Study of the Etiology and Immunological Pathogenesis in Acute Exacerbation of Idiopathic Pulmonary Fibrosis (AE-IPF)
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|ClinicalTrials.gov Identifier: NCT02509364|
Recruitment Status : Unknown
Verified July 2015 by Huiping Li, Shanghai Pulmonary Hospital, Shanghai, China.
Recruitment status was: Enrolling by invitation
First Posted : July 28, 2015
Last Update Posted : July 28, 2015
Idiopathic pulmonary fibrosis (IPF) is a highly heterogeneous and lethal pathological process with limited therapeutic options, which is the most common and severe of the idiopathic interstitial pneumonias (IIPs). During the past 20 years, the incidence of IPF increased significantly. Most of IPF patients show a median survival time of 2-3 years after diagnosis. Five-year survival rate is 30%-50%. It's difficult to diagnose in the early stage of IPF. Once the patients go to hospital, it's already in the late stage. Now there is no effective therapy except lung transplant for IPF in clinical application.
Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is a fatal condition with high mortality (over 80%). Its etiology and pathogenesis remain unknown. There is a lack of effective treatment for it. Based on the investigators' long-term clinical observation, most cases of AE-IPF initially got "common cold" and had coryza, more cough, nasal obstruction，rhinorrhea, sore throat, some patients had fever, headache, and etc. Some of these patients' condition developed very rapidly and then became very severe similar to the situation of acute respiratory distress syndrome (ARDS). Why these AE-IPF patients were so hypersensitive to "cold"? What were the immunologic and pathological mechanisms of their lung lesions after patients exposed to "common cold"? How to effectively offer interventional treatment for AE-IPF? All the above questions are yet to be explained clearly.
In the investigators' previous retrospective study, the investigators found that there were some obviously imbalanced immune responses in IPF patients, including increased cluster of differentiation 4 (CD4) T cell population, immunoglobulin and complements. The investigators also found highly expressed inflammatory cytokines (IL-17, MIG and IL-9) and high detection rates of pathogens in AE-IPF patients, especially serum immunoglobulin M (IgM) antibody of some respiratory virus. These findings provide a strong suggestion that there are some imbalance of immunologic function in IPF and there are relationship between AE-IPF and infection, especially "Cold" virus infection might be a key trigger for AE-IPF.
|Condition or disease||Intervention/treatment|
|Interstitial Lung Diseases||Other: No intervention|
|Study Type :||Observational|
|Estimated Enrollment :||300 participants|
|Observational Model:||Case Control|
|Study Start Date :||August 2015|
|Estimated Primary Completion Date :||December 2016|
|Estimated Study Completion Date :||September 2017|
Diagnosis criteria for AE-IPF:
Other: No intervention
Diagnosis criteria for Stable-IPF:
Other: No intervention
Other: No intervention
- Virus RNA by pathogen test chip and second generation sequencing in AE-IPF [ Time Frame: up to 30 weeks ]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.
- Macrophage phenotype and function [ Time Frame: up to 30 weeks ]Subtype and functional testing on phenotype and function of Macrophage cell in IPF/AE-IPF patients.
- Dendritic cell (DC) phenotype and function [ Time Frame: up to 30 weeks ]Subtype and functional testing on phenotype and function of DC in IPF/AE-IPF patients.
- T cell phenotype and function [ Time Frame: up to 30 weeks ]Subtype and functional testing on phenotype and function of T cell in IPF/AE-IPF patients.
- B cell phenotype and function [ Time Frame: up to 30 weeks ]Subtype and functional testing on phenotype and function of B cell in IPF/AE-IPF patients.
- Expression of IL-17 in AE-IPF [ Time Frame: up to 30 weeks ]The investigators will evaluate the expression of IL-17 by proteomics in IPF/AE-IPF patients.
- Expression of MIG in AE-IPF [ Time Frame: up to 30 weeks ]The investigators will evaluate the expression of MIG by proteomics in IPF/AE-IPF patients.
Biospecimen Retention: Samples With DNA
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Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT02509364
|Shanghai Pulmonary Hospital, Tongji University|
|Shanghai, Shanghai, China, 200433|
|Principal Investigator:||Huiping Li, MD,PhD||Shanghai Pulmonary Hospital, Tongji University, Shanghai, China|