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).

scholarly journals Distribution of interstitial lung diseases in a tertiary care centre of South India

2021 ◽  
Vol 8 (3) ◽  
pp. 420
Author(s):  
Mahesh Babu Vemuri ◽  
Manju Rajaram ◽  
Madhusmita Mohanty Mohapatra ◽  
Mathavaswami Vijayageetha ◽  
V. S. Negi ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Lung Diseases ◽  
South India ◽  
Tertiary Care ◽  
Interstitial Lung Diseases ◽  
Care Centre ◽  
Tertiary Care Centre

Background: The term interstitial lung diseases (ILD) refer to a broad category of lung diseases rather than a specific disease entity. True prevalence of ILD is difficult to estimate and it may vary according to the geography, environment, occupation etc. Aim and objectives were to find out the distribution of interstitial lung disease (ILD) subtypes in a tertiary care centre.  Methods: A descriptive study was conducted between August 2016 and November 2018 in a tertiary care centre in Puducherry, South India. Baseline demographic details, clinical symptoms, signs, radiological findings (chest radiograph and HRCT), pathological findings, and physiological findings were taken into consideration and diagnosis of type of interstitial lung disease was made using multidisciplinary discussion. Statistical analysis was done using SPSS 19.0 version.Results: A total of 150 patients were recruited of which 70.5% were females. Most common ILD subtype in our study was connective tissue disease associated ILD – 97 patients (65%) followed by idiopathic pulmonary fibrosis (IPF)–31 patients (22%). Most common type of CTD ILD observed in our study was progressive systemic sclerosis (46%) followed by mixed connective tissue disease (24%). The most common HRCT finding was NSIP pattern and most commonly observed physiological abnormality was moderate restriction and moderate diffusion impairment.  Conclusions: Connective tissue disease-associated ILD was the most common ILD found in our study amongst south Indian population. This suggests that the distribution of ILD would vary depending on the geographical area and the environmental exposure which was in contrast with the Indian ILD registry.  

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).

Targeting CD20 in the treatment of interstitial lung diseases related to connective tissue diseases: A systematic review

2020 ◽  
Vol 19 (2) ◽  
pp. 102453 ◽  
Author(s):  
Mattia Bellan ◽  
Filippo Patrucco ◽  
Francesco Barone-Adesi ◽  
Francesco Gavelli ◽  
Luigi Mario Castello ◽  
...  
Keyword(s):  
Systematic Review ◽  
Connective Tissue ◽  
Lung Diseases ◽  
Connective Tissue Diseases ◽  
Interstitial Lung Diseases

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.

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