The new challenge of CTD-ILD: from basic science to clinical setting

ILDs associated with connective tissue diseases (CTD-ILDs) share common biomarkers, suggesting common pathophysiological pathways, although there are also distinct clinical and radiological characteristics for each entity. Respiratory involvement in CTD and its early detection is crucial for optimal clinical management of these patients, since lung fibrosis is estimated to be the first cause of disease-related death in systemic sclerosis (SSc) and rheumatoid arthritis (RA). Conversely, all patients with ILD need a thorough evaluation for the presence of CTD symptoms and signs, not only at presentation but also on follow up. CTD symptoms and signs can be subtle and easily missed if not specifically looked for. Furthermore, CTD can develop even several years after the initial diagnosis of ILD. Rheumatological evaluation forms a key part of the evaluation of selected patients, with joint pulmonology-rheumatology clinics and MDTs being increasingly used to meet the complex diagnostic and management needs of CTD-ILD patients. An increasing array of immunomodulators, including biologics are becoming available for the treatment of CTDs and recently also of ILDs, such that a multidisciplinary approach is essential in selected cases.

Moreover, disease behaviour is unpredictable, resulting in poor prognostic estimation and uncertainties regarding timing and choice of treatment. Several serum biomarkers have been investigated as helpful for diagnosis and importantly prognosis of ILD, although none are sufficiently validated to be used in routine clinical practice. Furthermore, no biomarker has been shown to provide reliable early signal of response to treatment. Disease specific biomarkers are lacking, and large longitudinal prospective studies are needed to contribute to ameliorate the management of these patients.

Educational aims

The overall incidence of interstitial lung disease (ILD) in patients with CTD varies widely according to the CTD, In SSc, clinically significant ILD is estimated at 30-50%, while in RA it is believed to affect approximately 5-10% of patients . CTDs at risk of developing a progressive fibrotic lung disease phenotype, with ongoing ILD progression despite immunomodulation include rheumatoid arthritis (RA), systemic sclerosis (SSc), mixed CTD, and less commonly, Myositis Spectrum Disorders, and Sjögren’s syndrome (though rarely).

The webinar will focus on the current issues and pitfalls concerning the diagnostic pathway and the clinical management of CTD-ILDs, including acute exacerbations of disease. Particular attention will be paid on two key aspects: the evaluation of endoscopic (including bronchoalveolar lavage and transbronchial cryobiopsy) and/or surgical procedures in the diagnostic framework of CTD-ILD and the definition of interstitial pneumonia with autoimmune features (IPAF) and its impact in the clinical practice. The respective contributions of the rheumatologist and of the pulmonologist, and how they can interact to improve the management of patients with CTD-ILD will be provided by a Pulmonologist and a Rheumatologist with extensive experience in CTD-ILD.

A specific presentation will be focused on acute exacerbations of CTD-ILD, highlighting the differences and similarities with acute exacerbations of IPF, both from a diagnostic and therapeutic point of view.

Topics

• Cell and molecular biology
• Imaging
• Pulmonary function testing

Format

• Session (45 min)
  • Lectures by the speakers (10 min each) with interactive questions for the audience
    • Acute exacerbation of rheumatic diseases (Prof. Fabrizio Luppi)
    • Bronchoalveolar lavage and lung biopsy in connective tissue diseases – to do or not to do? (Prof. Marco Matui-Cerinic vs Prof. Sara Tomassetti)
  • Case presentations from participants (5 min)
• General discussion (15 min)

Learning outcomes

Following the webinar, participants will be able to:

• Improve knowledge of CTD-ILD and optimize diagnostic pathways through a multidisciplinary approach;
• Keep up to date to the newest therapeutic opportunities on CTD-ILDs setting;
• Implement diagnostic and prognostic biomarkers the clinical practice.

CME credit

An application for accreditation of this webinar has been made to the European Board for Accreditation in Pneumology (EBAP) for 1 CME credit per 1-hour attendance. If accredited, the CME credit will be granted upon attendance of at least 60 minutes during the live webinar only.

What is a webinar?

A webinar closely simulates a lecture-based teaching experience. The speaker can interact with the audience, just as in a classroom setting. During the webinar, you will be asked to share your opinion on issues related to the topic using interactive polls.

All participants will be able to hear the lecturer and see the slides throughout the presentation. As a participant you will be able to pose questions or discuss ideas with the other participants via the text chat facility and the speaker will respond to the questions via the microphone.

Login guidelines

More information will be communicated in due course.

• Please log in to the webinar 20 minutes before it is scheduled to commence. If you have any technical difficulties whilst trying to log in or during the session please contact e-learning@ersnet.org.
• Check Central European Time.
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