scholarly journals Comparison of Clinical Features and Prognostic Factors of Polymyositis/Dermatomyositis Associated Interstitial Lung Disease According to Autoantibodies: Anti-Aminoacyl tRNA Synthetase Antibodies Versus Anti-Melanoma Differentiation-Associated Gene 5 Antibody.

2020 ◽  
Author(s):  
Yujuan Gao ◽  
Yan Li ◽  
Xin Yan ◽  
Miaomiao Xie ◽  
Hui Li ◽  
...  

Abstract Objective: The serum myositis-specific autoantibodies has been considered to be relatively specific and be useful for diagnosis of polymyositis/dermatomyositis associated interstitial lung disease (PM/DM-ILD). The goal of our retrospective study was to identify clinical features and prognostic factors for PM/DM-ILD based on serological phenotypes.Methods: PM/DM-ILD patients were diagnosed in the Department of Respiratory Medicine, Nanjing Drum Tower Hospital. MSAs were measured by anti-myositis antibody profile IgG detection kit. Based on the results of MSAs, the patients were divided into three groups: anti-MDA5 group, anti-Jo-1 group and other anti-ARS group. Kaplan-Meier, log rank, Kruskal-Wallis test and chi-square tests were used for analysis.Results: We identified 30 patients (22.0%) with positive anti-MDA5, 42 patients (31.0%) with positive anti-Jo-1 and 64 patients(47.0%) with other anti-ARS. acute disease onset was more frequently observed in the anti-MDA5 group (P = 0.005). The highest mortality rate was in the anti-MDA5 group (66.7%, P < 0.001). The overall survival of patients with anti-Jo-1 was significantly better than that of patients with anti-MDA5 (P < 0.001) and similar to that of patients with other anti-ARS. The acute disease onset (P = 0.002), DAD pattern for HRCT imaging (P < 0.001), current smokers (P = 0.02), presence of Anti-MDA5 (P < 0.001), fever (P < 0.001) were significantly associated with the mortality of the study population. Interestingly, in the subgroup survival analysis for anti-MDA5 group, age was a risk factor for death of patients with anti-MDA5 (P = 0.02), and the treatment with PSL pulse and IVIG were markedly correlated with high mortality (P < 0.001 and P = 0.001, respectively).Conclusion: Anti–MDA5 antibody is significantly associated with associated with worse prognosis in PM/DM-ILD patients. The application of PSL pulse and IVIG are not necessarily an effective treatment for positive anti-MDA5 patients.

2020 ◽  
Vol 2020 ◽  
pp. 1-8
Author(s):  
Yuhui Li ◽  
Yimin Li ◽  
Jian Wu ◽  
Miao Miao ◽  
Xiaojuan Gao ◽  
...  

Objective. Antimelanoma differentiation-associated protein 5 (anti-MDA5) autoantibody has been reported in dermatomyositis (DM) to be associated with rapidly progressive interstitial lung disease (RP-ILD). Our study is aimed at determining the clinical characteristics and prognostic factors underpinning anti-MDA5-associated RP-ILD. Methods. Patients with anti-MDA5-associated DM (aMDA5-DM) were identified at the Peking University People’s Hospital. The presence of anti-MDA5 antibody was determined by immunoblotting. Kaplan-Meier, chi-square test, univariate, and multivariate data analyses were used. Results. Out of 213 patients with DM and clinically amyopathic dermatomyositis (CADM), 20.7% (44/213) of patients were identified as aMDA5-DM. Amongst the aMDA5-DM patients, 63.6% (28/44) were identified as having anti-MDA5-associated RP-ILD. During the follow-up, 32.1% (9/28) of patients with anti-MDA5-associated RP-ILD died of respiratory failure. We identified older age and periungual erythema as two independent risk factors for RP-ILD mortality. Age ≥ 57 years at disease onset was significantly associated with poor survival ( P = 0.02 ) in patients with anti-MDA5-associated RP-ILD, while patients with periungual erythema had a better survival rate than those without periungual erythema ( P < 0.05 ). Conclusions. Anti-MDA5-associated RP-ILD is significantly associated with poor survival rates in DM/CADM patients. More effective intervention should be administered to anti-MDA5-associated RP-ILD patients, especially to senior patients and those without periungual erythema.


