Connective tissue disease; Diagnosis; European reference networks; Expert opinion; Interstitial lung disease; Monitoring; Multidisciplinary team; Practice guidelines; Pulmonary fibrosis; Rheumatoid arthritis; Screening; Systemic sclerosis; Biomarkers; Humans; Disease Progression; Practice Guidelines as Topic; Lung Diseases, Interstitial/diagnosis; Lung Diseases, Interstitial/etiology; Lung Diseases, Interstitial/complications; Connective Tissue Diseases/diagnosis; Connective Tissue Diseases/complications; Early Diagnosis; Immunology and Allergy; Immunology
Abstract :
[en] [en] 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.
Disciplines :
Cardiovascular & respiratory systems
Author, co-author :
Guiot, Julien ; Université de Liège - ULiège > Département des sciences biomédicales et précliniques
Miedema, Jelle; Center of Excellence for Interstitial Lung Diseases and Sarcoidosis, Department of Pulmonology, Erasmus University Medical Center, Rotterdam, the Netherlands. Electronic address: j.miedema@erasmusmc.nl
De Vries-Bouwstra, Jeska K; Department of Rheumatology, Leiden University Medical Center, Leiden, the Netherlands,. Electronic address: J.K.de_Vries-Bouwstra@lumc.nl
Dimitroulas, Theodoros; 4(th) Department of Internal Medicine, Hippokration Hospital, School of Medicine, Aristotle University of Thessaloniki, Thessaloniki, Greece
Søndergaard, Klaus; Department of Rheumatology, Aarhus University Hospital, Aarhus, Denmark, Department of Clinical Medicine, Aarhus University, Aarhus, Denmark. Electronic address: klausoen@rm.dk
Tzouvelekis, Argyrios; Department of Respiratory Medicine, University of Patras, Patras, Greece
Smith, Vanessa; Department of Internal Medicine, Ghent University, Ghent, Belgium, Department of Rheumatology, Ghent University Hospital, Ghent, Belgium, Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Ghent, Belgium. Electronic address: vanessa.smith@ugent.be
Language :
English
Title :
Practical guidance for the early recognition and follow-up of patients with connective tissue disease-related interstitial lung disease.
This manuscript was based on discussions held at a meeting supported by Boehringer Ingelheim. The authors take full responsibility for the scope, direction, content of, and editorial decisions relating to the manuscript. They were involved at all stages of development and have approved the submitted manuscript. All authors contributed equally to the content of this article and have approved the final version. JG, JM and VS are the guarantors of the content of the manuscript. VS is senior clinical investigator of the Research Foundation\u2014Flanders (Belgium) (FWO) [1.8.029.20 N]. Medical writing assistance, in the form of the preparation and revision of the draft manuscript, was supported financially by Boehringer Ingelheim and provided by Hedwig Stals of HD Consultancy BV under the authors' conceptual direction and based on feedback from the authors. Boehringer Ingelheim was not involved in directing the content of the manuscript and was only given the opportunity to review the manuscript for factual accuracy. All recommendations stated in the manuscript are thus reflective of the authors' opinions, and as such are not necessarily supported by Boehringer Ingelheim.
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