scholarly journals Interstitial lung diseases with progressive pulmonary fibrosis: pathogenetic features and approaches to therapy

2020 ◽  
pp. 99-106
Author(s):  
N. A. Kuzubova ◽  
O. N. Titova ◽  
D. B. Skliarova
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Interstitial Pneumonia ◽  
Lung Diseases ◽  
Usual Interstitial Pneumonia ◽  
Connective Tissue Diseases ◽  
Immunosuppressive Drugs ◽  
Interstitial Lung Diseases ◽  
Fibrotic Process ◽  
Number Of Patients

A number of patients with interstitial lung diseases (ILD) of various etiologies, including hypersensitive pneumonitis, diffuse connective tissue diseases (rheumatoid arthritis, systemic scleroderma, dermatomyositis), sarcoidosis, idiopathic non-specific interstitial pneumonia (NSIP) and unclassified ILD develop rapid deterioration of lung ventilation function due to the progression of fibrotic changes, accompanied by a decrease in physical performance and quality of life. It is proposed to distinguish a progressive fibrotic phenotype from those with similar pathogenetic mechanisms, radiologic pattern, clinical course, and prognosis. The progressive course of the fibrotic process is assessed by reducing the forced vital capacity of the lungs (FVC), increasing the severity of signs of pulmonary fibrosis according to computed tomography (CT) and worsening respiratory symptoms. There are several risk factors for the progression of ILD, such as male gender, older age, lower initial pulmonary function, and radiological or pathological picture of usual interstitial pneumonia (UIP). Currently, the role of antifibrotic drugs in the treatment of this pathology is being actively studied. Previously, the common approach was to use this group of drugs in patients with idiopathic pulmonary fibrosis (IPF) and immunosuppressive drugs in patients with other fibrotic subtypes of IL. However, the results of clinical studies have shown a favorable response to antifibrotic therapy for a wider range of fibrotic ILD, manifested in a decrease in the annual rate of FVC reduction. And in 2020, the use of the first anti-fibrotic drug was approved for the treatment of patients with advanced pulmonary fibrosis, NOT related to idiopathic pulmonary fibrosis (IPF).

Idiopathic Pulmonary Fibrosis

Chest Imaging ◽  
2019 ◽  
pp. 453-457
Author(s):  
Cylen Javidan-Nejad
Keyword(s):  
Lung Cancer ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Pulmonary Edema ◽  
Interstitial Pneumonia ◽  
Lung Diseases ◽  
Usual Interstitial Pneumonia ◽  
Primary Lung Cancer ◽  
Interstitial Lung Diseases ◽  
Pathologic Diagnosis

Idiopathic pulmonary fibrosis (IPF) represents one of the most common chronic interstitial lung diseases. Usual interstitial pneumonia (UIP) is the pathologic diagnosis of IPF and can be diagnosed when honeycombing is present with a basilar and peripheral predominance and findings not typical of UIP are absent. In the current era, when a diagnosis of UIP is made with confidence on HRCT, biopsy can be avoided. Yet, one must be familiar with mimics of UIP/IPF (most notably pulmonary edema superimposed on emphysema) to avoid confusion misdiagnosis. Radiologists must also be familiar with potential complications of UIP including progression, infection, accelerated fibrosis (which can be lethal) and primary lung cancer (which has an increased incidence in UIP).

scholarly journals Usual interstitial pneumonia - secondary vs idiopathic pulmonary fibrosis

2019 ◽  
Vol 8 (1) ◽  
pp. 20
Author(s):  
Divyendu Sharma ◽  
Rajesh Agrawal ◽  
Rajat Agarwal ◽  
Amit Kumar ◽  
Utkarsh Gupta
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Interstitial Pneumonia ◽  
Usual Interstitial Pneumonia ◽  
Underlying Disease ◽  
Connective Tissue Disorder ◽  
Interstitial Lung Diseases ◽  
Specific Pattern ◽  
Small Airways ◽  
History Of

