The Spectrum of Interstitial Lung Disease Associated with Autoimmune Diseases: Data of a 3.6-Year Prospective Study from a Referral Center of Interstitial Lung Disease and Lung Transplantation †

Atienza‐Mateo, Belén; Remuzgo‐Martínez, Sara; Cuesta, Víctor Manuel Mora; Iturbe-Fernández, David; Fernández-Rozas, Sonia; Prieto-Peña, D.; Calderón-Goercke, Monica; Corrales, Alfonso; Rodríguez, Gerardo Blanco; Gómez-Román, Javier; González-Gay, Miguel Á.; Cifrián, José Manuel

doi:10.3390/jcm9061606

Cited by 23 publications

(27 citation statements)

References 51 publications

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“…The inconsistent terminology and definitions across studies resulted in different prevalence data and reflect the absence of established diagnostic criteria [26]. In our study, the uILD cohort represented approximately 15% of the total ILD cohort, and approximately 26% of the IIP cohort, similar to the study by Atienza-Mateo et al [27]. The study by Ryerson et al reported a percentage of uILD of 10% of the ILD cohort [28].…”

Section: Discussionsupporting

confidence: 74%

Clinical and Functional Characteristics of Patients with Unclassifiable Interstitial Lung Disease (uILD): Long-Term Follow-Up Data from European IPF Registry (eurIPFreg)

Krauss

El-Guelai

Pons-Kuehnemann

et al. 2020

JCM

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: (1) Aim of the study: In spite of extensive research, up to 20% of interstitial lung diseases (ILD) patients cannot be safely classified. We analyzed clinical features, progression factors, and outcomes of unclassifiable ILD (uILD). (2) Methods: A total of 140 uILD subjects from the University of Giessen and Marburg Lung Center (UGMLC) were recruited between 11/2009 and 01/2019 into the European Registry for idiopathic pulmonary fibrosis (eurIPFreg) and followed until 01/2020. The diagnosis of uILD was applied only when a conclusive diagnosis could not be reached with certainty. (3) Results: In 46.4% of the patients, the uILD diagnosis was due to conflicting clinical, radiological, and pathological data. By applying the diagnostic criteria of usual interstitial pneumonia (UIP) based on computed tomography (CT), published by the Fleischner Society, 22.2% of the patients displayed a typical UIP pattern. We also showed that forced vital capacity (FVC) at baseline (p = 0.008), annual FVC decline ≥10% (p < 0.0001), smoking (p = 0.033), and a diffusing capacity of the lung for carbon monoxide (DLco) ≤55% of predicted value at baseline (p < 0.0001) were significantly associated with progressive disease. (4) Conclusions: The most important prognostic factors in uILD are baseline level and decline in lung function and smoking. The use of Fleischner diagnostic criteria allows further differentiation and accurate diagnosis.

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Section: Discussionsupporting

confidence: 74%

Clinical and Functional Characteristics of Patients with Unclassifiable Interstitial Lung Disease (uILD): Long-Term Follow-Up Data from European IPF Registry (eurIPFreg)

Krauss

El-Guelai

Pons-Kuehnemann

et al. 2020

JCM

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“…Although ILD frequently develops in patients with established RA, it may also be presented as the initial or single manifestation of an unidentified RA [ 1 , 2 , 3 ]. In this regard, the identification of an underlying RA in ILD patients remains a challenge, considering that RA-ILD + shares clinical, pathological, and epidemiological similarities with idiopathic pulmonary fibrosis (IPF), the most common and severe ILD [ 2 , 3 , 4 ]. Consequently, some patients initially defined as having IPF may be finally diagnosed with RA-ILD + [ 4 ].…”

Section: Introductionmentioning

confidence: 99%

Endothelial Progenitor Cells as a Potential Biomarker in Interstitial Lung Disease Associated with Rheumatoid Arthritis

