scholarly journals Clinical and morphological characteristics of hypersensitivity pneumonitis

2021 ◽  
Vol 10 (3) ◽  
pp. 5-13
Author(s):  
E.V. Kusraeva ◽  
Keyword(s):  
Interstitial Pneumonia ◽  
Hypersensitivity Pneumonitis ◽  
Usual Interstitial Pneumonia ◽  
Connective Tissue Diseases ◽  
Morphological Characteristics ◽  
Interstitial Lung Diseases ◽  
External Respiration ◽  
Morphological Criteria ◽  
Pulmonary Manifestations ◽  
Cellular Components

Hypersensitivity pneumonitis (HP) is an immune-mediated disease that manifests in susceptible individu-als after exposure to a provoking inhalation factor. The literature review describes etiological factors of the disease, its epidemiology and pathogenesis, as well as clinical features of various disease courses. A new clinical and morphological classification of HP is also presented. We analyzed changes in functional tests (external respiration function), CT images, cellular components of bronchoalveolar lavages, and modern approaches to the disease treatment. The review includes detailed morphological criteria according to the new classification, which will facilitate the diagnosis by pathologists. Since the diagnosis of the condition presents significant challenges, there are histological criteria for differential diagnosis of the fibrotic HP and usual interstitial pneumonia (idiopathic pulmonary fibrosis), sarcoidosis, lymphoid interstitial pneumonia, and pulmonary manifestations of connective tissue diseases. Keywords: hypersensitivity pneumonitis, 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.

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 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.

The Pulmonary Histopathology of Anti-KS Transfer RNA Synthetase Syndrome

2015 ◽  
Vol 139 (1) ◽  
pp. 122-125 ◽  
Author(s):  
Frank Schneider ◽  
Rohit Aggarwal ◽  
David Bi ◽  
Kevin Gibson ◽  
Chester Oddis ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Usual Interstitial Pneumonia ◽  
Connective Tissue Disorder ◽  
Transfer Rna ◽  
Organizing Pneumonia ◽  
Antisynthetase Syndrome ◽  
Pulmonary Manifestations

Context The clinical spectrum of the antisynthetase syndromes (AS) has been poorly defined, although some frequently present with pulmonary manifestations. The anti-KS anti–asparaginyl-transfer RNA synthetase syndrome is one in which pulmonary interstitial lung disease is almost always present and yet the histopathologic spectrum is not well described. Objective To define the morphologic manifestations of pulmonary disease in those patients with anti-KS antiasparaginyl syndrome. Design We reviewed the connective tissue disorder registry of the University of Pittsburgh and identified those patients with anti-KS autoantibodies who presented with interstitial lung disease and had surgical lung biopsies. Results The 5 patients with anti-KS antisynthetase syndrome were usually women presenting with dyspnea and without myositis, but with mechanic's hands (60%) and Raynaud phenomenon (40%). They most often presented with a usual interstitial pneumonia pattern of fibrosis (80%), with the final patient displaying organizing pneumonia. Conclusions Pulmonary interstitial lung disease is a common presentation in patients with the anti-KS–antisynthetase syndrome, who are often women with rather subtle or subclinical connective tissue disease, whereas the literature emphasizes the nonspecific interstitial pneumonia pattern often diagnosed clinically. Usual interstitial pneumonia and organizing pneumonia patterns of interstitial injury need to be added to this clinical differential diagnosis.

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