Background: Interstitial Lung Diseases is a group of disorders where the pulmonary interstitium, alveolar structures and the small airways are affected. Identification of a specific pattern on HRCT, with a thorough clinical evaluation can help a physician in narrowing down the differential diagnosis for the underlying cause. Usual Interstitial Pneumonia (UIP) is a frequently identified pattern. Differentiating patients with definite UIP pattern, into IPF and non-IPF spectrums is important. Aim of this study is to compare UIP patients with a secondary cause vs Idiopathic Pulmonary Fibrosis.Methods: Statistically 33 patients having UIP pattern on HRCT were evaluated based on the history of extrapulmonary symptoms, environmental exposure, drugs and subsequent serology testing. Patients were divided into two groups - IPF and UIP with a secondary cause. Both groups were compared on various clinical parameters. Inferences were drawn from the same.Results: Total 66.6% patients were identified to have Idiopathic Pulmonary Fibrosis, 33.3% had UIP with a secondary cause. Majority of patients with a secondary cause had Connective Tissue Disorder (90.9%) and one patient of Chronic Hypersensitivity Pneumonitis (HP).Conclusions: Absence of extrapulmonary symptoms in UIP patients need no further investigations and can be diagnosed as a case of IPF. However, presence of extrapulmonary symptoms needs further evaluation to diagnose the underlying disease and start treatment for the same.

scholarly journals Making an Accurate Diagnosis of Chronic Hypersensitivity Pneumonitis

2014 ◽  
Vol 21 (6) ◽  
pp. 370-372 ◽  
Author(s):  
Kerri A Johannson ◽  
Christopher J Ryerson
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Present Article ◽  
Interstitial Pneumonia ◽  
Lung Diseases ◽  
Hypersensitivity Pneumonitis ◽  
Interstitial Lung Diseases ◽  
Accurate Diagnosis ◽  
Management Approach ◽  
Chronic Hypersensitivity Pneumonitis

Chronic hypersensitivity pneumonitis (HP) arises from repeated exposure to causative antigens. Although HP can be challenging to diagnose, it is important to differentiate from idiopathic pulmonary fibrosis and idiopathic non-specific interstitial pneumonia. HP has a unique management approach and portends a unique prognosis. The present article summarizes the recent published literature on chronic HP and highlights the features that may be helpful in distinguishing it from other chronic interstitial lung diseases.

scholarly journals Clinico-radiological profile of connective tissue disease related-interstitial lung diseases from a tertiary care centre of India: a cross sectional study

2021 ◽  
Author(s):  
Mehul Agarwal ◽  
Manohar Lal Gupta ◽  
Kunal Deokar ◽  
Benhur Joel Shadrach ◽  
Neha Bharti ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Interstitial Pneumonia ◽  
Lung Diseases ◽  
Tertiary Care ◽  
Usual Interstitial Pneumonia ◽  
Connective Tissue Diseases ◽  
Clinical Manifestations ◽  
Interstitial Lung Diseases ◽  
Western India ◽  
Tertiary Care Centre

Interstitial lung diseases (ILDs) are a frequently occurring pulmonary manifestation in patients of connective tissue diseases (CTD). Detailed understanding of this subset of lung diseases is vital, hence the study was conducted to analyze the clinico-radiological characteristics of CTD-ILD. The present study was conducted between March 2017 to February 2018 at a tertiary care teaching hospital from western India. A total of 100 patients having respiratory symptoms suggestive of ILD, who were either diagnosed cases of CTD or had clinical manifestations of underlying CTD, were included in the study.  27% of patients belonged to age group 41-50 years and 78% were females. Chief respiratory complaints were dyspnea and cough. Clubbing was present in 29% patients. The most common CTDs were rheumatoid arthritis [RA] (26%), systemic sclerosis [SSC] (21%), mixed connective tissue disorder [MCTD] (19%) and Sjogren’s syndrome [SS] (16%). Restrictive defect on spirometry was seen in 58% cases and was most significant among patients with RA (65%) and SS (62%). Pulmonary arterial hypertension was seen in 40% cases and was most evident in MCTD (78%) and SSC patients (58%). Chest radiograph was normal in 47% of subjects. Most common radiological pattern on high resolution computed tomography (HRCT) thorax was non-specific interstitial pneumonia (42%) followed by usual interstitial pneumonia (21%). ILD is a common manifestation of CTD. Work-up for an underlying CTD should be offered to all ILD patients and vice versa.

