4.1 Review

Progressive fibrotic interstitial lung disease

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Review Respiratory System

Progressive pulmonary fibrosis: an expert group consensus statement

Sujeet K. Rajan et al.

Summary: This expert group consensus statement highlights the importance of standardizing the definition of progressive fibrosing interstitial lung diseases (F-ILDs) and the need for accurate initial diagnosis. Case-by-case decisions on monitoring and management are essential due to the varying presentations and rates of progression. The guidance also emphasizes the value of diagnostic tests in risk stratification and the use of early antifibrotic therapy in progressive pulmonary fibrosis (PPF).

EUROPEAN RESPIRATORY JOURNAL (2023)

Article Rheumatology

Survival and prognostic factors from a multicentre large cohort of unselected Italian systemic sclerosis patients

Fabio Cacciapaglia et al.

Summary: The survival and death prognostic factors of SSc patients have varied during the past decades. This study aimed to update the 5- and 10-year survival rates of Italian SSc patients diagnosed after 2009 and identify prognostic factors. The results showed that in the past decade, SSc patients reached similar mortality rates as the Italian general population. Male gender, diffuse cutaneous involvement, comorbidities, and PAH with or without ILD were identified as the main poor prognostic factors.

RHEUMATOLOGY (2023)

Article Rheumatology

Prognostic value of automated assessment of interstitial lung disease on CT in systemic sclerosis

Aelle Le Gall et al.

Summary: A deep-learning-based algorithm for automated quantification of ILD on HRCT can effectively assess the extent of ILD and predict the risk of death in SSc patients.

RHEUMATOLOGY (2023)

Article Respiratory System

Pirfenidone in fibrotic hypersensitivity pneumonitis: a double-blind, randomised clinical trial of efficacy and safety

Evans R. Fernandez Perez et al.

Summary: In this study, the safety and effect of pirfenidone on disease progression in fibrotic hypersensitivity pneumonitis (FHP) patients were evaluated. The results showed that pirfenidone improved the relative decline in forced vital capacity (FVC%) and increased the progression-free survival (PFS) of these patients. Overall, pirfenidone was found to be safe and effective in controlling the disease in FHP patients.

THORAX (2023)

Article Medicine, General & Internal

Prognostication of progressive pulmonary fibrosis in connective tissue disease-associated interstitial lung diseases: A cohort study

Yu-Hsiang Chiu et al.

Summary: This study examines the prognostic relevance of different definitions of progressive pulmonary fibrosis (PPF) in patients with CTD-ILD. The results show that higher age, smoking, increased extent of fibrosis, and low baseline DLCO are associated with poor prognosis, while regular pulmonary function evaluation is associated with better survival. The INBUILD, ATS/ERS/JRS/ALAT 2022, and simplified PF criteria have similar prognostic implications in two cohorts.

FRONTIERS IN MEDICINE (2023)

Article Medicine, General & Internal

Fibrosis score predicts mortality in patients with fibrotic hypersensitivity pneumonitis

Ju Hyun Oh et al.

Summary: This study evaluated the usefulness of radiological parameters in predicting mortality in patients with fibrotic HP. The results showed that the fibrosis score is an independent prognostic factor for mortality in these patients, with higher fibrosis scores associated with higher mortality rates.

FRONTIERS IN MEDICINE (2023)

Article Biology

Predictors of Progression and Mortality in Patients with Chronic Hypersensitivity Pneumonitis: Retrospective Analysis of Registry of Fibrosing Interstitial Lung Diseases

Natalia V. V. Trushenko et al.

Summary: This study aimed to identify predictors of disease progression and mortality in patients with chronic hypersensitivity pneumonitis (HP) in real clinical practice. The significant predictors of adverse outcomes were a DLco < 50% predicted, an SpO2 at the end of a six-minute walk test (6-MWT) < 85%, and a GAP score >= 4 points. Early detection of these predictors is necessary for timely initiation of antifibrotic therapy in patients with chronic HP.

LIFE-BASEL (2023)

Article Respiratory System

Lung function trajectory in progressive fibrosing interstitial lung disease

Justin M. Oldham et al.

Summary: This study found that the change in lung function after satisfying progressive fibrosing interstitial lung disease (PF-ILD) criteria is closely associated with radiological progression of fibrosis. However, the impact may vary depending on the specific subtype of interstitial lung disease (ILD). These findings can inform future clinical trial design and emphasize the importance of considering ILD subtype when applying PF-ILD criteria.

EUROPEAN RESPIRATORY JOURNAL (2022)

Review Rheumatology

Acute exacerbation of interstitial lung disease associated with rheumatic disease

Fabrizio Luppi et al.

Summary: This review summarizes the clinical features, diagnosis, management, and prognosis of acute exacerbation of interstitial lung disease in patients with rheumatic diseases. It highlights the lack of specific definition and evidence-based therapeutic strategies for this condition, underscoring the need for further research and development in this area.

NATURE REVIEWS RHEUMATOLOGY (2022)

Article Respiratory System

Nintedanib in progressive interstitial lung diseases: data from the whole INBUILD trial

Kevin R. Flaherty et al.

Summary: This study demonstrates that nintedanib can slow the decline in lung function, reduce the risk of ILD progression, and decrease the proportion of acute exacerbations or deaths in patients with fibrosing interstitial lung diseases.

EUROPEAN RESPIRATORY JOURNAL (2022)

Article Respiratory System

Antigen identification and avoidance on outcomes in fibrotic hypersensitivity pneumonitis

Tananchai Petnak et al.

Summary: Our findings suggest that antigen identification and avoidance are associated with improved clinical outcomes in patients with fibrotic disease.

