Interstitial Lung Disease Due to Systemic Disease

The aim of this study is to examine the validity and reliability of the Turkish Validity and Reliability of The King's Brief Interstitial Lung Disease Questionnaire (K-BILD) in Individuals with Interstitial Lung Involvement Secondary to Rheumatological Diseases.

The goal of this clinical trial is to test efficacy of different investigational products (IPs) compared with placebo on the change from baseline to the end of the treatment period at Week 52 in lung capacity in participants with Interstitial Lung Disease Secondary to Systemic Sclerosis.

The main aim of the project is to identify key-factors involved in the development and progression of Systemic Sclerosis (SSc), a chronic invalidating rheumatic disease characterized by high mortality and insufficient treatment options. A cohort of patients with SSc will be collected at the Sahlgrenska University Hospital in Gothenburg, Skaraborg Hospital in Skövde, and Södra Älvsborg Hospital in Borås (Sweden). Thanks to a holistic approach including integrated analysis of blood, and skin samples as well as DNA, and with the use of state-of-the-art methods, this project aims to identify factors (e.g. genes, proteins, metabolites, and immune cell types) associated with the development of SSc and with the progression to a more aggressive phenotype. Functional studies using in vitro model systems and patient specimens will be also implemented. The findings of the current project could lead to the identification of possible diagnostic and prognostic markers for the disease as well as potential drug targets. This cohort will be also linked to the European Scleroderma Trial and Research (EUSTAR), which is an international SSc research network aiming to coordinate research activities on SSc from groups all over Europe in order to improve treatment, quality of life and mortality of patients with SSc.

Connective tissue disease-associated interstitial lung disease (CTD-ILD) is a rare and complex condition that affects some individuals with autoimmune disorders such as rheumatoid arthritis (RA). It can lead to inflammation and progressive scarring of the lungs, significantly affecting quality of life and overall prognosis.1 Current clinical guidelines recommend that patients with CTD-ILD receive care at specialized academic medical centers with multi-disciplinary teams experienced in managing interstitial lung disease (ILD).2 These centers, however, are often located in urban areas, making access particularly challenging for patients living in rural and underserved regions. In rural, medically underserved areas, the nearest ILD centers can be three or more hours away, posing substantial barriers to timely, consistent, and expert care. These logistical constraints make it difficult for patients residing in rural areas to access the high-quality, specialized care required to manage CTD-ILD effectively. The Teleconsultation Approach to CTD-ILD Care Delivery in Rural Underserved Communities (TRUST Initiative) it looking to address this gap in access to specialized care. The TRUST Initiative aims to evaluate whether integrating teleconsultation into a personalized, multidisciplinary care model, through regular, structured teleconsultation visits, can enhance the quality and coordination of treatment for patients and potentially other rural underserved patient populations. This model utilizes a regional rheumatologist, a regional pulmonologist and, an interstitial lung disease (ILD) specialist at the Mayo Clinic in Rochester, Minnesota. This model seeks to overcome the geographic and logistical challenges that prevent patients from accessing specialty care. It also offers a replicable care model for managing other complex chronic diseases in underserved rural communities by leveraging collaborative care, virtual health technology, and structured communication workflows. The study will not only track clinical outcomes such as pulmonary function but also evaluate patient satisfaction, highlighting both the effectiveness and acceptability of this care model. By addressing disparities in access to specialty care, the TRUST Initiative aims to inform future healthcare delivery strategies for rare and complex diseases in rural and underserved settings nationwide.

Interstitial lung diseases (ILD) constitute a group of entities characterized by inflammation and/or fibrosis of the lung parenchyma. In recent years, with the advent of new diagnostic tools and therapeutic options, multidisciplinary evaluation is essential, since it optimizes the interpretation of each case and the quality of care for these pathologies Consensus for the identification and management of ILD associated with SSc (ILD-SSc) is the only guideline published at present. In the others autoimmune ILD (Ai-ILD), screening, diagnosis, treatment and follow-up strategies are usually performed according to the criteria of the treating medical team. Guidelines regarding the follow-up and indication of immunosuppressive and antifibrotic treatment are lacking. Many questions on the horizon of the Ai-ILD should be answered as better quality evidence emerges from studies with a greater number of patients and better methodological design.