Clinical and radiological features of lung disorders related to connective-tissue diseases: a pictorial essay
Abstract Connective tissue diseases (CTDs) include a spectrum of disorders that affect the connective tissue of the human body; they include autoimmune disorders characterized by immune-mediated chronic inflammation and the development of fibrosis. Lung involvement can be misdiagnosed, since pulmona...
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Format: | Article |
Language: | English |
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SpringerOpen
2022-06-01
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Series: | Insights into Imaging |
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Online Access: | https://doi.org/10.1186/s13244-022-01243-2 |
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author | Stefano Palmucci Federica Galioto Giulia Fazio Agata Ferlito Giovanna Cancemi Alessia Di Mari Gianluca Sambataro Domenico Sambataro Giovanni Zanframundo Letizia Antonella Mauro Pietro Valerio Foti Carlo Vancheri Antonio Basile |
author_facet | Stefano Palmucci Federica Galioto Giulia Fazio Agata Ferlito Giovanna Cancemi Alessia Di Mari Gianluca Sambataro Domenico Sambataro Giovanni Zanframundo Letizia Antonella Mauro Pietro Valerio Foti Carlo Vancheri Antonio Basile |
author_sort | Stefano Palmucci |
collection | DOAJ |
description | Abstract Connective tissue diseases (CTDs) include a spectrum of disorders that affect the connective tissue of the human body; they include autoimmune disorders characterized by immune-mediated chronic inflammation and the development of fibrosis. Lung involvement can be misdiagnosed, since pulmonary alterations preceded osteo-articular manifestations only in 20% of cases and they have no clear clinical findings in the early phases. All pulmonary structures may be interested: pulmonary interstitium, airways, pleura and respiratory muscles. Among these autoimmune disorders, rheumatoid arthritis (RA) is characterized by usual interstitial pneumonia (UIP), pulmonary nodules and airway disease with air-trapping, whereas non-specific interstitial pneumonia (NSIP), pulmonary hypertension and esophageal dilatation are frequently revealed in systemic sclerosis (SSc). NSIP and organizing pneumonia (OP) may be found in patients having polymyositis (PM) and dermatomyositis (DM); in some cases, perilobular consolidations and reverse halo-sign areas may be observed. Systemic lupus erythematosus (SLE) is characterized by serositis, acute lupus pneumonitis and alveolar hemorrhage. In the Sjögren syndrome (SS), the most frequent pattern encountered on HRCT images is represented by NSIP; UIP and lymphocytic interstitial pneumonia (LIP) are reported with a lower frequency. Finally, fibrotic NSIP may be the interstitial disease observed in patients having mixed connective tissue diseases (MCTD). This pictorial review therefore aims to provide clinical features and imaging findings associated with autoimmune CTDs, in order to help radiologists, pneumologists and rheumatologists in their diagnoses and management. |
first_indexed | 2024-04-13T15:31:52Z |
format | Article |
id | doaj.art-2dc310478b1340bbb4aa4fda33ed6c65 |
institution | Directory Open Access Journal |
issn | 1869-4101 |
language | English |
last_indexed | 2024-04-13T15:31:52Z |
publishDate | 2022-06-01 |
publisher | SpringerOpen |
record_format | Article |
series | Insights into Imaging |
spelling | doaj.art-2dc310478b1340bbb4aa4fda33ed6c652022-12-22T02:41:22ZengSpringerOpenInsights into Imaging1869-41012022-06-0113112610.1186/s13244-022-01243-2Clinical and radiological features of lung disorders related to connective-tissue diseases: a pictorial essayStefano Palmucci0Federica Galioto1Giulia Fazio2Agata Ferlito3Giovanna Cancemi4Alessia Di Mari5Gianluca Sambataro6Domenico Sambataro7Giovanni Zanframundo8Letizia Antonella Mauro9Pietro Valerio Foti10Carlo Vancheri11Antonio Basile12Radiology Unit 1, Department of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, University Hospital Policlinico “G. Rodolico-San Marco”Radiology Unit 1, Department of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, University Hospital Policlinico “G. Rodolico-San Marco”Radiology Unit 1, Department of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, University Hospital Policlinico “G. Rodolico-San Marco”Radiology Unit 1, Department of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, University Hospital Policlinico “G. Rodolico-San Marco”Radiology Unit 1, Department