scholarly journals Nailfold Videocapillaroscopy Is a Useful Tool to Recognize Definite Forms of Systemic Sclerosis and Idiopathic Inflammatory Myositis in Interstitial Lung Disease Patients

Diagnostics ◽  
2020 ◽  
Vol 10 (5) ◽  
pp. 253 ◽  
Author(s):  
Domenico Sambataro ◽  
Gianluca Sambataro ◽  
Alessandro Libra ◽  
Giovanna Vignigni ◽  
Fabio Pino ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Correct Diagnosis ◽  
Diagnostic Assessment ◽  
Nailfold Videocapillaroscopy ◽  
High Resolution Computed Tomography ◽  
Inflammatory Myositis ◽  
Idiopathic Inflammatory Myositis ◽  
Diagnostic Role

Nailfold videocapillaroscopy (NVC) is an easy tool used for the assessment of patients with Raynaud’s phenomenon (RP) as possibly associated with systemic sclerosis (SSc). Recent insights have also highlighted its role in the diagnostic assessment of idiopathic inflammatory myopathies (IIMs). The aim of this study is to describe the diagnostic role of NVC in a series of 361 consecutive patients with interstitial lung disease (ILD). All the patients were assessed by clinical pulmonary and rheumatic examinations, blood exams, high-resolution computed tomography and NVC. NVC was considered positive only in the presence of avascular areas or giant capillaries, but also, the presence of bushy capillaries (BCs) was recorded. NVC was positive in 17.7% of ILD patients and in 78.1% of ILD patients associated with a diagnosis of connective tissue disease (CTD). In 25% of SSc-ILD patients, NVC proved necessary for a correct diagnosis. The presence of BCs and/or NVC positivity in ILD patients with normal levels of creatine phosphokinase is associated with amyopathic IIM, regardless the presence of RP. In conclusion, NVC is useful for the diagnostic assessment of incomplete forms of CTD and in amyopathic IIMs. NVC should be considered in the diagnostic assessment of ILD patients regardless of the presence of RP.

Eignung der Kapillarmikroskopie zur Diagnostik und Differenzierung einer ILD

2021 ◽  
pp. 1-3
Author(s):  
Sabine Adler
Keyword(s):  
Computed Tomography ◽  
Creatine Phosphokinase ◽  
Correct Diagnosis ◽  
Diagnostic Assessment ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Nailfold Videocapillaroscopy ◽  
High Resolution Computed Tomography ◽  
Diagnostic Role

Nailfold videocapillaroscopy (NVC) is an easy tool used for the assessment of patients with Raynaud’s phenomenon (RP) as possibly associated with systemic sclerosis (SSc). Recent insights have also highlighted its role in the diagnostic assessment of idiopathic inflammatory myopathies (IIMs). The aim of this study is to describe the diagnostic role of NVC in a series of 361 consecutive patients with interstitial lung disease (ILD). All the patients were assessed by clinical pulmonary and rheumatic examinations, blood exams, high-resolution computed tomography and NVC. NVC was considered positive only in the presence of avascular areas or giant capillaries, but also, the presence of bushy capillaries (BCs) was recorded. NVC was positive in 17.7% of ILD patients and in 78.1% of ILD patients associated with a diagnosis of connective tissue disease (CTD). In 25% of SSc-ILD patients, NVC proved necessary for a correct diagnosis. The presence of BCs and/or NVC positivity in ILD patients with normal levels of creatine phosphokinase is associated with amyopathic IIM, regardless the presence of RP. In conclusion, NVC is useful for the diagnostic assessment of incomplete forms of CTD and in amyopathic IIMs. NVC should be considered in the diagnostic assessment of ILD patients regardless of the presence of RP.

scholarly journals Predictive Features and Clinical Presentation of Interstitial Lung Disease in Inflammatory Myositis

2020 ◽  
Author(s):  
Tamara Vojinovic ◽  
Ilaria Cavazzana ◽  
Paolo Ceruti ◽  
Micaela Fredi ◽  
Denise Modina ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Function ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Usual Interstitial Pneumonia ◽  
Lung Function Test ◽  
High Resolution Computed Tomography ◽  
Inflammatory Myositis ◽  
Idiopathic Inflammatory Myositis

