Symposium 23：Interstitial Lung Disease
The etiology of ILD is quite diverse, which adds a lot of difficulty to the diagnosis. At the same time, although there are breakthroughs in the current treatment, there is still no way to reverse the disease process for the patients. Therefore, understanding the disease, early diagnosis and early treatment can truly benefit the patients. This symposium, we will introduce the latest developments in the current treatment strategy of ILD. We also introduce the consensus of hypersensitive pneumonitis and progressive pulmonary fibrosis which were written by TPSCCM
| Time (GMT+8) |
Topic | Speaker | Country / Region |
|---|---|---|---|
| 14:50-15:20 | The new treatment strategy of IPF | Dr. Toru Arai | Japan |
| 15:20-15:50 | The hypersensitivity pneumonitis consensus in Japan | Dr. Yoshikazu Inoue | Japan |
| 15:50-16:20 | The progressive pulmonary fibrosis consensus of Taiwan | Dr. Tang-Hsiu Huang | Taiwan |
The new treatment strategy of IPF
Abstract:
Idiopathic pulmonary fibrosis (IPF) is a poor prognostic interstitial lung disease with unknown etiology, showing progressive fibrosis and pathologically and radiologically usual interstitial pneumonia (UIP) pattern. Before the anti-fibrotic drugs were introduced for the treatment of IPF, steroid therapy was recommended according to the statement for IPF (AJRCCM 2000). However, steroid plus immunosuppressants was denied as a treatment for IPF based on PANTHER trial (Raghu, NEJM 2012). Effects of pirfenidone and nintedanib were proved by ASCEND trial (King, NEJM2014) and INPULSIS trial (NEJM 2014), respectively. And both anti-fibrotic drugs were recommended (conditional) in the ATS/ERS/JRS/ALAT guideline for IPF (Raghu, AJRCCM 2015).
Introduction of cryobiopsy is most important update for the diagnosis of ILDs including IPF. Simultaneously, each ILD diagnosis with confidence levels are determined according to the multi-disciplinary discussion (MDD). If only transbronchial lung biopsy (TBLB) is performed in addition to bronchoalveolar lavage (BAL), we have to diagnosis most patients as unclassifiable idiopathic interstitial pneumonia (IIP) or ILD. However, using cryobiopsy specimens we can diagnosis specific ILD adding confidence levels. As for IPF, same phenomenon happens and patients with IPF are supposed to be diagnosed earlier than before although confidence level might be low.
Treatment strategy for definite IPF patients might not be changed in recent years; however, such IPF patients with low confidence levels need to be managed with cautious observation with symptomatic, radiologic, physiologic and biomarker findings. Appearance of cryobiopsy enable us to diagnosis some patients more easily than before; however, our responsibility to manage appropriately increased than before at the same time.
I would like to summarize the present problems of IPF diagnosis and long term management of IPF in the era of cryobiopsy.
Dr. Toru Arai
Japan
Graduated from School of Medicine, Osaka University, Japan
Received PhD from Graduate School of Medicine, Faculty of Medicine, Osaka University.
2000-present: Staff physician, Department of Respiratory Medicine, National Hospital Organization Kinki-Chuo Chest Medical Center
2014-2022: Director, Department of Respiratory Failure, Clinical Research Center, National Hospital Organization Kinki-Chuo Chest Medical Center
2023-present: Executive Director, Clinical Research Center, National Hospital Organization Kinki-Chuo Chest Medical Center
Research focus
1 Interstitial lung diseases (ILDs)
2 Acute exacerbation of ILDs
3 Rare lung diseases
3.1 Pulmonary alveolar proteinosis
3.2 Lymphangioleiomyomatosis”
The hypersensitivity pneumonitis consensus in Japan
Abstract:
The Hypersensitivity Pneumonitis Consensus in Japan
Yoshikazu Inoue, M.D., Ph.D.