of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, University Hospital Policlinico “G. Rodolico-San Marco”Radiology Unit 1, Department of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, University Hospital Policlinico “G. Rodolico-San Marco”Department of Clinical and Experimental Medicine, Regional Referral Centre for Rare Lung Diseases, A. O. U. Policlinico “G. Rodolico - San Marco”, University of CataniaArtroreuma S.R.L, Rheumatology Outpatient Clinic Associated with the National Health SystemRheumatology Division, Department of Internal Medicine and Therapeutics, University of Pavia and IRCCS Policlinico S. Matteo FoundationRadiology Unit 1, University Hospital Policlinico “G. Rodolico-San Marco”Radiology Unit 1, Department of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, University Hospital Policlinico “G. Rodolico-San Marco”Department of Clinical and Experimental Medicine, Regional Referral Centre for Rare Lung Diseases, A. O. U. Policlinico “G. Rodolico - San Marco”, University of CataniaRadiology Unit 1, Department of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, University Hospital Policlinico “G. Rodolico-San Marco”Abstract Connective tissue diseases (CTDs) include a spectrum of disorders that affect the connective tissue of the human body; they include autoimmune disorders characterized by immune-mediated chronic inflammation and the development of fibrosis. Lung involvement can be misdiagnosed, since pulmonary alterations preceded osteo-articular manifestations only in 20% of cases and they have no clear clinical findings in the early phases. All pulmonary structures may be interested: pulmonary interstitium, airways, pleura and respiratory muscles. Among these autoimmune disorders, rheumatoid arthritis (RA) is characterized by usual interstitial pneumonia (UIP), pulmonary nodules and airway disease with air-trapping, whereas non-specific interstitial pneumonia (NSIP), pulmonary hypertension and esophageal dilatation are frequently revealed in systemic sclerosis (SSc). NSIP and organizing pneumonia (OP) may be found in patients having polymyositis (PM) and dermatomyositis (DM); in some cases, perilobular consolidations and reverse halo-sign areas may be observed. Systemic lupus erythematosus (SLE) is characterized by serositis, acute lupus pneumonitis and alveolar hemorrhage. In the Sjögren syndrome (SS), the most frequent pattern encountered on HRCT images is represented by NSIP; UIP and lymphocytic interstitial pneumonia (LIP) are reported with a lower frequency. Finally, fibrotic NSIP may be the interstitial disease observed in patients having mixed connective tissue diseases (MCTD). This pictorial review therefore aims to provide clinical features and imaging findings associated with autoimmune CTDs, in order to help radiologists, pneumologists and rheumatologists in their diagnoses and management.https://doi.org/10.1186/s13244-022-01243-2Connective tissue diseaseMultidetector computed tomographyLung disease (Interstitial)Autoimmune diseasesPulmonary fibrosis |
spellingShingle | Stefano Palmucci Federica Galioto Giulia Fazio Agata Ferlito Giovanna Cancemi Alessia Di Mari Gianluca Sambataro Domenico Sambataro Giovanni Zanframundo Letizia Antonella Mauro Pietro Valerio Foti Carlo Vancheri Antonio Basile Clinical and radiological features of lung disorders related to connective-tissue diseases: a pictorial essay Insights into Imaging Connective tissue disease Multidetector computed tomography Lung disease (Interstitial) Autoimmune diseases Pulmonary fibrosis |
title | Clinical and radiological features of lung disorders related to connective-tissue diseases: a pictorial essay |
title_full | Clinical and radiological features of lung disorders related to connective-tissue diseases: a pictorial essay |
title_fullStr | Clinical and radiological features of lung disorders related to connective-tissue diseases: a pictorial essay |
title_full_unstemmed | Clinical and radiological features of lung disorders related to connective-tissue diseases: a pictorial essay |
title_short | Clinical and radiological features of lung disorders related to connective-tissue diseases: a pictorial essay |
title_sort | clinical and radiological features of lung disorders related to connective tissue diseases a pictorial essay |
topic | Connective tissue disease Multidetector computed tomography Lung disease (Interstitial) Autoimmune diseases Pulmonary fibrosis |
url | https://doi.org/10.1186/s13244-022-01243-2 |
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