GENETIC DISORDERS OF SURFACTANT PROTEINS

2021 ◽  
Vol 100 (5) ◽  
pp. 82-89
Author(s):  
M.A. Zhestkova ◽  
◽  
D.Yu. Ovsyannikov ◽  
Keyword(s):  
Differential Diagnosis ◽  
Interstitial Pneumonia ◽  
Lung Diseases ◽  
Neonatal Period ◽  
Pulmonary Alveolar Proteinosis ◽  
Genetic Disorders ◽  
Interstitial Lung Diseases ◽  
Surfactant Proteins ◽  
Nonspecific Interstitial Pneumonia ◽  
Alveolar Proteinosis

The literature review provides up-to-date information on rare interstitial lung diseases, manifesting both in children, starting from the neonatal period, and in adults, – genetic disorders of surfactant proteins B, C, ATP-binding cassette protein A3 (ABCA3), manifested by such histopathological patterns, as chronic pneumonitis of infants, pulmonary alveolar proteinosis, desquamative interstitial pneumonia , nonspecific interstitial pneumonia. Information on epidemiology, genetics, pathogenesis, clinical picture, diagnosis and differential diagnosis, treatment of these diseases is given.

scholarly journals Pharmacological management of progressive-fibrosing interstitial lung diseases: a review of the current evidence

2018 ◽  
Vol 27 (150) ◽  
pp. 180074 ◽  
Author(s):  
Luca Richeldi ◽  
Francesco Varone ◽  
Miguel Bergna ◽  
Joao de Andrade ◽  
Jeremy Falk ◽  
...  
Keyword(s):  
Interstitial Pneumonia ◽  
Lung Diseases ◽  
Occupational Exposures ◽  
Interstitial Lung Diseases ◽  
Therapeutic Options ◽  
Current Evidence ◽  
Pharmacological Management ◽  
The Status ◽  
Nonspecific Interstitial Pneumonia ◽  
Randomised Controlled

A proportion of patients with interstitial lung diseases (ILDs) are at risk of developing a progressive-fibrosing phenotype, which is associated with a deterioration in lung function and early mortality. In addition to idiopathic pulmonary fibrosis (IPF), fibrosing ILDs that may present a progressive phenotype include idiopathic nonspecific interstitial pneumonia, connective tissue disease-associated ILDs, hypersensitivity pneumonitis, unclassifiable idiopathic interstitial pneumonia, ILDs related to other occupational exposures and sarcoidosis. Corticosteroids and/or immunosuppressive therapies are sometimes prescribed to patients with these diseases. However, this treatment regimen may not be effective, adequate on its own or well tolerated, suggesting that there is a pressing need for efficacious and better tolerated therapies. Currently, the only approved treatments to slow disease progression in patients with IPF are nintedanib and pirfenidone. Similarities in pathobiological mechanisms leading to fibrosis between IPF and other ILDs that may present a progressive-fibrosing phenotype provide a rationale to suggest that nintedanib and pirfenidone may be therapeutic options for patients with the latter diseases.This review provides an overview of the therapeutic options currently available for patients with fibrosing ILDs, including fibrosing ILDs that may present a progressive phenotype, and explores the status of the randomised controlled trials that are underway to determine the efficacy and safety of nintedanib and pirfenidone.

scholarly journals Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases

2018 ◽  
Vol 27 (150) ◽  
pp. 180076 ◽  
Author(s):  
Vincent Cottin ◽  
Nikhil A. Hirani ◽  
David L. Hotchkin ◽  
Anoop M. Nambiar ◽  
Takashi Ogura ◽  
...  
Keyword(s):  
Interstitial Pneumonia ◽  
Lung Diseases ◽  
Hypersensitivity Pneumonitis ◽  
Early Death ◽  
Occupational Exposures ◽  
Interstitial Lung Diseases ◽  
Lung Function Decline ◽  
Nonspecific Interstitial Pneumonia ◽  
Immunomodulatory Therapies

Although these conditions are rare, a proportion of patients with interstitial lung diseases (ILDs) may develop a progressive-fibrosing phenotype. Progressive fibrosis is associated with worsening respiratory symptoms, lung function decline, limited response to immunomodulatory therapies, decreased quality of life and, potentially, early death. Idiopathic pulmonary fibrosis may be regarded as a model for other progressive-fibrosing ILDs. Here we focus on other ILDs that may present a progressive-fibrosing phenotype, namely idiopathic nonspecific interstitial pneumonia, unclassifiable idiopathic interstitial pneumonia, connective tissue disease-associated ILDs (e.g.rheumatoid arthritis-related ILD), fibrotic chronic hypersensitivity pneumonitis, fibrotic chronic sarcoidosis and ILDs related to other occupational exposures. Differential diagnosis of these ILDs can be challenging, and requires detailed consideration of clinical, radiological and histopathological features. Accurate and early diagnosis is crucial to ensure that patients are treated optimally.