Abstract Interstitial lung disease (ILD) represents one of the most severe extra-muscular features of idiopathic inflammatory myositis (IIM). We aimed to identify any clinical and serological predictors of ILD in a monocentric cohort of 165 IIM patients. ILD+ patients were defined as having restrictive impairment in lung function tests and signs of ILD at chest high-resolution computed tomography (HRCT). Available HRCT images were centralized and classified in different ILD patterns: non-specific interstitial pneumonia (NSIP), organizing pneumonia (OP), usual interstitial pneumonia-like (UIP), indeterminate for UIP, and interstitial lung abnormalities (ILA). Lung function test data were recorded at onset, at 1 and 5 years after ILD diagnosis. ILD was found in 52 IIM patients (31.5%): 46.2% was affected by anti-synthetase syndrome (ARS), 21% by polymyositis (PM), 19% by dermatomyositis (DM), and 13.5% by overlap myositis. Most of ILD+ showed NSIP (31.9%), OP (19%), indeterminate for UIP (19%), and UIP (12.8%) patterns. At multivariate analysis, ILD was predicted by anti-Ro52 (p: 0.0026) and dyspnea (p: 0.015) at IIM onset. Most of ILD onset within is 12 months after IIM. In five cases, ILD occurs after 12 months since IIM diagnosis: these patients more frequently show dry cough and anti-Ku antibodies. Anti-Ro52 + ILD patients showed a significant increase of DLCO at 1 and 5 years of follow-up, compared with anti-Ro52 negative cases. ILD occurs in about one third of IIM and was predicted by dyspnea at onset and anti-Ro52 antibodies. Anti-Ro52 defines a subgroup of ILD showing a significant improvement of DLCO during follow-up. This retrospective study has been approved by local ethic committee (ASST-Spedali Civili of Brescia, Italy); protocol number: NP3511

scholarly journals The correlation between Ro-52 antibody and interstitial lung disease in dermatomyositis

2020 ◽  
Author(s):  
Xiaojing Xing ◽  
Anqi Li ◽  
Yonghong Hao ◽  
Chengxin Li
Keyword(s):  
Risk Factors ◽  
Computed Tomography ◽  
Risk Factor ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Classification Criteria ◽  
Serum Samples ◽  
High Resolution Computed Tomography ◽  
Inflammatory Myositis ◽  
Idiopathic Inflammatory Myositis

Abstract Background Recent studies have shown that Ro-52 antibody is associated with interstitial lung disease(ILD) in children with dermatomyositis(DM) and with the degree of disease severity. Moreover, we found that 50% or more cases of adult patients with DM tested positive for antibody Ro-52. Here, we analyzed the correlation between antibody Ro-52 and ILD in inpatients with DM. Objective To explore the correlation between Ro-52 antibody and ILD of DM. Methods A total of 153 patients with DM were collected, who met the classification criteria of idiopathic inflammatory myositis. Immunoblotting was used to detect 16 myositis-specific autoantibodies (MSAs) and myositis-associated autoantibodies (MAAs) from patient serum samples. High-resolution computed tomography (HRCT) was used to calculate the ILD severity score, and the tumors were screened. The clinical data and CT scores were analyzed retrospectively. Results Our data showed that antibodies of Ro-52 and MDA5 were risk factors for ILD development in patients with DM. Antibody Ro-52 was the most commonly occurring (52.9%) antibody in DM patients. The presence of Ro-52 in DM patients strongly suggested the possibility for the occurrence of ILD (86.4%). The severity of Ro-52-positive cases was higher than that of the Ro-52-negative cases.Ro-52 may increase the incidence of ILD when combined with other antibodies of PM-Scl75, PM-Scl100, TIF1-𝛾,Mi-2β,and MDA5. Conclusions The occurrence of ILD is highly likely in patients with DM having the antibody Ro-52. Thus, Ro-52 is a risk factor for ILD in DM.

Eignung der Kapillarmikroskopie zur Diagnostik und Differenzierung einer ILD

2020 ◽  
pp. 1-3
Author(s):  
Sabine Adler
Keyword(s):  
Computed Tomography ◽  
Creatine Phosphokinase ◽  
Correct Diagnosis ◽  
Diagnostic Assessment ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Nailfold Videocapillaroscopy ◽  
High Resolution Computed Tomography ◽  
Diagnostic Role

