Discipline collision Respiratory, rheumatism majors reveal the secret of progressive fibrotic interstitial lung disease

In recent years, the concept of progressive fibrosing interstitial lung disease (Progressive fibrosing ILD, PF-ILD) has been frequently mentioned by various top journals, compared with the clinically well-known idiopathic pulmonary interstitial fibrosis (IPF) What is the significance of PF-ILD? What clinical problems can it solve? We are fortunate to have the Chief Physician Dai Huaping of the Department of Respiratory and Critical Care Medicine of the China-Japan Friendship Hospital and Chief Physician Lu Xin of the Department of Rheumatology and Immunology to help everyone understand their doubts
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1 Why is the concept of progressive fibrotic interstitial lung disease proposed and how is it defined? The clinical manifestations, imaging changes, and functional changes of a variety of non-IPF ILD diseases may be consistent with the characteristics of progressive fibrosis, such as: CTD-ILD, chronic allergic pneumonia, sarcoidosis, non-specific interstitial pneumonia, and drug-related ILD progressive There are many similarities between the development of ILD and IPF.
For example, the biological behavior of progressive development.
The mechanism of lung fibrosis.
The concept of PF-ILD is proposed to assign and treat ILD diseases with progressive phenotype.
2 Distinguish idiopathic lung fibers What is the purpose of the concepts of both morphogenetic and progressive fibrotic interstitial lung disease? In order to give reasonable treatment to this type of ILD with similar performance of IPF
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It is reasonable to extend anti-fibrosis treatment from IPF patients to PF-ILD
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3How to evaluate connective tissue disease and progressive fibrotic interstitial lung disease? Many connective tissue diseases have interstitial lung disease manifestations, and some show progressive development; assessing the activity of connective tissue diseases requires simultaneous assessment of systemic and pulmonary diseases: assessment of systemic diseases by rheumatology indicators, such as: Rodnan's Modification of Scleroderma Skin assessment; Rheumatoid Arthritis Disease Activity Score (DAS28); Myositis MyoAct Muscle Involvement Score; Assessment of lung lesions by respiratory indicators, such as: lung function; imaging examination; walking test; blood gas analysis; 4 which It is more common for connective tissue disease to develop into progressive fibrotic interstitial lung disease, and how to deal with it? Many diffuse connective tissue diseases are easy to merge with ILD, and a large part of them will become PF-ILD.
For example, scleroderma, rheumatoid myositis, Sjogren’s syndrome, systemic lupus erythematosus, mixed connective tissue disease, research results show that 16%- Can 40% of CTD-ILD patients be included in the standardized process of PF-ILD5 management of progressive fibrotic interstitial lung disease? When progressive pulmonary fibrosis occurs, anti-fibrosis treatment can be started; studies have shown that starting treatment with mild lung function damage, the greater the benefit of delaying lung function decline; regular follow-up: lung function: every 3-6 Months; HRCT: The interval can be slightly longer, or as needed; Life status assessment: Every 3-6 months 6 The concept of treatment and management of progressive fibrotic lung disease, "How early is not too early, and how late is not counted Is it reasonable to be late? From the perspective of disease diagnosis and treatment, patients with early diagnosis and treatment benefit more 7 What are the most important results and hints of the INBUILD study? The results of the INBUILD study were published in the New England Journal in 2019 and are currently the largest drug study on PF-ILD; INBUILD study: a placebo-controlled study of nintedanib in the treatment of PF-ILD.
Nintedanib significantly delayed the reduction of FVC in patients FVC decline rate is 57%.
The difference between FVC groups is 107ml/year.
The effect of delaying lung function decline is not affected by whether HRCT is UIP or not.
Nintanib is currently the only chronic fibrotic interstitial with a progressive phenotype.
Drugs for "Lung Disease" Indications Conclusion The two experts discussed the diagnosis and treatment of PF-ILD from the perspectives of respiratory and rheumatism and immunity
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PF-ILD is a large group of diseases with progressive pulmonary fibrosis.
Once diagnosed, the initiation of anti-fibrosis treatment should be considered
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In terms of drug selection, the results of the INBUILD study confirmed the application value of nintedanib in patients with PF-ILD, and therefore became the only one currently receiving the indication of "chronic fibrotic interstitial lung disease with a progressive phenotype" Drugs
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Typesetting: Li Hui Editor: Li Hui Review: Xu Fengyan For more information, please click: Over 60% of newly diagnosed patients have insufficient understanding of cataract disease | China Cataract Awareness and Diagnosis and Treatment Status Survey Report Released 2021 China Atrial Fibrillation Day | Professor Ma Changsheng: Atrial Fibrillation Rhythm The era of control is coming.
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