scholarly journals A RARE CASE OF INTERSTITIAL LUNG DISEASE IN IDIOPATHIC INFLAMMATORY MYOSITIS ASSOCIATED WITH ANTI-MDA5

CHEST Journal ◽  
2021 ◽  
Vol 160 (4) ◽  
pp. A2092
Author(s):  
Peter Huh ◽  
Sudhir Rajan ◽  
Eduardo Solbes
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Rare Case ◽  
Inflammatory Myositis ◽  
Idiopathic Inflammatory Myositis

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 < 0.01), and between the Warrick score and serum KL-6 levels (r = 0.45, P < 0.01). A positive correlation between B-lines score and the Warrick score (r = 0.87, P < 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 < 0.0001 and r = −0.42, P < 0.05, respectively) and total lung capacity (r = −0.73, P < 0.0001 and r = −0.36, P < 0.05, respectively). Moreover, B-lines correlated inversely with forced vital capacity (r = −0.73, P < 0.0001), forced expiratory volume in 1 s (r = −0.69, P < 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 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.

scholarly journals AB0624 PREDICTIVE PARAMETERS FOR DEVELOPMENT OF INTERSTITIAL LUNG DISEASE IN IDIOPATHIC INFLAMMATORY MYOSITIS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1607.2-1607
Author(s):  
T. Vojinovic ◽  
M. Fredi ◽  
P. Ceruti ◽  
D. Modina ◽  
F. Franceschini ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Chest Ct ◽  
The Other ◽  
Inflammatory Myositis ◽  
Idiopathic Inflammatory Myositis ◽  
X Ray ◽  
Other Hand ◽  
Chest X Ray

Background:Idiopathic Inflammatory Myositis (IIM) is a group of heterogeneous connective tissue diseases, primarily characterized by chronic muscle inflammation as well as myositis-specific or myositis-associated autoantibodies and a spectrum of different extra-muscular features.The most frequent organ involment in IIM is Interstitial Lung Disease (ILD), occurring in 5-80% of different IIMs cases and considered the hallmark of morbidity and mortality in patients with IIMs.Objectives:To retrospectively assess the predictive factors for development of ILD in IIM patientsMethods:We retrospectively analyzed the prevalence of ILD in a single-center cohort of 165 IIM patients. Patient data was collected from clinical charts. ILD was diagnosed by chest X-ray scan and chest CT scan. All chest CT and chest X-ray scans available and performed at our hospital were consequently re-evaluated by our expert pneumologist for uniform evaluation.Results:Myositis-related ILD (M-ILD) was found in 52 IIM patients (31.5%): 46.15% was affected by anti-synthetase syndrome (ARS), 21.15% by polymyositis (PM), 19.23% by dermatomyositis (DM) and 13.46% by overlap myositis. The pulmonary involvement was characterized by Non-specific interstitial pneumonia (NSIP) (30.6%), Unusual Interstitial Pneumonia (UIP) (38.77%), Bronchiolitis Obliterans with Organizing Pneumonia (BOOP) (20.4%), overlap NSIP/BOOP (4.1%) and Undetermined/Unspecific pattern (6.12%). Eighty four percent of M-ILD consisted of non-smokers and 69.23% presented with dyspnea at onset.ILD was diagnosed in 90.38% of patients within the first year of IIM diagnosis (early onset ILD) and was associated with dyspnea and/or cough in 70.2% and 17% respectively. On the other hand, late onset ILD presented mostly with dyspnea and/or cough in 60% of cases and was significantly associated with anti-Ku antobodies.At onset ILD was significantly associated with: ARS (p<0.0001; OR:12.98), anti-Jo-1 (p<0.0001; OR:6.1), anti-Ro (p=0.038; OR:2.2), mechanic’s hands (p<0.0001; OR:10.41), arthritis (p=0.01; OR:2.58), polyarthritis (p=0.001; OR:4.578), dyspnea (p<0.0001; OR:9.66), and high levels of CPK (p=0.0001) and GOT (p=0.0146). By contrast, the following features: DM (p=0.012; OR:0.36), facial rash (p=0.003; OR:0.31), anti-NXP-2 (p=0.019; OR<0.0001), anti-PL-12 (p=0,03; OR<0.0001) and myositis (p<0.0001; OR:0.173) present at onset were less frequently associated with M-ILD.At multivariate analysis M-ILD was predicted by anti-Ro (p=0.0448), polyarthritis (p=0.0093) and dyspnea (p=0.0001) at onset. On the other hand, patients presenting myositis (p=0.0383) and facial rash (p=0.0398) at onset were less likely to developed M-ILD.Conclusion:ILD occurs in about one third of patients with IIM, mostly affected by ARS. The presence of anti-Ro antibodies as well as polyarthritis and dyspnea at onset predict the development of ILD.Disclosure of Interests:None declared

scholarly journals Treatment of idiopathic inflammatory myositis associated interstitial lung disease: A systematic review and meta-analysis

2019 ◽  
Vol 18 (2) ◽  
pp. 113-122 ◽  
Author(s):  
Thomas Barba ◽  
Romain Fort ◽  
Vincent Cottin ◽  
Steeve Provencher ◽  
Isabelle Durieu ◽  
...  
Keyword(s):  
Systematic Review ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Meta Analysis ◽  
Inflammatory Myositis ◽  
Idiopathic Inflammatory Myositis

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.

scholarly journals Myositis-Related Interstitial Lung Disease: A Respiratory Physician’s Point of View

Medicina ◽  
2021 ◽  
Vol 57 (6) ◽  
pp. 599
Author(s):  
Yuko Waseda
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Unknown Origin ◽  
Trna Synthetase ◽  
Point Of View ◽  
Aminoacyl Trna Synthetase ◽  
Inflammatory Myositis ◽  
Idiopathic Inflammatory Myositis ◽  
Inflammatory Drugs

Idiopathic inflammatory myositis (IIM) is an umbrella term for diseases of unknown origin that cause muscle inflammation. Dermatomyositis and polymyositis are IIMs that commonly cause interstitial lung disease (ILD). When a patient presents with ILD, the evaluation of whether the case displays the characteristics of myositis should be determined by interview, physical examination, imaging findings, the measurement of myositis-related antibodies, and the determination of disease severity after diagnosis. Rapidly progressing anti-melanoma differentiation-associated gene 5 antibody-positive ILD may require rapid multi-drug therapy, while anti-aminoacyl tRNA synthetase (ARS) antibody-positive ILD can be treated with anti-inflammatory drugs. Importantly, however, anti-ARS antibody-positive ILD often recurs and sometimes develops into fibrosis. Early diagnosis is crucial for treatment, and we therefore need to clarify the features of myositis associated with ILD and suspect these pathologies early. This section reviews what clinicians need to look for and what findings are evaluated in patients when diagnosing myositis associated with ILD.

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