4.5 Review

The usual Interstitial pneumonia pattern in autoimmune rheumatic diseases

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Lung Damage in Rheumatoid Arthritis-A Retrospective Study

Georgiana Dinache et al.

Summary: This study aimed to assess the clinical characteristics of rheumatoid arthritis (RA) patients with interstitial lung disease (ILD) and determine their association with X-ray and high-resolution computed tomography (HR-CT) findings. Results showed that male RA patients had a higher prevalence of nodules, combined fibrosis and nodules, and combined bronchiectasis and nodules on chest X-rays, while rheumatoid factor (RF)-positive patients had a higher prevalence of fibrosis and anti-cyclic citrullinated peptide antibodies (ACPA)-positive patients had a higher prevalence of bronchiectasis. In terms of HR-CT findings, patients on methotrexate treatment had a higher prevalence of nodules, combined fibrosis and nodules, combination of emphysema and nodules, and combination of fibrosis, emphysema, and nodules. ILD was found to develop within approximately 5 years from RA diagnosis, and it was more prevalent in men with RA, among patients with positive RA serology (RF and/or ACPA), and RA patients on methotrexate.

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Usual interstitial pneumonia as a stand-alone diagnostic entity: the case for a paradigm shift?

Moises Selman et al.

Summary: Usual interstitial pneumonia (UIP) has a distinctive appearance that has been considered the hallmark of idiopathic pulmonary fibrosis (IPF), but it is also seen in other fibrotic interstitial lung diseases. This article advocates for the designation of UIP as a single diagnostic entity, including its primary form and secondary processes in various disorders. The current separation between primary and secondary UIP does not reflect the similarities between them in terms of appearance, behavior, pathogenesis, and efficacy of treatment.

LANCET RESPIRATORY MEDICINE (2023)

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Evolution of Rheumatoid-Arthritis-Associated Interstitial Lung Disease in Patients Treated with JAK Inhibitors: A Retrospective Exploratory Study

Vincenzo Venerito et al.

Summary: The aim of this multicenter retrospective study was to investigate the effectiveness and safety of JAK-inhibitors (JAKi) in patients with rheumatoid arthritis (RA) and interstitial lung disease (ILD). The study suggests that JAKi therapy might be a safe therapeutic option for patients with RA-ILD in a short-term follow-up.

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Safety, tolerability, and efficacy of pirfenidone in patients with rheumatoid arthritis-associated interstitial lung disease: a randomised, double-blind, placebo-controlled, phase 2 study

Joshua J. Solomon et al.

Summary: This study evaluated the safety, tolerability, and efficacy of pirfenidone for the treatment of patients with rheumatoid arthritis-associated interstitial lung disease (RA-ILD). The results showed that although it did not meet the primary endpoint, pirfenidone slowed the rate of decline in lung function in patients with RA-ILD.

LANCET RESPIRATORY MEDICINE (2023)

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Current and Future Treatment Landscape for Idiopathic Pulmonary Fibrosis

Francesco Bonella et al.

Summary: Idiopathic pulmonary fibrosis (IPF) is a disease with poor survival and complex pathogenesis. While current drugs can slow disease progression, there is a need for new treatment strategies. Several new drugs are currently in clinical development, but our understanding of IPF is still limited.
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Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline

Ganesh Raghu et al.

Summary: This article provides updates on the guidelines for idiopathic pulmonary fibrosis (IPF) and addresses the progression of pulmonary fibrosis in patients with interstitial lung diseases (ILDs) other than IPF. The recommendations are based on consensus and systematic reviews, aiming to provide evidence-based guidance for clinicians.

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Canonical and noncanonical regulatory roles for JAK2 in the pathogenesis of rheumatoid arthritis-associated interstitial lung disease and idiopathic pulmonary fibrosis

Shaohua Wang et al.

Summary: This study uses RNA sequencing of lung biopsies from patients with RA-ILD and IPF to identify shared and distinct disease-causing pathways. The results suggest that JAK2 plays a crucial role in the development of fibrotic lung diseases.

