Contribution of pulmonary function tests (PFTs) to the diagnosis and follow up of connective tissue diseases
Abstract Introduction Connective Tissue Diseases (CTDs) are systemic autoimmune conditions characterized by frequent lung involvement. This usually takes the form of Interstitial Lung Disease (ILD), but Obstructive Lung Disease (OLD) and Pulmonary Artery Hypertension (PAH) can also occur. Lung invol...
Main Authors: | , , , , , , , , |
---|---|
Format: | Article |
Language: | English |
Published: |
PAGEPress Publications
2019-05-01
|
Series: | Multidisciplinary Respiratory Medicine |
Subjects: | |
Online Access: | http://link.springer.com/article/10.1186/s40248-019-0179-2 |
id |
doaj-3691773754c44cb7b273eb2c12ebe2f3 |
---|---|
record_format |
Article |
spelling |
doaj-3691773754c44cb7b273eb2c12ebe2f32020-11-25T03:03:37ZengPAGEPress PublicationsMultidisciplinary Respiratory Medicine2049-69582019-05-0114111110.1186/s40248-019-0179-2Contribution of pulmonary function tests (PFTs) to the diagnosis and follow up of connective tissue diseasesNicola Ciancio0Mauro Pavone1Sebastiano Emanuele Torrisi2Ada Vancheri3Domenico Sambataro4Stefano Palmucci5Carlo Vancheri6Fabiano Di Marco7Gianluca Sambataro8Regional Referral Center for Rare Lung Diseases, A. O. U. “Policlinico-Vittorio Emanuele” Department of Clinical and Experimental Medicine, University of CataniaRegional Referral Center for Rare Lung Diseases, A. O. U. “Policlinico-Vittorio Emanuele” Department of Clinical and Experimental Medicine, University of CataniaRegional Referral Center for Rare Lung Diseases, A. O. U. “Policlinico-Vittorio Emanuele” Department of Clinical and Experimental Medicine, University of CataniaRegional Referral Center for Rare Lung Diseases, A. O. U. “Policlinico-Vittorio Emanuele” Department of Clinical and Experimental Medicine, University of CataniaArtroreuma S.R.L. Outpatient Clinic accredited with the Italian National Health SystemDepartment of Medical Surgical Sciences and Advanced Technologies- Radiology I Unit, University Hospital “Policlinico-Vittorio Emanuele”Regional Referral Center for Rare Lung Diseases, A. O. U. “Policlinico-Vittorio Emanuele” Department of Clinical and Experimental Medicine, University of CataniaDepartment of Health Sciences, Università degli studi di Milano, Head Respiratory Unit, Papa Giovanni XXIII HospitalRegional Referral Center for Rare Lung Diseases, A. O. U. “Policlinico-Vittorio Emanuele” Department of Clinical and Experimental Medicine, University of CataniaAbstract Introduction Connective Tissue Diseases (CTDs) are systemic autoimmune conditions characterized by frequent lung involvement. This usually takes the form of Interstitial Lung Disease (ILD), but Obstructive Lung Disease (OLD) and Pulmonary Artery Hypertension (PAH) can also occur. Lung involvement is often severe, representing the first cause of death in CTD. The aim of this study is to highlight the role of Pulmonary Function Tests (PFTs) in the diagnosis and follow up of CTD patients. Main body Rheumatoid Arthritis (RA) showed mainly an ILD with a Usual Interstitial Pneumonia (UIP) pattern in High-Resolution Chest Tomography (HRCT). PFTs are able to highlight a RA-ILD before its clinical onset and to drive follow up of patients with Forced Vital Capacity (FVC) and Carbon Monoxide Diffusing Capacity (DLCO). In the course of Scleroderma Spectrum Disorders (SSDs) and Idiopathic Inflammatory Myopathies (IIMs), DLCO appears to be more sensitive than FVC in highlighting an ILD, but it can be compromised by the presence of PAH. A restrictive respiratory pattern can be present in IIMs and Systemic Lupus Erythematosus due to the inflammatory involvement of respiratory muscles, the presence of fatigue or diaphragm distress. Conclusions The lung should be carefully studied during CTDs. PFTs can represent an important prognostic tool for diagnosis and follow up of RA-ILD, but, on their own, lack sufficient specificity or sensitivity to describe lung involvement in SSDs and IIMs. Several composite indexes potentially able to describe the evolution of lung damage and response to treatment in SSDs are under investigation. Considering the potential severity of these conditions, an HRCT jointly with PFTs should be performed in all new diagnoses of SSDs and IIMs. Moreover, follow up PFTs should be interpreted in the light of the risk factor for respiratory disease related to each disease.http://link.springer.com/article/10.1186/s40248-019-0179-2Interstitial lung diseaseRheumatoid arthritisConnective tissue diseaseAntisynthetase SyndromeSystemic sclerosisSjӧgren Syndrome |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Nicola Ciancio Mauro Pavone Sebastiano Emanuele Torrisi Ada Vancheri Domenico Sambataro Stefano Palmucci Carlo Vancheri Fabiano Di Marco Gianluca Sambataro |