Osaka Anti-tuberculosis Association Osaka Fukujuji Hospital, Osaka, Japan
Clinical Research Center, NHO Kinki-Chuo Chest Medical Center, Osaka, Japan
Hypersensitive pneumonitis (HP) is typically an immune-mediated diseasethatmanifests as ILD in susceptibleindividuals after exposure to an identified orunidentified factor. In 2020, ATS/JRS/ALAT officially first published a Clinical Practice Guideline (Raghu G, et al. Am J Respir Crit Care Med, 2020), and in 2021, ACCP published the CHEST guideline (Pérez ER F, et al. Chest. 2021). Recently, JRS officially published the guide in 2022 (Japanese) and in 2024 (English version. Tomioka H et al. Resp Invest 2024) as a JRS own practice guide.Major typesof HP in Japan include summer-type, home-related, bird-related, farmer’s lung, humidifier lung,etc.. Identifying causative antigens is critical for increasing diagnostic confidence, aswell as improving prognosis through appropriate antigen avoidance. This guide proposes a comprehensive questionnaire for Japanese patients. Thedetection of specific antibodies against causative antigens is an important diagnostic predictorof HP. In Japan,the assessments of bird-specific IgG and the Trichosporonasahiiantibody are commercially available and are recently covered bymedical insurance. The JRS guide basically adopts theATS/JRS/ALAT guideline, and the diagnostic criteria was based onthe combination of imaging findings, exposure assessment, bronchoalveolar lavage fluid, and histopathologicalfindings, but the JRS added some annotations to facilitate the interpretation of the Japan specific situation. The JRS guide recommends checking biomarkers such as seasonal changes in the KL-6 concentration(increase in winter for bird-related HP/humidifier lung and in summer for summer-type HP) and high (and variable) KL-6concentrations (suspicion of HP). Antigen avoidance is crucial for the managementsof HP. JRS guide also addresses the pharmacological management of HP, highlighting the treatment strategy forfibrotic HP including anti-inflammatory, anti-immunosuppressive and antifibrotic drugs.
Dr. Yoshikazu Inoue
Japan
Dr. Yoshikazu Inoue, MD, PhD, is a well-known expert of interstitial lung diseases (ILD) and rare lung diseases. He has served as an executive director of clinical research center, National Hospital Organization (NHO) Kinki-Chuo Chest Medical Center (Osaka, Japan) and an Invited Professor, Graduate School of Medicine, Osaka University (Osaka, Japan). From April, 2023, he is now working both in NHO Kinki-Chuo Chest Medical Center, and in Osaka Anti-tuberculosis Association Osaka Fukujuji Hospital (Osaka, Japan). He studied ILD at Ehime University School of Medicine (Ehime, Japan), and at University of Colorado, Health Science Center (Colorado, USA). Recently, he has spent his professional career focusing on translational and clinical aspects of ILDs and rare intractable lung diseases (PAP, LAM). He has conducted and involved in many international clinical trials and studies. He served as presidents, delegates, and board members of domestic and international scientific societies (ATS, ERS, APSR, Fleischner Society, WASOG, JRS, JSSOG, etc.). He has published more than 400 original articles, review articles, and case reports. Noteworthy, he has served for patient advocacy groups as advisors (LAM Foundation, PAP Foundation, Japanese PAP association, Japanese LAM association, Japanese interstitial pneumonia/pulmonary fibrosis patients society).
The progressive pulmonary fibrosis consensus of Taiwan
Abstract:
Idiopathic pulmonary fibrosis (IPF) is the archetype of fibrosing interstitial lung diseases. However, many non-IPF interstitial pneumonias, whether idiopathic or secondary, can also cause increasingly fibrotic destruction of the pulmonary parenchyma despite treatments, and this is a process that is now referred to as progressive pulmonary fibrosis (PPF). In 2021, under the initiation and coordination of Taiwan Society of Pulmonary and Critical care Medicine, multi-disciplinary collaboration among specialists from pulmonology, rheumatology, and medical radiology led to the publication of Taiwan Consensus of Progressive Pulmonary Fibrosis, which has just been recently updated. Based on the local and international evidence and experience, the Consensus presents concise summaries and suggestions regarding the epidemiology, diagnosis and differential diagnosis, pharmacological and non-pharmacological treatments, and follow-up monitoring of PPF. Moreover, the National Health Insurance of Taiwan has recently extended the coverage of the antifibrotic agent nintedanib to patients with PPF. Timely identification of PPF, and the prompt initiation of antifibrotic treatment in addition to disease-targeted therapies, are critical to slow down further fibrotic destruction and functional deterioration. The medical system and medical community of Taiwan are actively assisting clinicians in this regard.
Dr. Tang-Hsiu Huang
Taiwan
Dr. Tang-Hsiu Huang is an attending physician and clinical assistant professor at National Cheng Kung University in Taiwan. His clinical and research interest focuses on interstitial and rare lung diseases and pulmonary fibrosis.
Dr. Hao-Chien Wang
Taiwan
Dr. Hao-Chien Wang is professor of Internal medicine in College of Medicine National Taiwan University and consultant physician in Chest specialty practicing at National Taiwan University Hospital in Taipei city.
Dr. Wang has an outstanding career marked by contributions to academic research, education, training and clinical practice. Dr. Wang’s research focuses mainly on basic and clinical researches on obstructive airway diseases, acute lung injury and chest ultrasonography in diagnosing of pulmonary diseases. He has published more than 120 papers in national and international peer reviewed journals. Furthermore, Dr. Wang is an investigator for several ongoing global clinical studies in the area of respiratory disease including pneumonia, COPD & asthma.