Rheumatology ◽  
2019 ◽  
Vol 59 (7) ◽  
pp. 1626-1631 ◽  
Author(s):  
Sarah L Tansley ◽  
Zoe Betteridge ◽  
Hui Lu ◽  
Emma Davies ◽  
Simon Rothwell ◽  
...  

Abstract Objectives It has been over 10 years since the first report of autoantibodies directed against phenylalanyl tRNA synthetase (anti-Zo) in a patient with features of the anti-synthetase syndrome. In that time no further cases have been published. Here we aim to characterize more fully the clinical phenotype of anti-Zo–associated myositis by describing the clinical features of nine patients. Methods Anti-Zo was identified by protein-immunoprecipitation in patients referred for extended spectrum myositis autoantibody testing at our laboratory. Results were confirmed by immunodepletion using a reference serum. Medical records were retrospectively reviewed to provide detailed information of the associated clinical phenotype for all identified patients. Where possible, HLA genotype was imputed using Illumina protocols. Results Nine patients with anti-Zo were identified. The median age at disease onset was 51 years, and six patients were female. Seven patients had evidence of inflammatory muscle disease, seven of interstitial lung disease and six of arthritis. The reported pattern of interstitial lung disease varied with usual interstitial pneumonia, non-specific interstitial pneumonia and organizing pneumonia all described. Other features of the anti-synthetase syndrome such as RP and mechanics hands were common. HLA data was available for three patients, all of whom had at least one copy of the HLA 8.1 ancestral haplotype. Conclusion Patients with anti-Zo presenting with features of the anti-synthetase syndrome and interstitial lung disease is a common finding. Like other myositis autoantibodies, there is likely to be a genetic association with the HLA 8.1 ancestral haplotype.


Rheumatology ◽  
2020 ◽  
Author(s):  
Shan Li ◽  
Yuxin Sun ◽  
Chi Shao ◽  
Hui Huang ◽  
Qian Wang ◽  
...  

Abstract Objectives Few studies have investigated the prognostic factors for idiopathic inflammatory myopathy-associated interstitial lung disease (IIM-ILD) across different clinical/serological phenotypes. Methods We conducted a retrospective analysis of patients diagnosed with IIM between January 2012 and December 2017. Results Of the 760 IIM cases registered, 679 adult cases were included in this study. ILD was present in 508 cases, and the presence of ILD in the clinically amyopathic DM, DM and PM groups was 92.7, 73.6 and 55.1%, respectively (P &lt; 0.01). The prevalence of ILD in the anti-synthetase antibody (ASA)+-IIM group was higher than that in ASA–-IIM group (95.2 vs 72.4%, P &lt; 0.01); no such difference was found between the anti-histidyl-tRNA synthetase (Jo-1)+-IIM and Jo-1–ASA+-IIM groups (93.0 vs 98.5%, P &gt; 0.05). The prevalence of ILD in the melanoma differentiation-associated protein-5 (MDA-5)+-IIM group was higher than that in MDA-5–-IIM group (97.8 vs 72.1%, P &lt; 0.01). Among adults with IIM, men with concurrent ILD, who were older than 50 years, were most likely to die. No significant difference was found in the all-cause mortality rates between DM-ILD and clinically amyopathic DM-ILD groups (33.3 vs 23%, P &gt; 0.05), although both were higher than that in PM group (13.2%, P = 0.01 and P &lt; 0.05, respectively). No difference was found in the all-cause mortality rates between MDA5–ASA–-IM-ILD and MDA5–ASA+-IM-ILD groups (17.2 vs 12.8%, P &gt; 0.05), and both were lower than that in MDA5+ASA–-IM-ILD group (33.7%, P &lt; 0.05). Conclusion The prevalence of ILD in IIM and the prognosis of IIM-ILD patients may vary depending on the statuses of the ASA and MDA-5 antibodies.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Xi Zhan ◽  
Wei Yan ◽  
Ying Wang ◽  
Qing Li ◽  
Xuhua Shi ◽  
...  