DIFFERENTIAL DIAGNOSTICS OF NON-SPECIFIC INTERSTITIAL PNEUMONIA

2020 ◽  
Vol 2 (1) ◽  
pp. 50-52
Author(s):  
Nargiza Makhmatmuradova ◽  
◽  
Olga Ibadova ◽  
Parvina Zikriyaeva
Keyword(s):  
Differential Diagnosis ◽  
Interstitial Pneumonia ◽  
Lung Diseases ◽  
Clinical Presentation ◽  
Differential Diagnostics ◽  
Hospital Treatment ◽  
Case Histories ◽  
Allergic Alveolitis ◽  
Nonspecific Interstitial Pneumonia ◽  
Exogenous Allergic Alveolitis

A retrospective analysis of case histories of 82 patients with nonspecific interstitial pneumonia, 24 patients with idiopathic pulmonary fibrosis, 8 with exogenous allergic alveolitis, 12 with systemic scleroderma and 6 with drug pneumonia was carried out in hospital treatment in the pulmonology department of the Samarkand city medical hospital in 2010-2019 years. It has been established that in the differential diagnosis of nonspecific interstitial pneumonia and other lung diseases, particular attention should be paid to the anamnesis, clinical presentation and CT signs

scholarly journals Radiological diagnosis of interstitial lung diseases

2013 ◽  
Vol 66 (suppl. 1) ◽  
pp. 29-33
Author(s):  
Ruza Stevic ◽  
Vucinic Mihailovic ◽  
Dragana Jovanovic ◽  
Nada Vasic
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Lung Diseases ◽  
Usual Interstitial Pneumonia ◽  
Interstitial Lung Diseases ◽  
Ground Glass ◽  
Cryptogenic Organizing Pneumonia ◽  
Acute Interstitial Pneumonia ◽  
Nonspecific Interstitial Pneumonia

Introduction. Interstitial lung diseases include the entities of idiopathic pulmonary fibrosis, nonspecific interstitial pneumonia, cryptogenic organizing pneumonia, acute interstitial pneumonia, respiratory bronchiolitis-associated interstitial lung disease, desquamative interstitial pneumonia, and lymphocytic interstitial pneumonia. Recent introduction of high-resolution computed tomography has made the diagnosis of interstitial lung disease much easier. Usual interstitial pneumonia A predominantly subpleural reticulation and honeycombing at the basal posterior part of the lung with a progression to anterior and superior parts are characteristic of usual interstitial pneumonia/ idiopathic pulmonary fibrosis. Nonspecific interstitial pneumonia Typical findings of nonspecific interstitial pneumonia are bilateral, relatively symmetrical subpleural ground glass opacifications and irregular linear opacities. Desquamative interstitial pneumonia is characterized by diffuse symmetrical ground glass opacifications. Respiratory bronchiolitisassociated interstitial lung disease Centrilobular nodules and irregular ground glass opacifications are present. Cryptogenic organizing pneumonia Subpleural and peribronchial consolidations are prominent findings that are not present in other idiopathic interstitial pneumonias. Acute interstitial pneumonia. Bilateral ground-glass opacifications are present and areas of peripheral consolidations may also be seen in acute interstitial pneumonia. Lymphocytic interstitial pneumonia. Diffuse or patchy areas of ground glass opacification with centrilobular nodules and occasionally well-defined cysts are seen. Conclusion. Imaging plays a crucial role in identifying interstitial lung diseases but precise diagnosis requires a dynamic interdisciplinary approach that correlates clinical, radiological and pathologic features.

scholarly journals Treatment of chronic fibrosing interstitial lung diseases

2021 ◽  
Vol 64 (4) ◽  
pp. 277-285
Author(s):  
Jongmin Lee
Keyword(s):  
Pulmonary Fibrosis ◽  
Interstitial Pneumonia ◽  
Lung Diseases ◽  
Early Death ◽  
Interstitial Lung Diseases ◽  
Appropriate Treatment ◽  
Heterogenous Group ◽  
Nonspecific Interstitial Pneumonia ◽  
Prediction Of Prognosis