High‐resolution CT in smoking‐related interstitial lung diseases

2021 ◽  
Vol 25 (2) ◽  
pp. 106-112
Author(s):  
E. Carlicchi ◽  
A. Caminati ◽  
P. Fughelli ◽  
G. Pelosi ◽  
S. Harari ◽  
...  
Keyword(s):  
High Resolution ◽  
Pulmonary Fibrosis ◽  
Interstitial Pneumonia ◽  
Lung Diseases ◽  
Usual Interstitial Pneumonia ◽  
Interstitial Lung Diseases ◽  
Pathological Process ◽  
Chronic Obstructive ◽  
High Resolution Computed Tomography ◽  
Obstructive Pulmonary Disease

In addition to chronic obstructive pulmonary disease (COPD) and bronchogenic carcinoma, smoking can also cause interstitial lung diseases (ILDs) such as respiratory bronchiolitis (RB), RB with ILD (RB‐ILD), desquamative interstitial pneumonia (DIP), Langerhans cell granulomatosis (LCG) and idiopathic pulmonary fibrosis‐usual interstitial pneumonia (IPF‐UIP). However, smoking seems to have a protective effect against hypersensitivity pneumonitis (HP), sarcoidosis and organising pneumonia (OP). High‐resolution computed tomography (HRCT) has a pivotal role in the differential diagnosis. RB is extremely frequent in smokers, and is considered a marker for smoking exposure. It has no clinical relevance in itself since most patients with RB are asymptomatic. It is frequent to observe the association of RB with other smoking-related diseases, such as LCG or pulmonary neoplasms. In RB‐ILD, HRCT features are more conspicuous and diffuse than in RB, but there is no definite cut‐off between the two entities and any distinction can only be made by integrating imaging and clinical data. RB, RB‐ILD and DIP may represent different degrees of the same pathological process, consisting in a bronchiolar and alveolar inflammatory reaction to smoking. Smoking is also a well‐known risk factor for pulmonary fibrosis. Multidisciplinary discussion and follow‐up can generally solve even the most difficult cases.

scholarly journals Idiopathic pulmonary fibrosis: possibilities of multidisciplinary diagnostic approach

2018 ◽  
Vol 28 (5) ◽  
pp. 622-625
Author(s):  
A. М. Kardangusheva ◽  
Н. A. Sabanchieva
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Biopsy ◽  
Poor Prognosis ◽  
Lung Diseases ◽  
Multidisciplinary Approach ◽  
Usual Interstitial Pneumonia ◽  
Interstitial Lung Diseases ◽  
High Resolution Computed Tomography ◽  
Antifibrotic Drugs

Idiopathic pulmonary fibrosis (IPF) is the commonest form of idiopathic interstitial pneumonias with very poor prognosis. Currently, diagnostic and treatment approaches to this disease have been revised. Confirmation of the diagnosis requires careful exclusion of other known causes of interstitial lung diseases and the presence of usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography (HRTC) and/or on lung biopsy. Also, multidisciplinary discussion involving experts with experience in the diagnosis of interstitial lung diseases is recommended. Given recent knowledge on pathogenesis of IPF antifibrotic drugs are recommended for the therapy of this disease. A clinical case that demonstrates the multidisciplinary approach to diagnosis of IPF is reported in this article.

scholarly journals Antifibrotic Therapies and Progressive Fibrosing Interstitial Lung Disease (PF-ILD): Building on INBUILD

2021 ◽  
Vol 10 (11) ◽  
pp. 2285
Author(s):  
John N. Shumar ◽  
Abhimanyu Chandel ◽  
Christopher S. King
Keyword(s):  
Interstitial Lung Disease ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Lung Diseases ◽  
Interstitial Lung Diseases ◽  
Treatment Pathway ◽  
Antifibrotic Therapy ◽  
New Treatment ◽  
Fibrotic Lung Diseases

Progressive fibrosing interstitial lung disease (PF-ILD) describes a phenotypic subset of interstitial lung diseases characterized by progressive, intractable lung fibrosis. PF-ILD is separate from, but has radiographic, histopathologic, and clinical similarities to idiopathic pulmonary fibrosis. Two antifibrotic medications, nintedanib and pirfenidone, have been approved for use in patients with idiopathic pulmonary fibrosis. Recently completed randomized controlled trials have demonstrated the clinical efficacy of antifibrotic therapy in patients with PF-ILD. The validation of efficacy of antifibrotic therapy in PF-ILD has changed the treatment landscape for all of the fibrotic lung diseases, providing a new treatment pathway and opening the door for combined antifibrotic and immunosuppressant drug therapy to address both the fibrotic and inflammatory components of ILD characterized by mixed pathophysiologic pathways.

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