Abstract Background Anti-synthetase syndrome (ASSD) is a chronic autoimmune condition characterized by antibodies directed against an aminoacycl transfer RNA synthetase (ARS) along with a group of clinical features including the classical clinical triad: inflammatory myopathy, arthritis, and interstitial lung disease (ILD). ASSD is highly heterogenous due to different organ involvement, and ILD is the main cause of mortality and function loss, which presents as different patterns when diagnosed. We designed this retrospective cohort to describe the clinical features and disease behaviour of ASSD associated ILD. Methods Data of 108 cases of ASSD associated ILD were retrospectively collected in Beijing Chaoyang Hospital from December 2017 to March 2019. Data were obtained from the Electronic Medical Record system. Patients were divided into 5 groups according to distinct aminoacyl tRNA synthetase (ARS) antibodies. Results Overall, 108 consecutive patients were recruited. 33 were JO-1 positive, 30 were PL-7 positive, 23 were EJ positive, 13 were PL-12 positive and 9 were OJ positive. The JO-1 (+) group had a significant higher rate of mechanic’s hand (57.6%) than other 4 groups. Polymyositis/dermatomyositis (PM/DM) was diagnosed in 25 (23.1%) patients and no difference was observed among the 5 groups. The PL-7 (+) group had a higher frequency of UIP pattern (13.3%) than the other 4 groups but the difference was not significant, and the EJ (+) group had the most frequent OP pattern (78.2%), which was significantly higher than the PL-7 (+) (P < 0.001) and PL-12 (+) groups (P = 0.025). The median follow-up time was 10.7 months, during which no patients died. All received prednisone treatment, with or without immunosuppressants. At the 6-month follow-up, 96.3% of all patients (104/108) had a positive response to therapy, the JO-1 (+) and EJ (+) groups had a significantly higher improvement of forced vital capacity than the other 3 groups (P < 0.05), and the PL-7 group had the lowest FVC improvement (P < 0.05). The JO-1 (+) group and EJ (+) group had significantly higher anti-Ro-52 positive occurrence than the other 3 groups (P < 0.05). Conclusion Anti PL-7 antibody had the same frequency as anti-JO-1 in ASSD-ILD, in which the ILD pattern was different with distinct anti-ARS antibodies. Most ASSD-ILD had a positive response to steroid therapies, with or without immunosuppressants. The PL-7 (+) group had the highest occurrence of UIP pattern, and a significantly lower response to therapy.


RMD Open ◽  
2020 ◽  
Vol 6 (2) ◽  
pp. e001202
Author(s):  
Naoshi Nishina ◽  
Shinji Sato ◽  
Kenichi Masui ◽  
Takahisa Gono ◽  
Masataka Kuwana

ObjectivesTo investigate whether the onset of polymyositis (PM)/dermatomyositis (DM)-associated interstitial lung disease (ILD) is influenced by season and residence in the context of myositis-specific autoantibodies.MethodsFor patients with PM/DM-associated ILD enrolled in a multicentre cohort, 365 and 481 patients were eligible for seasonal and geographical analysis, respectively, based on the availability of reliable clinical information. The patients were divided into three groups: (1) anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive patients, (2) anti-aminoacyl tRNA synthetase (anti-ARS) antibody-positive patients and (3) patients negative for those antibodies. Seasonality was assessed by the Rayleigh test. Distance from residence to the nearest waterfront was measured on Google Map and was compared between groups by the exact Wilcoxon rank-sum test.ResultsIn anti-MDA5-positive patients, the disease developed more frequently in October–March (p=0.03), whereas a seasonal relationship was not found in the remaining two patient groups. Residence at disease onset in anti-MDA5-positive patients was significantly closer to the waterfront, especially to freshwater, compared with that in anti-ARS-positive or anti-MDA5-/ARS-negative patients (p=0.003 and 0.006, respectively).ConclusionsAnti-MDA5-associated ILD occurred predominantly from October to March in individuals residing near freshwater, suggesting an environmental influence on the onset of this disease subset.


2019 ◽  
Vol 78 (7) ◽  
pp. 988-995 ◽  
Author(s):  
Sara Sabbagh ◽  
Iago Pinal-Fernandez ◽  
Takayuki Kishi ◽  
Ira N Targoff ◽  
Frederick W Miller ◽  
...  