Nailfold videocapillaroscopy (NVC) is an easy tool used for the assessment of patients with Raynaud’s phenomenon (RP) as possibly associated with systemic sclerosis (SSc). Recent insights have also highlighted its role in the diagnostic assessment of idiopathic inflammatory myopathies (IIMs). The aim of this study is to describe the diagnostic role of NVC in a series of 361 consecutive patients with interstitial lung disease (ILD). All the patients were assessed by clinical pulmonary and rheumatic examinations, blood exams, high-resolution computed tomography and NVC. NVC was considered positive only in the presence of avascular areas or giant capillaries, but also, the presence of bushy capillaries (BCs) was recorded. NVC was positive in 17.7% of ILD patients and in 78.1% of ILD patients associated with a diagnosis of connective tissue disease (CTD). In 25% of SSc-ILD patients, NVC proved necessary for a correct diagnosis. The presence of BCs and/or NVC positivity in ILD patients with normal levels of creatine phosphokinase is associated with amyopathic IIM, regardless the presence of RP. In conclusion, NVC is useful for the diagnostic assessment of incomplete forms of CTD and in amyopathic IIMs. NVC should be considered in the diagnostic assessment of ILD patients regardless of the presence of RP.

scholarly journals AB0605 CLINICAL PROFILE AND CHEST HIGH-RESOLUTION COMPUTED TOMOGRAPHY (HRCT) FINDINGS IN PATIENTS WITH CONNECTIVE TISSUE DISEASES AND INTERSTITIAL LUNG DISEASE: EXPERIENCE OF A SINGLE REFERENCE RHEUMATOLOGY CENTER

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1598.2-1599
Author(s):  
I. Rusu ◽  
L. Muntean ◽  
M. M. Tamas ◽  
I. Felea ◽  
L. Damian ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Systemic Sclerosis ◽  
High Resolution ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Connective Tissue Diseases ◽  
High Resolution Computed Tomography ◽  
Hrct Patterns

Background:Interstitial lung disease (ILD) is a common manifestation of connective tissue diseases (CTDs), and is associated with significant morbidity and mortality. Chest high-resolution computed tomography (HRCT) play an important role in the diagnosis of ILD and may provide prognostic information.Objectives:We aimed to characterize the clinical profile and chest HRCT abnormalities and patterns of patients diagnosed with CTDs and ILD.Methods:In this retrospective, observational study we included 80 consecutive patients with CTDs and ILD referred to a tertiary rheumatology center between 2015 and 2019. From hospital charts we collected clinical data, immunologic profile, chest HRCT findings. HRCT patterns were defined according to new international recommendations.Results:Out of 80 patients, 64 (80%) were women, with a mean age of 55 years old. The most common CTD associated with ILD was systemic sclerosis (38.8%), followed by polymyositis (22.5%) and rheumatoid arthritis (18.8%). The majority of patients had dyspnea on exertion (71.3%), bibasilar inspiratory crackles were present in 56.3% patients and 10% had clubbing fingers. Antinuclear antibodies (ANA) were present in 78.8% patients, and the most frequently detected autoantibodies against extractable nuclear antigen were anti-Scl 70 (28.8%), followed by anti-SSA (anti-Ro, 17.5%), anti-Ro52 (11.3%) and anti-Jo (7.5%). Intravenous cyclophosphamide therapy for 6-12 months was used in 35% of patients, while 5% of patients were treated with mycophenolate mofetil.The most frequent HRCT abnormalities were reticular abnormalities and ground glass opacity. Non-specific interstitial pneumonia (NSIP) was identified in 46.3% CTDs patients. A pattern suggestive of usual interstitial pneumonia (UIP) was present in 32.5% patients, mainly in patients with systemic sclerosis. In 21.3% patients the HRCT showed reticulo-nodular pattern, micronodules and other abnormalities, not diagnostic for UIP or NSIP pattern.Conclusion:Nonspecific interstitial pneumonia (NSIP) is the most common HRCT pattern associated with CTDs. Further prospective longitudinal studies are needed in order to determine the clinical and prognostic significance of various HRCT patterns encountered in CTD-associated ILD and for better patient management.References:[1]Ohno Y, Koyama H, Yoshikaua T, Seki S. State-of-the-Art Imaging of the Lung for Connective Tissue Disease (CTD). Curr Rheumatol Rep. 2015;17(12):69.[2]Walsh SLF, Devaraj A, Enghelmeyer JI, Kishi K, Silva RS, Patel N, et al. Role of imaging in progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150)Disclosure of Interests:None declared

scholarly journals POS0842 LUNG ULTRASOUND TO ASSESS THE SEVERITY OF INTERSTITIAL LUNG DISEASE IN SYSTEMIC SCLEROSIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 675.1-675
Author(s):  
C. Bruni ◽  
L. Mattolini ◽  
L. Tofani ◽  
L. Gargani ◽  
N. Landini ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Quantitative Methods ◽  
Lung Ultrasound ◽  
Significant Negative Correlation ◽  
High Resolution Computed Tomography ◽  
Quantitative Score ◽  
Quantitative Scoring ◽  
B Lines

