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

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)

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).

The following biospecimens will be collected: - blood (whole venous blood and serum) - bronchoalveolar lavage fluid - samples of bronchial mucosa

Interstitial Lung Disease

Interstitial Pneumonia

Interstitial Fibrosis

Autoimmune Diseases

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.

Bronchoalveolar lavage and taking bronchial mucosa samples

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

6minute walk test

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.

Arterial blood gas

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

Blood drawing

Universita' degli Studi di Messina

Katedra i Klinika Pneumonologii, Onkologii i Alergologii Uniwersytetu Medycznego w Lublinie

II Katedra Chorob Wewnetrznych Uniwersytetu Jagiellonskiego Collegium Medicum im. prof. A. Szczeklika Szpitala Uniwersyteckiego

Klinika Alergologii Gdanskiego Uniwersytetu Medycznego

Katedra i Klinika Pneumonologii Slaskiego Uniwersytetu Medycznego w Katowicach

Samodzielny Publiczny Szpital Kliniczny nr 1 im. prof. St. Szyszko Sląskiego Uniwersytetu Medycznego w Katowicach

Katedra i Klinika Pulmonologii, Alergologii i Onkologii Pulmonologicznej Uniwersytetu Medycznego im. K. Marcinkowskiego w Poznaniu

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

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).

Adam Barczyk

Professor, Ph.D., M.D.

Medical University of Silesia

Diagnosis criteria for AE-IPF:
Diagnosed IPF patient experiences unexplained dyspnea within 1 month
With objective evidence of hypoxia and new onset of pulmonary infiltration based on imaging examination
With other diagnosis like pulmonary embolism, pneumothorax or heart failure excluded.

Diagnosis criteria for Stable-IPF:
Exclusion of other known causes of ILDs
Presence of a usual interstitial pneumonitis (UIP) pattern on high-resolution computed tomography (HRCT)
Specific combinations of HRCT and surgical lung biopsy pattern in patients subjected to surgical lung biopsy.

Sputum, throat swab, BALF and blood will be collected in this study.

Interstitial Lung Diseases

No intervention

Shanghai Pulmonary Hospital, Tongji University

Study of the Etiology and Immunological Pathogenesis in Acute Exacerbation of Idiopathic Pulmonary Fibrosis (AE-IPF)

Testing of pathogen nucleic acid chips:Based on PathGEN® Pathogen Chip Kit (PathGEN Dx, Singapore). Next Generation Sequencing (NGS) has become the powerful tool in pathogen identification based on its rapidness and sensitivity. The final report will provide the species classification of the bacteria and viruses, as well as corresponding designator.

Subtype and functional testing on phenotype and function of Macrophage cell in IPF/AE-IPF patients.

Subtype and functional testing on phenotype and function of DC in IPF/AE-IPF patients.

Subtype and functional testing on phenotype and function of T cell in IPF/AE-IPF patients.

Subtype and functional testing on phenotype and function of B cell in IPF/AE-IPF patients.

The investigators will evaluate the expression of IL-17 by proteomics in IPF/AE-IPF patients.

The investigators will evaluate the expression of MIG by proteomics in IPF/AE-IPF patients.

cryptogenic organizing pneumonia/toux

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

Study of the Etiology and Immunological Pathogenesis in Acute Exacerbation of Idiopathic Pulmonary Fibrosis (AE-IPF)

La base de données d'herbes médicinales la plus complète soutenue par la science