ObjectivesAnti-Ro52 autoantibodies are associated with more severe interstitial lung disease (ILD) in adult myositis patients with antiaminoacyl transfer (t)RNA synthetase autoantibodies. However, few studies have examined anti-Ro52 autoantibodies in juvenile myositis. The purpose of this study was to define the prevalence and clinical features associated with anti-Ro52 autoantibodies in a large cohort of patients with juvenile myositis.MethodsWe screened sera from 302 patients with juvenile dermatomyositis (JDM), 25 patients with juvenile polymyositis (JPM) and 44 patients with juvenile connective tissue disease–myositis overlap (JCTM) for anti-Ro52 autoantibodies by ELISA. Clinical characteristics were compared between myositis patients with and without anti-Ro52 autoantibodies.ResultsAnti-Ro52 autoantibodies were found in 14% patients with JDM, 12% with JPM and 18% with JCTM. Anti-Ro52 autoantibodies were more frequent in patients with antiaminoacyl tRNA synthetase (64%, p<0.001) and anti-MDA5 (31%, p<0.05) autoantibodies. After controlling for the presence of myositis-specific autoantibodies, anti-Ro52 autoantibodies were associated with the presence of ILD (36% vs 4%, p<0.001). Disease course was more frequently chronic, remission was less common, and an increased number of medications was received in anti-Ro52 positive patients.ConclusionsAnti-Ro52 autoantibodies are present in 14% of patients with juvenile myositis and are strongly associated with anti-MDA5 and antiaminoacyl tRNA synthetase autoantibodies. In all patients with juvenile myositis, those with anti-Ro52 autoantibodies were more likely to have ILD. Furthermore, patients with anti-Ro52 autoantibodies have more severe disease and a poorer prognosis.


Neurology ◽  
2018 ◽  
Vol 90 (23) ◽  
pp. e2068-e2076 ◽  
Author(s):  
Rebecca De Lorenzo ◽  
Iago Pinal-Fernandez ◽  
Wilson Huang ◽  
Jemima Albayda ◽  
Eleni Tiniakou ◽  
...  

ObjectiveTo define the clinical features of myositis patients with anti-PM/Scl-75 and/or anti-PM/Scl-100 autoantibodies at disease onset and during the course of disease and compare them to patients with other forms of myositis.MethodsIn this longitudinal cohort study, the prevalence and severity of clinical features at disease onset and during follow-up were compared between anti-PM/Scl-positive patients and those with the antisynthetase syndrome (AS), dermatomyositis (DM), and immune-mediated necrotizing myopathy (IMNM).ResultsForty-one anti-PM/Scl-positive, 132 AS, 178 DM, and 135 IMNM patients were included. Although muscle weakness was a presenting feature in just 37% of anti-PM/Scl-positive patients, 93% eventually developed weakness. Unlike the other groups, anti-PM-Scl-positive patients had more severe weakness in arm abductors than hip flexors. Interstitial lung disease was a presenting feature in just 10% of anti-PM/Scl-positive patients, but occurred in 61% during follow-up; fewer patients with DM (13%, p < 0.001) and IMNM (6%, p < 0.001) and more patients with AS (80%, p < 0.05) developed interstitial lung disease during the course of disease. Mechanic's hands (80%), Raynaud syndrome (78%), sclerodactyly (66%), telangiectasias (66%), esophageal reflux disease (61%), subcutaneous edema (46%), puffy hands (39%), and calcinosis (39%) occurred more frequently in anti-PM/Scl-positive patients than in the other groups. Although 30% of anti-PM/Scl-positive patients met criteria for systemic sclerosis, less than 5% had renal crisis or finger ulcerations. No differences were found between patients with only anti-PM/Scl-100 or only anti-PM/Scl-75 autoantibodies.ConclusionsUnlike patients with DM, AS, or IMNM, anti-PM/Scl-positive patients have weaker arm abductors than hip flexors. Anti-PM/Scl-positive patients also have the most extensive extramuscular features.


2021 ◽  
Author(s):  
Xi Zhan ◽  
YAN Wei ◽  
WANG Ying ◽  
LI Qing ◽  
SHI Xuhua ◽  
...  

