Clinical Characteristics of Interstitial Pneumonia With Autoimmune Features (IPAF) - a Multicenter Prospective Study
Incidence, Clinical Characteristics and Evaluation of Prognostic and Diagnostic Markers of Interstitial Pneumonia With Autoimmune Features (IPAF) - a Multicenter Prospective Study
1 other identifier
observational
210
2 countries
7
Brief Summary
Interstitial pneumonia with autoimmune features (IPAF) was defined in 2015 by the Working Group of the European Respiratory Society (ERS) and the American Thoracic Society (ATS) as interstitial pneumonia with some clinical and/or serological features suggesting presence of an underlying autoimmune disorder. However, ofiicial criteria for diagnosis of an autoimmune disease are not met. Aims of the study:
- 1.Determine the incindence of IPAF in comparison with interstitial lung diseases (ILDs) and classic autoimmune diseases (ADs) in polish pulmonological centers.
- 2.Clinical, serological, functional and radiological and histopathological characteristics of IPAF patients.
- 3.Analysis of diagnostic strategies towards specific IPAF subgroups.
- 4.Characterictics of potencial diagnostic, predictive and prognostic features of IPAF.
- 5.Prospective assessment of IPAF patients in the courseof 5 years in order to determine stability of the diagnosis and potential progression to other diseases, e.g. ADs.
Trial Health
Trial Health Score
Automated assessment based on enrollment pace, timeline, and geographic reach
participants targeted
Target at P75+ for all trials
Started Mar 2019
Longer than P75 for all trials
7 active sites
Health score is calculated from publicly available data and should be used for screening purposes only.
Trial Relationships
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Study Timeline
Key milestones and dates
First Submitted
Initial submission to the registry
March 10, 2019
CompletedFirst Posted
Study publicly available on registry
March 12, 2019
CompletedStudy Start
First participant enrolled
March 18, 2019
CompletedPrimary Completion
Last participant's last visit for primary outcome
February 18, 2020
CompletedStudy Completion
Last participant's last visit for all outcomes
December 31, 2025
CompletedMarch 12, 2019
March 1, 2019
11 months
March 10, 2019
March 10, 2019
Conditions
Keywords
Outcome Measures
Primary Outcomes (1)
Identification of IPAF diagnostic markers
It is still unclear whether there are diagnostic markers specific for IPAF or whether there is a significant difference in concentration of fibrosis biomarkers in IPAF, CTD-ILD and ILD groups. Both blood and BAL biomarkers will be taken under consideration, they include chemokine C-C motif ligand 18 (CXCL18), Surfactant Protein A- (SP-A), Surfactant Protein D (SP-D), Krebs von den Lungen-6 protein (KL-6) and chitotrisidase 1 (CHIT1).
10.2019-10.2020
Study Arms (3)
Study group IPAF
Patients with IPAF which is defined according to the Work Group of the European Respiratory Society/American Thoracic Society. The interventions to be administered include:bronchoalveolar lavage and taking bronchial mucosa samples lung function tests,6 minute walk test, use of cough and dyspnea scales, transthoracic echocardiography, blood testing, arterial blood gas and pulse oximetry
Control group CTD-ILD
Patients with connective tissue disease associated intestitial lung disease: rheumatoid arthritis - RA, systemic sclerosis - SSc, polymyositis - PM, dermatomyositis - DM, (anti-synthetase syndrome - AS, Sjögren's syndrome - SjS, mixed connective tissue disease - MCTD ,systemic lupus erythematosus - SLE, diagnosed according to diagnostic criteria issued by European League Against Rheumatism (EULAR) and/or American College of Rheumatology (ACR)
Control group ILD
Idiopathic interstitial pneumonia group: idiopathic pulmonary fibrosis - IPF, nonspecific interstitial pneumonia - NSIP, cryptogenic organizing pneumonia - COP, acute interstitial pneumonia - AIP; respiratory bronchiolitis associated interstitial lung disease - RB-ILD, desquamative interstitial pneumonia - DIP, lymphocytic interstitial pneumonia - LIP).
