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Background: The early detection and management of (progressive) interstitial lung disease in patients with connective tissue diseases requires the attention and skills of a multidisciplinary team. However, there are currently no well-established standards to guide the daily practice of physicians treating this heterogenous group of diseases., Research Question: This paper aimed to identify gaps in scientific knowledge along the journey of patients with connective tissue disease-related interstitial lung disease and to provide tools for earlier identification of interstitial lung disease and progressive disease., Study Design and Methods: The opinions of an international expert panel, which consisted of pulmonologists and rheumatologists were collected and interpreted in the light of peer-reviewed data., Results: Interstitial lung disease is a common complication of connective tissue diseases, but prevalence estimates vary by subtype. Screening and monitoring by means of clinical examination, chest radiography, pulmonary function testing, and disease-specific biomarkers provide insight into the disease activity of patients presenting with connective tissue diseases in a routine setting. Multiple phenotypic and genotypic characteristics have been identified as predictors of the development and progression of interstitial lung disease. However, these risk factors differ between subtypes. To ensure earlier diagnosis of rapidly progressive phenotypes, a risk-based method is necessary for determining the need for HRCT and additional testing., Interpretation: To reduce the underdiagnosis of CTD-ILDs in clinical practice, a standardized and systematic multidisciplinary risk-based approach is suggested. Collaboration across disciplines is essential for the management of CTD-ILD., Competing Interests: Declaration of competing interest JG reports personal fees for advisory board, work and lectures from Boehringer Ingelheim, Janssen, SMB, GSK, Roche, AstraZeneca, Aquilon, Volition, Oncoradiomics, and Chiesi, non-financial support for meeting attendance from AstraZeneca, Chiesi, MSD, Roche, Boehringer Ingelheim and Janssen. He is in the permanent SAB of Radiomics (Oncoradiomics SA) for the SALMON trial without any specific consultancy fee for this work. He is co-inventor of one issued patent on radiomics licensed to Radiomics (Oncoradiomics SA). He confirms that none of the above entities or funding was involved in the preparation of this work.JM has received grant/research support to the institution from the Erasmus Research innovation grants and sarcoidosis patients support group sarcoidosis.nl, and reports consulting fees and lecture honoraria from Boehringer Ingelheim and personal fees from Hoffmann la Roche and Novartis.AC  has received speaker fees from Boehringer IngelheimJdVB has received grant/research support to her institution from ReumaNederland (Dutch patient Society for Rheumatology), Nationale Vereniging voor mensen met lupus, APS, sclerodermie en MCTD (Dutch patient society), ARCH (Autoimmune Research and Collaboration Hub; Dutch interdisciplinary society for patients and caregivers), Janssen-Cilag, and Boehringer Ingelheim; consulting fees from Boehringer Ingelheim, Jansen-Cilag and Abbvie (payments made to institution); speaker fees from Dutch Society (payments made to institution), Boehringer Ingelheim (payments made to institution), Janssen-Cilag (payments made to institution).TD reports consultancy fees, speaker fees, honoraria, advisory boards from Abbvie, Amgen, Boehringer Ingelheim, GSK, Genesis Pharma, Janssen, Lilly, SOBI, UCB and Pfizer, non-financial support for meeting attendance from Abbvie, Genesis Pharma, Janssen, Elpen, Enorasis, UCB and Pfizer.KS has received fees for speaking and/or consulting fees from Boehringer Ingelheim; Travel expenses from UCB, Boehringer Ingelheim; research grants to Aarhus University Hospital from Danish Rheumatism Association; and has been primary investigator in trials by GSK and Boehringer IngelheimAT has received grant/research support to his institution for Scientific Research in Interstitial Lung Diseases from Boehringer Ingelheim and Roche; consulting fees from Boehringer Ingelheim, Roche, Chiesi, GlaxoSmithKline, Astra Zeneca and Elpen (payments made to self and institution); speaker fees from Boehringer Ingelheim, Roche, Chiesi, GlaxoSmithKline, Astra Zeneca and Elpen; support for attending meetings and/or travel from Boehringer Ingelheim, Chiesi, Roche, Menarini, Elpen and AstraZeneca.VS has received grant/research support to her institution from the Research Foundation Flanders, Belgian Fund for Scientific Research in Rheumatic Diseases, Janssen-Cilag, Boehringer Ingelheim and Vlaeynatie; consulting fees from Boehringer Ingelheim (payments made to self and institution) and Janssen-Cilag (payments made to institution); speaker fees from UCB (payments made to institution), Boehringer Ingelheim (payments made to self and institution), Janssen-Cilag (payments made to institution); support for attending meetings and/or travel from Boehringer Ingelheim (payments made to institution)., (Copyright © 2024 The Authors. Published by Elsevier B.V. All rights reserved.)