Abstract BackgroundAnti-synthetase syndrome (ASSD) is a chronic autoimmune condition characterized by antibodies directed against an aminoacycl transfer RNA synthetase (ARS) along with a group of clinical features including the classical clinical triad: inflammatory myopathy, arthritis, and interstitial lung disease (ILD). ASSD is highly heterogenous due to different organ involvement, and ILD is the main cause of mortality and function loss, which presents as different patterns when diagnosed. We designed this retrospective cohort to describe the clinical features and disease behaviour of ASSD associated ILD. MethodsData of 108 cases of ASSD associated ILD were retrospectively collected in Beijing Chaoyang Hospital from 2017.12 to 2019.3. Data were obtained from the Electronic Medical Record (EMR) system. Patients were divided into 5 groups according to distinct aminoacyl tRNA synthetase (ARS) antibodies. Results:Overall, 108 consecutive patients were recruited. 33 were JO-1 positive,30 were PL-7 positive,23 were EJ positive, 13 were PL12 positive and 9were OJ positive. The JO-1 group had a significant higher rate of mechanic’s hand (57.6%) than other 4 groups. Polymyositis/ dermatomyositis (PM/DM) was diagnosed in 25 (23.1%) patients and no difference was observed among the 5 groups. The anti-PL7 positive group had a higher frequency of UIP pattern (13.3%) than the other 4 groups but the difference was not significant, and the EJ group had the most frequent OP pattern(78.2%), which was significantly higher than the PL-7 (P<0.001) and PL-12 groups (P=0.025). The median follow-up time was 10.7 months, during which no patients died. All received prednisone treatment, with or without immunosuppressants. At the 6-month-follow up, 96.3% of all patients (104/108) had a positive response to therapy, the JO-1 and EJ groups had a significantly higher improvement of forced vital capacity than the other 3 groups (P<0.05), and the PL-7 group had the lowest FVC improvement (P<0.05). The anti-JO1-positive group and anti-EJ-positive group had significantly higher anti-RO52-positive occurrence than the other 3 groups (P<0.05).ConclusionAnti PL-7 antibody had the same frequency as anti-JO1 in ASSD-ILD, in which the ILD pattern was different with distinct anti-ARS antibodies. Most ASSD-ILD had a positive response to steroid therapies, with or without immunosuppressants. The PL-7 group had the highest occurrence of UIP pattern, and a significantly lower response to therapy.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1256.1-1256
Author(s):  
J. Raja ◽  
S. Muthusamy ◽  
C. M. Ng

Background:Interstitial lung disease (ILD) is the leading cause of death in scleroderma (SSc) with decline in FVC as a predictor of mortality in patients with SSc-ILD, especially in the early course of the disease.Objectives:The aim of this study is to determine the survival rate of SSc-ILD in a Malaysian cohort of patients from University Malaya Medical Centre (UMMC).Methods:61 patients clinically diagnosed with SSc-ILD were identified and prospectively recruited. Baseline demographic data were collected. Kaplan-Meier analysis was used to estimate the survival.Results:Females were predominant (56, 91.8%). 39 (64%) had limited cutaneous SSc. Majority were ethnicity Chinese 30 (49.2%), followed by Malays 20 (32.8%), Indians 7 (11.4%) and others 4 (6.6%). Mean age was 56.25 (SD ± 12.5) years while mean duration of disease (non-Raynaud’s disease onset) was 10.5 years (SD ± 9.2) (range of 1 year to 44 years) years. 29 (47.5%) patients were positive for anti-ScL-70, whereas 6 (9.8%) patients were anti-centromere positive. There were 16 (26.2%) deaths. Median survival was 24 years. Patients had a sharper drop in survival probability for the first 10 years compared to the next 20 years (Figure 1). Median survival in limited subset was 24 years whereas in diffuse subset was 11 years. Patients from the limited subset appeared to have higher chance of surviving for 10 years and above, compared to those in the diffuse subset (Figure 2).Conclusion:The results demonstrate the poor survival in SSc-ILD patients. The survival rate tends to be worse in the first 10 years of SSc disease duration. Survival rate was poorer in patients with diffuse cutaneous subset.References:[1]Parelas A et al. Lancet Resp Med 2020[2]Goh NS et al. Am J Respir Crit Care Med 2008Disclosure of Interests:JASMIN RAJA Speakers bureau: For Boehringer Ingelheim for topic on Scleroderma-ILD, Grant/research support from: From Boehringer Ingelheim for scleroderma research, Shantini Muthusamy: None declared., CHOUNG MIN NG: None declared.


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