Background:Interstitial lung disease (ILD) is one of the most common complications and one of the main causes of morbidity and mortality in Systemic Sclerosis (SSc). High-resolution computed tomography (HRCT) is the gold standard for the diagnosis of ILD and it allows its quantification. Among semi-quantitative methods, Goh et al proposed a semi-quantitative scoring system to visually quantify ILD extent, with categorical cut-off of 20% to distinguish limited and extensive parenchymal involvement with prognostic implications. More recently, the use of radiomics has allowed the objective quantification of ILD through the use of dedicated software, which calculate different parameters of lung density.Given the exposure to ionizing radiation that the procedure entails, other methods of ILD evaluation are being studied, among which lung ultrasound (LUS) identifies the B-lines as a main feature of ILD. So far, different evidences have proposed the use of LUS for the screening of ILD, even in the early phases of the disease and in subclinical lung involvement.Objectives:the aim of this study is to test the role of LUS in quantifying the severity of SSc-ILD, evaluated with both semi-quantitative visual radiological and quantitative radiomic scores.Methods:Adult SSc patients classified according to the ACR/EULAR 2013 criteria patients were assessed with pulmonary function test (PFTs), lung ultrasound and HRCT over 60 days. CT images were analysed qualitatively (by presence/absence of ILD), semi-quantitatively (categorical Goh score <20% vs> 20% of extent and the continuous extent Goh score made from 5 levels’ assessment– 0 to 100%) and quantitatively [with the densitometric radiomic data obtained through the Horos software - Mean lung attenuation (MLA), Standard Deviation (SD), Kurtosis, Skewness and Lung volume (LV)]. LUS was used to quantify the B-lines detected in each patient by scanning a total of 13 intercostal spaces, on both anterior and posterior chest wall.Results:Among 59 SSc patients (81% women, mean age 48±14 years, 45% anti-Scl70 positive), 23 (39%) presented ILD on HRCT, of which 14 limited and 9 extensive. The mean visual semi-quantitative score was 6%, ranging from 0 to 66%. Our data showed a significantly different number of B-Lines in ILD vs non-ILD patients (median 38 vs 9, p <.005), a result which was further confirmed among non-ILD vs ILD> 20% (median 47 vs 9, p=.001) and ILD <20% (median 36 vs 9, p=.001) patients. Conversely, the number of B-lines was not statistically different between patients with ILD <20% and >20% (median 47 vs 36, p=.78). We observed a significant negative correlation between the number of B-lines and FVC (r=-.472, p<.05) TLC (r=-.436, p=.003), DLco (r=-.515, p<.001), DLCO/VA (r=.-306, p=.03). Finally, the number of B-lines showed a statistically significant correlation with the Goh score on 5 levels (r=.437, p=.001), MLA (r=.571, p<.001), kurtosis (r=-.285, p=.028), skewness (r=-.370, p = .004) and LV (r=-.277, p=.033). All data were confirmed analysing anterior and posterior B-Lines separately.Conclusion:Our study confirms that LUS represents a useful tool for the identification of SSc-ILD. In addition, we showed that LUS may be useful also for the quantification of the severity of SSc-ILD, by correlating with PFT parameters, radiomics parameters and visual radiological evaluation. Together with the PFTs, LUS could be used to increase the accuracy of the screening and, potentially, of the follow-up of SSc-ILD patients.Disclosure of Interests:Cosimo Bruni: None declared, Lavinia Mattolini: None declared, Lorenzo Tofani: None declared, Luna Gargani Consultant of: GE Healthcare, Philips Healthcare and Caption Health, Nicholas Landini: None declared, Gemma Lepri: None declared, Martina Orlandi: None declared, Serena Guiducci: None declared, Silvia Bellando Randone: None declared, Marco Matucci-Cerinic: None declared

scholarly journals Lung ultrasound B-lines and serum KL-6 correlate with the severity of idiopathic inflammatory myositis-associated interstitial lung disease

Rheumatology ◽  
2019 ◽  
Vol 59 (8) ◽  
pp. 2024-2029 ◽  
Author(s):  
Yukai Wang ◽  
Shaoqi Chen ◽  
Jianqun Lin ◽  
Xuezhen Xie ◽  
Shijian Hu ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Pulmonary Function ◽  
Lung Disease ◽  
Lung Ultrasound ◽  
Pulmonary Function Tests ◽  
Forced Expiratory Volume ◽  
Inflammatory Myositis ◽  
Idiopathic Inflammatory Myositis ◽  
Function Tests ◽  
B Lines