EUROPEAN RESPIRATORY JOURNAL (2022)

Article Pharmacology & Pharmacy

Prognostic Predictive Characteristics in Patients With Fibrosing Interstitial Lung Disease: A Retrospective Cohort Study

Yuanying Wang et al.

Summary: In patients with interstitial lung disease with a progressive fibrosing feature, clubbing of fingers and an HRCT-documented UIP-like fibrotic pattern are associated with fibrosis progression. Among patients with progressive fibrosing, those with hypoxemia, lower baseline DLCO% predicted, or UIP-like fibrotic pattern showed poor mortality.

FRONTIERS IN PHARMACOLOGY (2022)

Letter Respiratory System

The burden of Progressive Fibrotic Interstitial lung disease across the UK

Thomas Simpson et al.

EUROPEAN RESPIRATORY JOURNAL (2021)

Article Critical Care Medicine

Incidence and Prognostic Significance of Hypoxemia in Fibrotic Interstitial Lung Disease An International Cohort Study

Yet H. Khor et al.

Summary: Patients with idiopathic pulmonary fibrosis (IPF) have higher cumulative incidence of exertional and resting hypoxemia compared with patients without IPF. The extended ILD-GAP-O-2 model provides additional risk stratification for 1-year prognosis in patients with fibrotic ILD.
Article Pharmacology & Pharmacy

Predictors of Mortality in Progressive Fibrosing Interstitial Lung Diseases

Xianqiu Chen et al.

Summary: This study assessed clinical characteristics and outcomes of PF-ILD patients in a single-center cohort, finding that extent of fibrotic changes on HRCT and pulmonary hypertension were predictors of mortality in patients with PF-ILD.

FRONTIERS IN PHARMACOLOGY (2021)

Article Medicine, Research & Experimental

Insights on chronic hypersensitivity pneumonitis' treatment: Factors associated with a favourable response to azathioprine

Sara Raimundo et al.

Summary: The study found that azathioprine (AZA) is effective for some patients with chronic hypersensitivity pneumonitis (CHP) but ineffective for others. Bronchoalveolar lavage (BAL) lymphocytosis may be a useful strategy to identify AZA responders, although its accuracy is limited. Further research is needed to clarify other response markers to immunosuppressive agents for optimizing therapeutic options for this condition.

LIFE SCIENCES (2021)

Review Respiratory System

Thoracic computed tomography in the progressive fibrotic phenotype

Anand Devaraj et al.

Summary: Discussing the rationale and methods for determining progressive lung fibrosis on thoracic computed tomography is crucial to identify optimal treatment. Serial high-resolution computed tomography serves as a method of determining disease progression. Clinicians and radiologists need to understand the optimal methods and limitations for identifying changes in disease extent using serial CT scans.

CURRENT OPINION IN PULMONARY MEDICINE (2021)

Review Respiratory System

The justification for the progressive fibrotic phenotype

Martin RJ. Kolb et al.

Summary: Accurate diagnosis is crucial for determining appropriate therapy and predicting prognosis in the field of ILD. Different types of ILD may require different treatment approaches, but once fibrosis is present, the biological pathways responsible for progression may respond similarly to antifibrotic therapy.

CURRENT OPINION IN PULMONARY MEDICINE (2021)

Article Respiratory System

Efficacy of treatment with corticosteroids for fibrotic hypersensitivity pneumonitis: a propensity score-matched cohort analysis

Masaru Ejima et al.

Summary: The study demonstrated that corticosteroid treatment improved survival and slowed fibrotic progression in patients with fibrotic HP. Patients with less severe fibrosis may benefit from corticosteroid treatment. Early initiation of corticosteroids should be considered when worsening fibrosis is observed in fibrotic HP.

BMC PULMONARY MEDICINE (2021)

Review Respiratory System

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

Meihua Qiu et al.

Summary: Through this systematic review and meta-analysis, older age, male sex, smoking history, higher CPI, lower DLCO% predicted, lower FVC% predicted, UIP pattern on HRCT, emphysema presence, and acute exacerbation of ILD were found to be associated with increased risk of mortality in RA-ILD patients. However, rheumatoid factor (RF) positive status was not associated with mortality.

RESPIRATORY RESEARCH (2021)

Article Respiratory System

Acute exacerbation of fibrotic hypersensitivity pneumonitis: incidence and outcomes

Jieun Kang et al.

Summary: Acute exacerbation (AE) is not uncommon among patients with fibrotic hypersensitivity pneumonitis and significantly affects prognosis. Lower DLCO value and radiologic UIP-like pattern at diagnosis are associated with the development of AE in these patients. AE remains a significant predictor of all-cause mortality.

RESPIRATORY RESEARCH (2021)

Review Respiratory System

Prognostic factors in chronic hypersensitivity pneumonitis

Andrew W. Creamer et al.

EUROPEAN RESPIRATORY REVIEW (2020)

Review Respiratory System

The natural history of progressive fibrosing interstitial lung diseases

Martin Kolb et al.

RESPIRATORY RESEARCH (2019)

Review Respiratory System

Role of imaging in progressive-fibrosing interstitial lung diseases

Simon L. F. Walsh et al.

EUROPEAN RESPIRATORY REVIEW (2018)

Article Respiratory System

Acute exacerbation of idiopathic pulmonary fibrosis: shifting the paradigm

Christopher J. Ryerson et al.

EUROPEAN RESPIRATORY JOURNAL (2015)

Article Respiratory System

A haemodynamic study of pulmonary hypertension in chronic hypersensitivity pneumonitis

Rudolf K. F. Oliveira et al.

EUROPEAN RESPIRATORY JOURNAL (2014)