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 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).

scholarly journals Incidence and predictive factors of lung cancer in patients with idiopathic pulmonary fibrosis

2018 ◽  
Vol 4 (1) ◽  
pp. 00111-2016 ◽  
Author(s):  
Eisuke Kato ◽  
Noboru Takayanagi ◽  
Yotaro Takaku ◽  
Naho Kagiyama ◽  
Tetsu Kanauchi ◽  
...  
Keyword(s):  
Risk Factors ◽  
Lung Cancer ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Interstitial Pneumonia ◽  
Cox Regression ◽  
Usual Interstitial Pneumonia ◽  
Cancer Development ◽  
Smoking History ◽  
Incidence Density

The incidence and risk factors of lung cancer in patients with idiopathic pulmonary fibrosis (IPF) have been poorly investigated.We conducted a retrospective study of 632 patients with IPF to assess the incidence and risk factors of lung cancer development.Seventy patients developed lung cancer over a median follow-up period of 3.8 years. The incidence density of lung cancer development was 25.2 cases per 1000 person-years. The most frequent type was squamous cell carcinoma (30%), the majority developed lung cancer in the peripheral lung (82.9%) and adjacent to usual interstitial pneumonia (75.7%). In a multivariate Cox regression hazard model, pack-years of smoking ≥35 and coexisting emphysema were associated with lung cancer development. The 1-, 3- and 5-year all-cause mortality rates after lung cancer diagnosis were 53.5%, 78.6% and 92.9%, respectively.The incidence density of lung cancer is high in IPF patients and occurs more frequently in patients with smoking history of pack-years of smoking ≥35 and with coexisting emphysema. The majority of lung cancers develop adjacent to usual interstitial pneumonia. Knowledge of these factors may help direct efforts for early detection of lung cancer and disease management.

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 An increased risk of lung cancer in combined pulmonary fibrosis and emphysema patients with usual interstitial pneumonia compared with patients with idiopathic pulmonary fibrosis alone: a systematic review and meta-analysis

2021 ◽  
Vol 15 ◽  
pp. 175346662110170
Author(s):  
Qianqian Chen ◽  
Ping Liu ◽  
Hong Zhou ◽  
Hui Kong ◽  
Weiping Xie
Keyword(s):  
Lung Cancer ◽  
Systematic Review ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Interstitial Pneumonia ◽  
Fixed Effects ◽  
Meta Analysis ◽  
Usual Interstitial Pneumonia ◽  
Fixed Effects Model ◽  
Increased Risk

Background: Lung cancer is an important complication of combined pulmonary fibrosis and emphysema (CPFE). Whether the risk of lung cancer is higher in CPFE patients with usual interstitial pneumonia (UIP) than those with idiopathic pulmonary fibrosis (IPF) alone, remains controversial. We conducted this systematic review and meta-analysis to evaluate the prevalence of lung cancer in CPFE patients with UIP compared with IPF patients. Methods: We searched the PubMed, Embase, and Cochrane databases for studies that focused on the incidence of lung cancer in CPFE/UIP and IPF groups. We used a fixed-effects model to analyze the odds ratios (ORs) with 95% confidence intervals (CIs) according to data heterogeneity. The cumulative effects based on the publication year and sample size were assessed by cumulative meta-analysis. Results: A total of nine studies with 933 patients, including 374 CPFE patients with UIP, fulfilled the inclusion criteria. Overall, CPFE patients with UIP have a higher risk of lung cancer than those with IPF alone (OR = 2.69; 95% CI: 1.78–4.05). There were increased risks of lung cancer in CPFE/UIP patients with the presence of emphysema (OR = 2.93; 95% CI: 1.79–4.79) or emphysema in ⩾10% of the lung volume (OR = 2.22; 95% CI: 1.06–4.68). Conclusions: Our systematic review and meta-analysis indicated a significantly higher prevalence of lung cancer in CPFE patients with UIP than in patients with IPF alone. The reviews of this paper are available via the supplemental material section.

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.

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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