Interventions
Regional anesthesia and sedation with use of lidocaine and midazolam will be performed, according to anesthesia protocols applied in respective endoscopy units. Intravenous cannula will be inserted prior to BF. During endoscopy, the patient will be monitored according to safety protocols applied in respective endoscopy units. Bronchoalveolar lavage will be performed in the bronchus from the middle robe of right lung or the lingula of left lung (B4, B5). Localization will be chosen based on HRCT results and will be recorded in patient's medical history. Sterile solution of 0, 9% NaCl will be instilled. 200 ml fluid will be applied with a syringe in portions of 25 ml or 50 ml. It is recommended that minimum 60 % of the lavage fluid is retrieved.
a submaximal exercise test which entails measurement of distance walked over a span of 6 minutes. Blood pressure, pulse oximetry are measured directly before and after the test. The participant is also periodically asked about their dyspnea sensation
If SpO2 is measured to be \< 92%, an artery (radial or femoral) is punctured in order to take a sample of arterial blood. Then, the artery is compressed in order to prevent bleeding/ hematoma.
A vein will be punctured in order to take a blood sample for further tests
Eligibility Criteria
The study population will be recruited from patients hospitalized in pneumonology wards in clinical centers participating in the study. They will be divided into 3 subgroups: Study group - IPAF. 2. Control groups: 1. Connective tissue disease associated interstitial lung disease (CTD-ILD) patients: 2. Idiopathic interstitial pneumonia group
You may qualify if:
- written informed consent to participate in the study
- diagnosis of one of interstitial lung diseases/autoimmune diseases as described below
You may not qualify if:
- age \< 18 years
- withdrawal od consent
- pregnancy
- lactation
- infectious disease 4 weeks prior
Contact the study team to confirm eligibility.
Sponsors & Collaborators
Study Sites (7)
Universita' degli Studi di Messina
Messina, Province Of Messina, 98122, Italy
Katedra i Klinika Pulmonologii, Alergologii i Onkologii Pulmonologicznej Uniwersytetu Medycznego im. K. Marcinkowskiego w Poznaniu
Poznan, Greater Poland Voivodeship, 60-514, Poland
II Katedra Chorob Wewnetrznych Uniwersytetu Jagiellonskiego Collegium Medicum im. prof. A. Szczeklika Szpitala Uniwersyteckiego
Krakow, Lesser Poland Voivodeship, 31-066, Poland
Katedra i Klinika Pneumonologii, Onkologii i Alergologii Uniwersytetu Medycznego w Lublinie
Lublin, Lublin Voivodeship, 20-400, Poland
Klinika Alergologii Gdanskiego Uniwersytetu Medycznego
Gdansk, Pomeranian Voivodeship, 80-211, Poland
Katedra i Klinika Pneumonologii Slaskiego Uniwersytetu Medycznego w Katowicach
Katowice, Silesian Voivodeship, Poland
Samodzielny Publiczny Szpital Kliniczny nr 1 im. prof. St. Szyszko Sląskiego Uniwersytetu Medycznego w Katowicach
Zabrze, Silesian Voivodeship, 41-800, Poland
Related Publications (1)
Rzepka-Wrona P, Skoczynski S, Barczyk A. Are There Differences in Inflammatory and Fibrotic Pathways between IPAF, CTD-ILDs, and IIPs? A Single-Center Pilot Study. Int J Mol Sci. 2022 Dec 2;23(23):15205. doi: 10.3390/ijms232315205.
PMID: 36499525DERIVED
Biospecimen
The following biospecimens will be collected: * blood (whole venous blood and serum) * bronchoalveolar lavage fluid * samples of bronchial mucosa
MeSH Terms
Conditions
Interventions
Condition Hierarchy (Ancestors)
Intervention Hierarchy (Ancestors)
Study Officials
- STUDY CHAIR
Szymon Skoczynski, MD, PhD
Slaski Uniwersytet Medyczny
Central Study Contacts
Study Design
- Study Type
- observational
- Observational Model
- COHORT
- Time Perspective
- PROSPECTIVE
- Sponsor Type
- OTHER
- Responsible Party
- PRINCIPAL INVESTIGATOR
- PI Title
- Professor, Ph.D., M.D.
Study Record Dates
First Submitted
March 10, 2019
First Posted
March 12, 2019
Study Start
March 18, 2019
Primary Completion
February 18, 2020
Study Completion
December 31, 2025
Last Updated
March 12, 2019
Record last verified: 2019-03
Data Sharing
- IPD Sharing
- Will not share