Introduction: Systemic sclerosis (SSc) is a rare chronic autoimmune disease characterised by microvascular damage, immune dysregulation and fibrosis, affecting the skin, joints and internal organs. Interstitial lung disease (ILD) is frequently associated with systemic sclerosis (SSc-ILD), leading to a poor prognosis and a high mortality rate. The aim of the BUILDup study (BUrden of Interstitial Lung Disease Consensus Panel) was to investigate the overall disease management and to estimate the social and economic burden of SSc-ILD across 8 European countries., Methods: A modified Delphi method was used to obtain information on the management of SSc-ILD patients among 40 specialists (panellists) from 8 European countries. Average annual costs per patient and country were estimated by means of a direct cost-analysis study., Results: The panellists had managed 805 SSc-ILD patients in the last year, 39.1% with limited (L-SSc-ILD) and 60.9% with extensive (E-SSc-ILD) disease. Of these, 32.8% of the panellists started treatment at diagnosis, 42.3% after signs of deterioration/progression and 24.7% when the disease had become extensive. The average annual cost of SSc-ILD per patient ranged from €6191 in Greece to €25,354 in Sweden. Main cost drivers were follow-up procedures, accounting for 80% of the total annual costs. Hospitalisations were the most important cost driver of follow-up costs. Healthcare resource use was more important for E-SSc-ILD compared to L-SSc-ILD. Early retirement was taken by 40.4% of the patients with an average of 11.9 years before the statutory retirement age., Conclusions: SSc-ILD entails not only a clinical but also a social and economic burden, and is higher for E-SSc-ILD.

Introduction: The term progressive fibrosing interstitial lung disease (ILD) describes patients with fibrotic ILDs who, irrespective of the aetiology of the disease, show a progressive course of their disease despite current available (and non-licensed) treatment. Besides in idiopathic pulmonary fibrosis, little is known about management and the burden of patients with fibrotic ILD, particularly those with a progressive behaviour., Methods: Using the Delphi method, 40 European experts in ILD management delivered information on management of (progressive) fibrosing ILD and on the impact of the disease on patients' quality of life (QoL) and healthcare resource utilisation (HCRU). Annual costs were calculated for progressive and non-/slow-progressive fibrosing ILD for diagnosis, follow-up management, exacerbation management, and end-of-life care based on the survey data., Results: Physicians reported that progression in fibrosing ILD worsens QoL in both patients and their caregivers. Progression of fibrosing ILD was associated with a greater use of HCRU for follow-up visits and maintenance treatment compared with the non-/slow progression. The number of patients who suffered at least one acute exacerbation was reported to be more than three times higher in progressive fibrosing ILD patients than in patients with non-/slow-progressive fibrosing ILD. On average, annual estimated costs of progressive fibrosing ILD per patient were 1.8 times higher than those of the non-/slow-progressive form of the disease., Conclusions: Progression in fibrosing ILD causes a significant impact on QoL and HCRU and costs. These survey data underline the need for safe and effective therapies to slow the disease progression.

Thin-slices multi-detector computed tomography (MDCT) plays a key role in the differential diagnosis of interstitial lung disease (ILD). However, thin-slices MDCT has a limited ability to detect active inflammation, which is an important target of newly developed ILD drug therapy. Magnetic resonance imaging (MRI), thanks to its multi-parameter capability, provides better tissue characterisation than thin-slices MDCT.Our aim was to summarise the current status of MRI applications in ILD and to propose an ILD-MRI protocol. A systematic literature search was conducted for relevant studies on chest MRI in patients with ILD.We retrieved 1246 papers of which 55 original papers were selected for the review. We identified 24 studies comparing image quality of thin-slices MDCT and MRI using several MRI sequences. These studies described new MRI sequences to assess ILD parenchymal abnormalities, such as honeycombing, reticulation and ground-glass opacity. Thin-slices MDCT remains superior to MRI for morphological imaging. However, recent studies with ultra-short echo-time MRI showed image quality comparable to thin-slices MDCT. Several studies demonstrated the added value of chest MRI by using functional imaging, especially to detect and quantify inflammatory changes.We concluded that chest MRI could play a role in ILD patients to differentiate inflammatory and fibrotic changes and to assess efficacy of new ILD drugs., Competing Interests: Conflict of interest: C. Romei has nothing to disclose. Conflict of interest: L. Turturici has nothing to disclose. Conflict of interest: L. Tavanti has nothing to disclose. Conflict of interest: J. Miedema has nothing to disclose. Conflict of interest: S. Fiorini has nothing to disclose. Conflict of interest: M. Marletta has nothing to disclose. Conflict of interest: P. Wielopolski has nothing to disclose. Conflict of interest: H. Tiddens reports industry funding from Roche, lecture and advisory board fees from Novartis, and grants from CFF, Vertex, Gilead and Chiesi, outside the submitted work. In addition, H. Tiddens has a patent licensed with Vectura, and a patent PRAGMA-CF scoring system issued. Conflict of interest: F. Falaschi has nothing to disclose. Conflict of interest: P. Ciet reports personal fees from Vertex Pharmaceutical, outside the submitted work., (Copyright ©ERS 2018.)