spellingShingle |
Nicola Ciancio Mauro Pavone Sebastiano Emanuele Torrisi Ada Vancheri Domenico Sambataro Stefano Palmucci Carlo Vancheri Fabiano Di Marco Gianluca Sambataro Contribution of pulmonary function tests (PFTs) to the diagnosis and follow up of connective tissue diseases Multidisciplinary Respiratory Medicine Interstitial lung disease Rheumatoid arthritis Connective tissue disease Antisynthetase Syndrome Systemic sclerosis Sjӧgren Syndrome |
author_facet |
Nicola Ciancio Mauro Pavone Sebastiano Emanuele Torrisi Ada Vancheri Domenico Sambataro Stefano Palmucci Carlo Vancheri Fabiano Di Marco Gianluca Sambataro |
author_sort |
Nicola Ciancio |
title |
Contribution of pulmonary function tests (PFTs) to the diagnosis and follow up of connective tissue diseases |
title_short |
Contribution of pulmonary function tests (PFTs) to the diagnosis and follow up of connective tissue diseases |
title_full |
Contribution of pulmonary function tests (PFTs) to the diagnosis and follow up of connective tissue diseases |
title_fullStr |
Contribution of pulmonary function tests (PFTs) to the diagnosis and follow up of connective tissue diseases |
title_full_unstemmed |
Contribution of pulmonary function tests (PFTs) to the diagnosis and follow up of connective tissue diseases |
title_sort |
contribution of pulmonary function tests (pfts) to the diagnosis and follow up of connective tissue diseases |
publisher |
PAGEPress Publications |
series |
Multidisciplinary Respiratory Medicine |
issn |
2049-6958 |
publishDate |
2019-05-01 |
description |
Abstract Introduction Connective Tissue Diseases (CTDs) are systemic autoimmune conditions characterized by frequent lung involvement. This usually takes the form of Interstitial Lung Disease (ILD), but Obstructive Lung Disease (OLD) and Pulmonary Artery Hypertension (PAH) can also occur. Lung involvement is often severe, representing the first cause of death in CTD. The aim of this study is to highlight the role of Pulmonary Function Tests (PFTs) in the diagnosis and follow up of CTD patients. Main body Rheumatoid Arthritis (RA) showed mainly an ILD with a Usual Interstitial Pneumonia (UIP) pattern in High-Resolution Chest Tomography (HRCT). PFTs are able to highlight a RA-ILD before its clinical onset and to drive follow up of patients with Forced Vital Capacity (FVC) and Carbon Monoxide Diffusing Capacity (DLCO). In the course of Scleroderma Spectrum Disorders (SSDs) and Idiopathic Inflammatory Myopathies (IIMs), DLCO appears to be more sensitive than FVC in highlighting an ILD, but it can be compromised by the presence of PAH. A restrictive respiratory pattern can be present in IIMs and Systemic Lupus Erythematosus due to the inflammatory involvement of respiratory muscles, the presence of fatigue or diaphragm distress. Conclusions The lung should be carefully studied during CTDs. PFTs can represent an important prognostic tool for diagnosis and follow up of RA-ILD, but, on their own, lack sufficient specificity or sensitivity to describe lung involvement in SSDs and IIMs. Several composite indexes potentially able to describe the evolution of lung damage and response to treatment in SSDs are under investigation. Considering the potential severity of these conditions, an HRCT jointly with PFTs should be performed in all new diagnoses of SSDs and IIMs. Moreover, follow up PFTs should be interpreted in the light of the risk factor for respiratory disease related to each disease. |
topic |
Interstitial lung disease Rheumatoid arthritis Connective tissue disease Antisynthetase Syndrome Systemic sclerosis Sjӧgren Syndrome |
url |
http://link.springer.com/article/10.1186/s40248-019-0179-2 |
work_keys_str_mv |
AT nicolaciancio contributionofpulmonaryfunctiontestspftstothediagnosisandfollowupofconnectivetissuediseases AT mauropavone contributionofpulmonaryfunctiontestspftstothediagnosisandfollowupofconnectivetissuediseases AT sebastianoemanueletorrisi contributionofpulmonaryfunctiontestspftstothediagnosisandfollowupofconnectivetissuediseases AT adavancheri contributionofpulmonaryfunctiontestspftstothediagnosisandfollowupofconnectivetissuediseases AT domenicosambataro contributionofpulmonaryfunctiontestspftstothediagnosisandfollowupofconnectivetissuediseases AT stefanopalmucci contributionofpulmonaryfunctiontestspftstothediagnosisandfollowupofconnectivetissuediseases AT carlovancheri contributionofpulmonaryfunctiontestspftstothediagnosisandfollowupofconnectivetissuediseases AT fabianodimarco contributionofpulmonaryfunctiontestspftstothediagnosisandfollowupofconnectivetissuediseases AT gianlucasambataro contributionofpulmonaryfunctiontestspftstothediagnosisandfollowupofconnectivetissuediseases |
_version_ |
1724684803163291648 |