Lung magnetic resonance imaging in systemic sclerosis: a new promising approach to evaluate pulmonary involvement and progression.

Published on May 1, 2021in Clinical Rheumatology2.98
· DOI :10.1007/S10067-020-05491-9
Luna Gargani45
Estimated H-index: 45
(National Research Council),
Cosimo Bruni18
Estimated H-index: 18
(UniFI: University of Florence)
+ 11 AuthorsMarco Matucci-Cerinic97
Estimated H-index: 97
(UniFI: University of Florence)
Interstitial lung disease (ILD) is frequent and highly disabling in systemic sclerosis (SSc). Magnetic resonance imaging (MRI) is not routinely used to evaluate the lung, due to poorer spatial resolution compared to high-resolution computed tomography (HRCT). We aimed to compare lung MRI signal with HRCT and evaluate the role of MRI in predicting ILD progression. Thirty SSc patients underwent lung MRI and HRCT. STIR and T1 mapping sequences were acquired before and after gadolinium injection. Patients were classified as normal (group 1 with normal HRCT and MRI), discordant (group 2 without ILD signs on HRCT but areas of hyperintensity on MRI), and abnormal (group 3 with ILD signs on HRCT and areas of hyperintensity on MRI). Patients were followed up for ILD progression. Mean STIR and T1 values were different between the three groups (p  90 ms discriminated patients at a higher risk of worsening pulmonary involvement (HR 8.80; CI 1.81–42.74; p < 0.007). Lung MRI can detect SSc-related ILD, with good correlations with other ILD markers. STIR values, independently of HRCT appearance, may predict worsening lung involvement. Lung MRI, although very preliminary, is a promising tool that in a near future could help selecting patients for an early treatment of SSc-related ILD and a more appropriate use of HRCT.
#1Elizabeth R. Volkmann (UCLA: University of California, Los Angeles)H-Index: 24
#2Aryeh Fischer (University of Colorado Denver)H-Index: 43
Contemporary studies of systemic sclerosis consistently demonstrate that interstitial lung disease is a leading cause of disease-related death. This review summarizes morbidity and mortality outcom...
#1Barbara RuaroH-Index: 20
Last. Cosimo Bruni (UniFI: University of Florence)H-Index: 18
view all 7 authors...
Systemic sclerosis (SSc) is a connective tissue disease, characterized by vascular damage and progressive fibrosis, affecting the skin and internal organs. The vascular changes include functional and structural abnormalities in the microcirculation, which play a central role not only in diagnosis, but also in the prognosis and follow-up of systemic sclerosis patients. Nailfold videocapillaroscopy (NVC) is a safe, validated, non-invasive, inexpensive, reliable and reproducible method that allows ...
#1Luna Gargani (National Research Council)H-Index: 45
#2Cosimo Bruni (UniFI: University of Florence)H-Index: 18
Last. Marco Matucci-Cerinic (UniFI: University of Florence)H-Index: 97
view all 20 authors...
Abstract Background A high percentage of systemic sclerosis (SSc) patients develop interstitial lung disease (ILD) during the disease course. Recent data have shown that lung ultrasound (LUS) can assess ILD by the evaluation of B-lines, the sonographic sign of pulmonary interstitial involvement. Our aim was to establish the prognostic value of B-lines in a large number of SSc patients. Methods A total of 396 consecutive SSc patients, enrolled at three Rheumatology Departments, underwent a compre...
#1Anna-Maria Hoffmann-Vold (University of Oslo)H-Index: 20
#2Yannick Allanore (University of Paris)H-Index: 86
Last. Michael Kreuter (Heidelberg University)H-Index: 40
view all 8 authors...
Systemic sclerosis (SSc) is a multi-organ autoimmune disease with complex interactions between immune-mediated inflammatory processes and vascular pathology leading to small vessel obliteration, promoting uncontrolled fibrosis of skin and internal organs. Interstitial lung disease (ILD) is a common but highly variable manifestation of SSc and is associated with high morbidity and mortality. Treatment approaches have focused on immunosuppressive therapies, which have shown some efficacy on lung f...
#1Mariaelena Occhipinti (UniFI: University of Florence)H-Index: 10
#2Cosimo Bruni (UniFI: University of Florence)H-Index: 18
Last. Marco Matucci-Cerinic (UniFI: University of Florence)H-Index: 97
view all 15 authors...
Objective To prospectively investigate whether differences in pulmonary vasculature exist in systemic sclerosis (SSc) and how they are distributed in patients with different pulmonary function. Methods Seventy-four patients with SSc undergoing chest CT scan for interstitial lung disease (ILD) screening or follow-up were prospectively enrolled. A thorough clinical, laboratory and functional evaluation was performed the same day. Chest CT was spirometry gated at total lung capacity and images were...
#1Kimberly Showalter (HSS: Hospital for Special Surgery)H-Index: 2
#2Peter A. Merkel (UPenn: University of Pennsylvania)H-Index: 95
Last. Jessica K. Gordon (HSS: Hospital for Special Surgery)H-Index: 21
view all 4 authors...
Background:The modified Rodnan skin score is a common primary outcome measurement tool in clinical trials of systemic sclerosis (scleroderma). However, it is unknown how often physicians perform th...
#1Svetlana I. Nihtyanova (UCL: University College London)H-Index: 21
#2Christopher P. Denton (UCL: University College London)H-Index: 109
Systemic sclerosis is an autoimmune disease leading to vasculopathy and fibrosis of skin and internal organs. Despite likely shared pathogenic mechanisms, the patterns of skin and lung fibrosis dif...
#1David Roofeh (UM: University of Michigan)H-Index: 11
#2Oliver Distler (UZH: University of Zurich)H-Index: 98
Last. Dinesh Khanna (UM: University of Michigan)H-Index: 90
view all 5 authors...
Systemic sclerosis–associated interstitial lung disease remains a leading cause of mortality. Despite decades of clinical trials, the treatment effects of disease modifying anti-rheumatic drugs con...
#1Elizabeth R. Volkmann (UCLA: University of California, Los Angeles)H-Index: 24
The natural history of interstitial lung disease in patients with systemic sclerosis is highly variable. Historical observational studies have demonstrated that the greatest decline in lung functio...
#1Anna-Maria Hoffmann-Vold (Oslo University Hospital)H-Index: 20
#2Toby M. Maher (NIH: National Institutes of Health)H-Index: 68
Last. Oliver Distler (UZH: University of Zurich)H-Index: 98
view all 32 authors...
Summary Background Systemic sclerosis-associated interstitial lung disease (ILD) carries a high mortality risk; expert guidance is required to aid early recognition and treatment. We aimed to develop the first expert consensus and define an algorithm for the identification and management of the condition through application of well established methods. Methods Evidence-based consensus statements for systemic sclerosis-associated ILD management were established for six domains (ie, risk factors, ...
Cited By2
#1Martina Orlandi (UniFI: University of Florence)H-Index: 11
#2Nicholas Landini (UniFI: University of Florence)H-Index: 3
Last. Stefano Colagrande (UniFI: University of Florence)H-Index: 26
view all 4 authors...
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