Abstract Objective Idiopathic inflammatory myositis-associated interstitial lung disease (IIM-ILD) significantly increases morbidity and mortality. Lung ultrasound B-lines and Krebs von den Lungen-6 (KL-6) are identified as new sonographic and serum markers of ILD, respectively. The aim of our work was to assess the role of B-lines and KL-6 as markers of the severity of IIM-ILD. For this purpose, the correlation among B-lines score, serum KL-6 levels, high-resolution CT (HRCT) score, and pulmonary function tests were investigated in IIM-ILD patients. Methods Thirty-eight patients with IIM-ILD underwent chest HRCT scans, lung ultrasound and pulmonary function tests (independently performed within 1 week) examination. To assess severity and extent of ILD at HRCT, the Warrick score was used. The B-lines score denoting the extension of ILD was calculated by summing the number of B-lines on a total of 50 scanning sites. Serum KL-6 levels (U/ml) was measured by chemiluminescent enzyme immunoassay. Results A significant correlation was found between the B-lines score and serum KL-6 levels (r = 0.43, P &lt; 0.01), and between the Warrick score and serum KL-6 levels (r = 0.45, P &lt; 0.01). A positive correlation between B-lines score and the Warrick score (r = 0.87, P &lt; 0.0001) was also confirmed. Both B-lines score and KL-6 levels inversely correlated to diffusion capacity for carbon monoxide (r = −0.77, P &lt; 0.0001 and r = −0.42, P &lt; 0.05, respectively) and total lung capacity (r = −0.73, P &lt; 0.0001 and r = −0.36, P &lt; 0.05, respectively). Moreover, B-lines correlated inversely with forced vital capacity (r = −0.73, P &lt; 0.0001), forced expiratory volume in 1 s (r = −0.69, P &lt; 0.0001). Conclusion B-lines score and serum KL-6 levels correlate with HRCT findings and pulmonary function tests, supporting their use as measures of IIM-ILD severity.

scholarly journals Applicability of radiomics in interstitial lung disease associated with systemic sclerosis: proof of concept

2020 ◽  
Author(s):  
K. Martini ◽  
B. Baessler ◽  
M. Bogowicz ◽  
C. Blüthgen ◽  
M. Mannil ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Pulmonary Function ◽  
Lung Disease ◽  
Data Augmentation ◽  
Visual Analysis ◽  
Area Under The Curve ◽  
Information Criterion ◽  
Visual Assessment ◽  
High Resolution Computed Tomography

Abstract Objective To retrospectively evaluate if texture-based radiomics features are able to detect interstitial lung disease (ILD) and to distinguish between the different disease stages in patients with systemic sclerosis (SSc) in comparison with mere visual analysis of high-resolution computed tomography (HRCT). Methods Sixty patients (46 females, median age 56 years) with SSc who underwent HRCT of the thorax were retrospectively analyzed. Visual analysis was performed by two radiologists for the presence of ILD features. Gender, age, and pulmonary function (GAP) stage was calculated from clinical data (gender, age, pulmonary function test). Data augmentation was performed and the balanced dataset was split into a training (70%) and a testing dataset (30%). For selecting variables that allow classification of the GAP stage, single and multiple logistic regression models were fitted and compared by using the Akaike information criterion (AIC). Diagnostic accuracy was evaluated from the area under the curve (AUC) from receiver operating characteristic (ROC) analyses, and diagnostic sensitivity and specificity were calculated. Results Values for some radiomics features were significantly lower (p < 0.05) and those of other radiomics features were significantly higher (p = 0.001) in patients with GAP2 compared with those in patients with GAP1. The combination of two specific radiomics features in a multivariable model resulted in the lowest AIC of 10.73 with an AUC of 0.96, 84% sensitivity, and 99% specificity. Visual assessment of fibrosis was inferior in predicting individual GAP stages (AUC 0.86; 83% sensitivity; 74% specificity). Conclusion The correlation of radiomics with GAP stage, but not with the visually defined features of ILD-HRCT, implies that radiomics might capture features indicating severity of SSc-ILD on HRCT, which are not recognized by visual analysis. Key Points • Radiomics features can predict GAP stage with a sensitivity of 84% and a specificity of almost 100%. • Extent of fibrosis on HRCT and a combined model of different visual HRCT-ILD features perform worse in predicting GAP stage. • The correlation of radiomics with GAP stage, but not with the visually defined features of ILD-HRCT, implies that radiomics might capture features on HRCT, which are not recognized by visual analysis.

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