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The histologic diagnosis of usual interstitial pneumonia of idiopathic pulmonary fibrosis. Where we are and where we need to go

Maxwell L. Smith

Summary: This manuscript reviews the evolution of histologic diagnosis of UIP, its relationship with IPF, and methods for distinguishing fibrotic lung disease mimics of UIP/IPF.

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Eric L. Matteson et al.

Summary: In fibrosing autoimmune disease-related interstitial lung diseases (ILDs) with a progressive phenotype, nintedanib demonstrated efficacy in slowing the rate of decline in forced vital capacity (FVC), with manageable adverse events.

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A Risk Score to Detect Subclinical Rheumatoid Arthritis-Associated Interstitial Lung Disease

Pierre-Antoine Juge et al.

Summary: This study developed and validated a risk score to identify high-risk patients of subclinical RA-ILD, with findings supporting an important contribution of MUC5B rs35705950 to the risk of subclinical RA-ILD.

ARTHRITIS & RHEUMATOLOGY (2022)

Review Respiratory System

Current best clinical practices for monitoring of interstitial lung disease

Elisabeth Bendstrup et al.

Summary: Interstitial lung diseases (ILDs) are a heterogeneous group of inflammatory and/or fibrotic conditions with variable outcome and often a dismal prognosis. Monitoring of ILDs is a multimodality process and includes measurement of pulmonary function and exercise capacity, symptom registration and quality of life (QoL), imaging, comorbidities and/or involvement of other organs. Future strategies for monitoring ILDs may include telemedicine, artificial intelligence, and new imaging tools.

EXPERT REVIEW OF RESPIRATORY MEDICINE (2022)

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Pharmacological treatment for connective tissue disease-associated interstitial lung involvement: Protocol for an overview of systematic reviews and meta-analyses

Fotini Karassa et al.

Summary: This overview aims to summarize existing evidence on the effectiveness and harm of pharmacological therapies for adults with CTD-ILD. A literature search will be conducted to identify systematic reviews with or without meta-analysis that examine pharmacological treatment for CTD-ILD. The primary outcomes will be changes in lung function measures and adverse events.

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Maria Molina-Molina et al.

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Immunomodulatory treatment of interstitial lung disease

Laura van den Bosch et al.

Summary: Interstitial lung diseases (ILDs) other than idiopathic pulmonary fibrosis (IPF) have more immunomodulatory treatment options due to their inflammatory component. However, there is a lack of guidance on managing this group of diseases. Immunosuppression is the main therapy for ILDs, with different levels of evidence for each specific ILD. Classification of ILDs is crucial for treatment decisions.

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Progressive interstitial lung disease in patients with systemic sclerosis-associated interstitial lung disease in the EUSTAR database

Anna-Maria Hoffmann-Vold et al.

Summary: This study utilized data from the European Scleroderma Trials And Research (EUSTAR) database to analyze the disease course, progression patterns, and predictive factors for progressive interstitial lung disease (ILD) in patients with systemic sclerosis-associated ILD (SSc-ILD). The results showed that male sex, higher modified Rodnan skin score, and reflux/dysphagia symptoms were the strongest predictive factors for forced vital capacity (FVC) decline over a 5-year period in SSc-ILD patients.

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Amy Olson et al.

Summary: Through a systematic review of ILD prevalence, it was found that 13-40% of ILDs may progress to a fibrosing phenotype, with overall prevalence estimates of 2.2-20.0 per 100,000 in Europe and 28.0 per 100,000 in the USA for progressive fibrosing ILDs. Prevalence estimates for individual progressive fibrosing ILDs varied up to 16.7 per 100,000 people. These conditions represent a significant portion of chronic respiratory disorders and have high unmet needs.

ADVANCES IN THERAPY (2021)

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Two sides of the same coin? A review of the similarities and differences between idiopathic pulmonary fibrosis and rheumatoid arthritis-associated interstitial lung disease

Scott Matson et al.