Pulito‐Cueto

Remuzgo‐Martínez

Genre

et al. 2020

JCM

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Interstitial lung disease (ILD) increases morbidity and mortality in patients with rheumatoid arthritis (RA). Although the pathogenesis of ILD associated with RA (RA-ILD+) remains poorly defined, vascular tissue is crucial in lung physiology. In this context, endothelial progenitor cells (EPC) are involved in endothelial tissue repair. However, little is known about their implication in RA-ILD+. Accordingly, we aimed to investigate the potential role of EPC related to endothelial damage in RA-ILD+. EPC quantification in peripheral blood from 80 individuals (20 RA-ILD+ patients, 25 RA-ILD− patients, 21 idiopathic pulmonary fibrosis (IPF) patients, and 14 healthy controls) was performed by flow cytometry. EPC were considered as CD34+, CD45low, CD309+ and CD133+. A significant increase in EPC frequency in RA-ILD+ patients, as well as in RA-ILD− and IPF patients, was found when compared with controls (p < 0.001, p = 0.02 and p < 0.001, respectively). RA-ILD+ patients exhibited a higher EPC frequency than the RA-ILD− ones (p = 0.003), but lower than IPF patients (p < 0.001). Our results suggest that EPC increase may represent a reparative compensatory mechanism in patients with RA-ILD+. The degree of EPC frequency may help to identify the presence of ILD in RA patients and to discriminate RA-ILD+ from IPF.

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“…However, in clinical practice, it is common to come across patients with an “idiopathic” interstitial pneumonia (IIP) associated with features suggestive of, but not diagnostic for, a classical CTD [ 5 , 6 ].…”

Section: Introductionmentioning

confidence: 99%

Interstitial Pneumonia with Autoimmune Features: Why Rheumatologist-Pulmonologist Collaboration Is Essential

et al. 2020

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In 2015 the European Respiratory Society (ERS) and the American Thoracic Society (ATS) “Task Force on Undifferentiated Forms of Connective Tissue Disease-associated Interstitial Lung Disease” proposed classification criteria for a new research category defined as “Interstitial Pneumonia with Autoimmune Features” (IPAF), to uniformly define patients with interstitial lung disease (ILD) and features of autoimmunity, without a definite connective tissue disease. These classification criteria were based on a variable combination of features obtained from three domains: a clinical domain consisting of extra-thoracic features, a serologic domain with specific autoantibodies, and a morphologic domain with imaging patterns, histopathological findings, or multicompartment involvement. Features suggesting a systemic vasculitis were excluded. Since publication of ERS/ATS IPAF research criteria, various retrospective studies have been published focusing on prevalence; clinical, morphological, and serological features; and prognosis of these patients showing a broad heterogeneity in the results. Recently, two prospective, cohort studies were performed, confirming the existence of some peculiarities for this clinical entity and the possible progression of IPAF to a defined connective tissue disease (CTD) in about 15% of cases. Moreover, a non-specific interstitial pneumonia pattern, an anti-nuclear antibody positivity, and a Raynaud phenomenon were the most common findings. In comparison with idiopathic pulmonary fibrosis (IPF), IPAF patients showed a better performance in pulmonary function tests and less necessity of oxygen delivery. However, at this stage of our knowledge, we believe that further prospective studies, possibly derived from multicenter cohorts and through randomized control trials, to further validate the proposed classification criteria are needed.

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The Spectrum of Interstitial Lung Disease Associated with Autoimmune Diseases: Data of a 3.6-Year Prospective Study from a Referral Center of Interstitial Lung Disease and Lung Transplantation †

Cited by 23 publications

References 51 publications

Clinical and Functional Characteristics of Patients with Unclassifiable Interstitial Lung Disease (uILD): Long-Term Follow-Up Data from European IPF Registry (eurIPFreg)

Clinical and Functional Characteristics of Patients with Unclassifiable Interstitial Lung Disease (uILD): Long-Term Follow-Up Data from European IPF Registry (eurIPFreg)

Endothelial Progenitor Cells as a Potential Biomarker in Interstitial Lung Disease Associated with Rheumatoid Arthritis

Interstitial Pneumonia with Autoimmune Features: Why Rheumatologist-Pulmonologist Collaboration Is Essential

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