Clinical Characteristics of Interstitial Pneumonia With Autoimmune Features (IPAF) - a Multicenter Prospective Study

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.

Conditions

• Interstitial Lung Disease
• Interstitial Pneumonia
• Interstitial Fibrosis
• Autoimmune Diseases

Interventions

DIAGNOSTIC_TEST

Bronchoalveolar lavage and taking bronchial mucosa samples

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.

DIAGNOSTIC_TEST

6minute walk test

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

DIAGNOSTIC_TEST

Arterial blood gas

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.

PROCEDURE

Blood drawing

A vein will be punctured in order to take a blood sample for further tests

Sponsors & Collaborators

• Medical University of Silesia

  lead OTHER

Principal Investigators

• Szymon Skoczynski, MD, PhD · Slaski Uniwersytet Medyczny

Eligibility

Min Age

18 Years

Sex

ALL

Healthy Volunteers

No

Timeline & Regulatory

Start

2019-03-18

Primary Completion

2020-02-18

Completion

2025-12-31

Countries

• Italy
• Poland

Study Locations