Without Lung Disease as a Risk Factor for Developing ILD

This National Jewish Health (NJH) Investigator Initiated pilot study funded by the Shah Foundation will prospectively perform a non invasive lung function test called Lung Clearance Index (LCI) to determine if 50 scleroderma patients without evidence of lung disease who have evidence of small airway inflammation or impairment to airflow are more likely to develop ILD than those with normal vales.50 subjects will be enrolled from the Rheumatology practice at NJH and followed with phone interviews or Electronic Medical Record (EMR) record review yearly times 5 to determine if they have subsequently developed evidence of ILD or pulmonary artery hypertension.

Study Overview

• Status: Recruiting
• Conditions: Scleroderma
• Intervention / Treatment: Diagnostic test: LCI testing

Detailed Description

50 scleroderma patients without evidence of lung disease who have been seen by an NJH Rheumatologist will be invited to participate. They will have their medical records reviewed to determine eligibility and then perform hand held spirometry and LCI testing. This measures evidence of small airway airflow obstruction and requires normal breathing while first on room air followed by inhaling a gas mixture of 100% oxygen. The Oxygen concentration in exhaled breathing and the number of breaths needed to return to room air concentration of Oxygen will be analyzed by a computer. Medical information from the EMR will be recorded and placed in a secure database, REDCap, for statistical analysis to determine if LCI results or other clinical features are predictive of which Scleroderma patients are most likely to subsequently develop evidence of Interstitial Lung disease (ILD) based upon yearly phone call interviews and EMR record reviews within 5 years of baseline testing.

• Study Type: Observational
• Enrollment (Anticipated): 50

Contacts and Locations

• Study Contact: Name: Richard T meehan, MD; Phone Number: 303 3981-1475; Email: meehanr@njhealth.org
• Study Contact Backup: Name: Mary Gill, RN; Phone Number: 720 936-9619; Email: gillm@njhealth.org

Study Locations

• United States
  • Colorado
    • Denver, Colorado, United States, 80206
      • Recruiting
      • National Jewish Health
      • Contact: Richard T Meehan, MD; Phone Number: 303-398-1475; Email: meehanr@njhealth.org
      • Sub-Investigator: Mehrnaz Maleki, MD
      • Sub-Investigator: Liudmila Kastsianok, MD

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 21 years to 75 years (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

Scleroderma patients without lung disease

Description

Inclusion Criteria:

• Confirmed diagnosis of systemic sclerosis (SSC) by an NJH Rheumatologist
• age 21-75
• No evidence of Scleroderma related Lung disease (ILD)
• Able to comprehend and sign an Institutional Review Board (IRB) approved consent form.

Exclusion Criteria:

• Patients without a Rheumatologists confirming a diagnosis (DX) of SSC
• Patients with evidence of lung involvement based upon; Thoracic imaging, abnormal Pulmonary Function Tests (PFTs); Forced Vital Capacity( FVC), Forced Expiratory Volume in 1 second (FEV1) or a Diffusion Capacity for Carbon Monoxide (DLCO) < the Lower Limit of Normal (LLN) or Saturation of Oxygen in arterial blood (SaO2) < 90% .
• Cardiac echocardiogram or right heart catheter evidence of Pulmonary Arterial Hypertension (PAH).
• < 21 or > 75 years of age
• Must not be pregnant or had eye surgery within 2 weeks of LCI testing
• Must be able to comprehend and sign an IRB approved consent form and complete LCI testing

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Prospective

Number of groups / cohorts

1

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
LCI testing; LCI testing will be performed on all study subjects	Diagnostic test: LCI testing; all subjects will have LCI testing then contacted via a phone interview or EMR review yearly times 5 to determine if they have subsequently developed ILD; Other Names: no drugs will be administered

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
The Utility of Lung Clearance Index Scores at predicting Interstitial Lung disease ( ILD) development within 5 years among Scleroderma patients without ILD.	Lung clearance index values (LCI) will be measured from all Scleroderma patients at baseline (normal value is < 7 ). The LCI scores from the cohort of Scleroderma patients who subsequently develop ILD based upon physician review of the EMR or phone interviews will be compared to the LCI scores from those scleroderma patients who did not develop ILD. Statistical analysis of LCI scores between both cohorts will determine the potential clinical utility of this non-invasive measurement of lung function in identifying those scleroderma patients who are at greatest risk for the subsequent development of ILD so treatment can be initiated early in their disease before irreversible pulmonary fibrosis develops.	5 years after baseline testing

Collaborators and Investigators

• Sponsor: National Jewish Health
• Investigators: Principal Investigator: Richard T Meehan, MD, National Jewish Health

Study record dates

Study Major Dates

• Study Start (Actual): January 4, 2021
• Primary Completion (Anticipated): January 15, 2026
• Study Completion (Anticipated): January 15, 2026

Study Registration Dates

• First Submitted: August 12, 2020
• First Submitted That Met QC Criteria: August 31, 2020
• First Posted (Actual): September 1, 2020

Study Record Updates

• Last Update Posted (Actual): March 24, 2023
• Last Update Submitted That Met QC Criteria: March 22, 2023
• Last Verified: March 1, 2023

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Skin Diseases; Connective Tissue Diseases; Scleroderma, Systemic; Scleroderma, Diffuse; Scleroderma, Localized
• Other Study ID Numbers: HS 3471

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

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