Interstitial lung diseases (ILD) refers to a large and heterogenous group of parenchymal lung disorders. It is difficult to diagnose and classify ILD. Nevertheless, accurate diagnosis of ILD is crucial for appropriate treatment selection and prediction of prognosis. Idiopathic pulmonary fibrosis (IPF), the most severe of the chronic forms of ILD, is defined as a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown cause. In addition to IPF, a subset of patients with ILD may develop progressive fibrotic changes in lungs. As pulmonary fibrosis progresses, lung function gradually deteriorates and respiratory symptoms worsen; besides, quality of life is also impaired. Progressive fibrosis is also associated with limited response to immunomodulatory thrapies and, potentially, early death. A progressive fibrosing phenotype of ILD (PF-ILD), a subtype of ILD, shows morphological similarities, common underlying pathophysiologic mechanisms, and consistently progressive worsening. PF-ILD include idiopathic nonspecific interstitial pneumonia, unclassifiable idiopathic interstitial pneumonia, autoimmune ILD, chronic sarcoidosis, chronic hypersensitivity pneumonitis and environmental lung diseases. Antifibrotic agents pirfenidone and nintedanib have showed positive results not only for IPF but also for PF-ILD. Immunosuppressive therapy can be used for some types of PF-ILD. If a patient with PF-ILD does not respond to conventional treatment, lung transplantation may be a treatment option. Clinical trials on the treatment of PF-ILD are actively underway. Therefore, over the course of the next several years, major advances in PF-ILD treatment can be expected.

CURRENT FEATURES OF NON-SPECIFIC INTERSTITIAL PNEUMONIA

2020 ◽  
Vol 93 (1) ◽  
pp. 11-13
Author(s):  
Nematillo Aralov ◽  
Nargiza Makhmatmuradova ◽  
Parvina Zakiryaeva
Keyword(s):  
Medical Association ◽  
Retrospective Analysis ◽  
Interstitial Pneumonia ◽  
Lung Diseases ◽  
Clinical Characteristics ◽  
Interstitial Lung Diseases ◽  
Frequency Of Occurrence ◽  
Case Histories ◽  
Working Age ◽  
Nonspecific Interstitial Pneumonia

In order to study the frequency of occurrence, clinical characteristics, and diagnosis of interstitial pneumonia in the pulmonary department of hospitals, a retrospective analysis of case histories of 22 patients with nonspecific interstitial pneumonia who were hospitalized in the pulmonology department of the Samarkand city medical association in 2018-2019 was performed. An increase was found in patients with interstitial lung diseases, which are observed more often in women of working age, with prevalence of dyspnea, weakness, cough in the clinic, and the importance of the use of imaging technologies is indicated. Consultation of a pulmonologist is recommended for patients with rheumatic diseases.

scholarly journals Multidisciplinary approach in the diagnosis of idiopathic nonspecific interstitial pneumonia

2019 ◽  
Vol 91 (3) ◽  
pp. 101-106
Author(s):  
S I Ovcharenko ◽  
E A Son ◽  
V A Kapustina
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Interstitial Pneumonia ◽  
Lung Biopsy ◽  
Lung Diseases ◽  
Multidisciplinary Approach ◽  
Interstitial Lung Diseases ◽  
American Thoracic Society ◽  
Modern Classification ◽  
Nonspecific Interstitial Pneumonia

The article provides a modern classification of interstitial lung diseases. The focus is on the poorly studied and difficult to diagnose idiopathic nonspecific interstitial pneumonia, isolated in a separate nosological form and included in the classification only in 2002. The paper presents the features of the clinical, radiological, histological picture of idiopathic nonspecific interstitial pneumonia in comparison with idiopathic pulmonary fibrosis. The presented materials are based on the results of the largest study conducted by the working group of the American Thoracic Society, which shows the importance of the interaction of specialists in the diagnosis of idiopathic nonspecific interstitial pneumonia. A comprehensive assessment of the examination of patients conducted in collaboration with the clinician and radiologist, after their cooperative discussion, in many cases can help to avoid lung biopsy, and the study of morphological material is more often necessary only in difficult diagnostic situations.

scholarly journals Blood Testing for Differential Diagnosis of Pulmonary Alveolar Proteinosis Syndrome

CHEST Journal ◽  
2019 ◽  
Vol 155 (2) ◽  
pp. 450-452 ◽  
Author(s):  
Cormac McCarthy ◽  
Brenna Carey ◽  
Bruce C. Trapnell
Keyword(s):  
Differential Diagnosis ◽  
Pulmonary Alveolar Proteinosis ◽  
Blood Testing ◽  
Alveolar Proteinosis
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