Summary: Although RA-ILD and IPF are distinct diseases, they share several clinical, radiographic, and genetic features, sparking interest in their relationship. Understanding the links between these diseases could fundamentally change strategies for the diagnosis, screening, and treatment of ILD.

EUROPEAN RESPIRATORY JOURNAL (2021)

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Volume-related structures predict UIP pathology in those with a non-IPF pattern on CT

Jonathan H. Chung et al.

Summary: The study indicates that VRS can differentiate patients with UIP histological features from non-IPF diagnosis in CT images, showing predictive value. The sensitivity and specificity of VRS in predicting pathological UIP are comparable to standard qualitative CT assessment.

EUROPEAN RADIOLOGY (2021)

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Rheumatoid arthritis related interstitial lung disease

Andreina Manfredi et al.

Summary: Rheumatoid arthritis-related interstitial lung disease is a common extra-articular manifestation that significantly impacts patients' quality of life and overall prognosis, necessitating early diagnosis and treatment. High-resolution computed tomography is the gold standard for ILD diagnosis, but determining the correct therapeutic strategy remains challenging due to the high variability of clinical presentation and disease progression. A multidisciplinary approach involving rheumatologists, pulmonologists, and radiologists is currently recommended to define optimal therapy and follow-up strategies.

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Interstitial Lung Disease in Connective Tissue Diseases: Survival Patterns in a Population-Based Cohort

Charlotte Hyldgaard et al.

Summary: The study found that the frequency of ILD and pulmonary hypertension varied among different connective tissue disease subtypes. The five-year survival rate was lowest in SSc-ILD and highest in MCTD-ILD, myositis-ILD, SLE-ILD, and Sjogren-associated ILD. Overall, the survival rate was lower in CTD-ILD compared to CTD alone across different age groups.

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Systematic review of the impact of drugs on diffuse interstitial lung disease associated with rheumatoid arthritis

Carmen Carrasco Cubero et al.

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Pirfenidone in patients with progressive fibrotic interstitial lung diseases other than idiopathic pulmonary fibrosis (RELIEF): a double-blind, randomised, placebo-controlled, phase 2b trial

Juergen Behr et al.

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LANCET RESPIRATORY MEDICINE (2021)

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Efficacy and safety of abatacept in interstitial lung disease of rheumatoid arthritis: A systematic literature review

Esther F. Vicente-Rabaneda et al.

Summary: Current evidence suggests that ABA may be a plausible alternative to treat RA patients with ILD. It would be highly desirable to develop prospective randomized controlled studies to confirm these findings.

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Computed Tomography Findings Suggestive of Connective Tissue Disease in the Setting of Usual Interstitial Pneumonia

Jonathan H. Chung et al.

Summary: In patients with UIP pattern, the presence of SES or AULS is more common in CTD-ILD than IPF. The presence of SES is associated with improved survival, especially in IPF patients. AULS is linked to pulmonary functional abnormalities.

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Disease pathology in fibrotic interstitial lung disease: is it all about usual interstitial pneumonia?

Elisabetta A. Renzoni et al.

Summary: Interstitial pneumonias encompass a diverse group of diseases with distinct histopathological features, often defined by their cause. There is a growing focus on the progressive fibrosing phenotype of interstitial lung disease, particularly due to the failure of conventional therapies in some patients and the widespread use of antifibrotic therapies. Apart from the typical usual interstitial pneumonia, other histological entities are also associated with progressive fibrosis, and techniques like immunohistochemistry and single-cell RNA sequencing are providing pathogenetic insights that could impact the pathological classification.

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Factors associated with mortality in rheumatoid arthritis-associated interstitial lung disease: a systematic review and meta-analysis

Meihua Qiu et al.

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Natural history and screening of interstitia lung disease in systemic autoimmune rheumatic disorders

Panagiotis Panagopoulos et al.

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Kristine E. Konopka et al.

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