Interstitial Lung Disease Associated with Anti-PM/Scl or Anti-Aminoacyl-tRNA Synthetase Autoantibodies: A Similar Condition?

2010 ◽  
Vol 37 (5) ◽  
pp. 1000-1009 ◽  
Author(s):  
JEAN-CHRISTOPHE LEGA ◽  
VINCENT COTTIN ◽  
NICOLE FABIEN ◽  
FRANÇOISE THIVOLET-BÉJUI ◽  
JEAN-FRANÇOIS CORDIER
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Clinical Manifestations ◽  
Trna Synthetase ◽  
Imaging Features ◽  
High Resolution Computed Tomography ◽  
Trna Synthetases ◽  
Ground Glass Attenuation ◽  
Pulmonary Manifestations ◽  
Cutaneous Rash

Objective.To compare anti-PM/Scl autoantibody-associated interstitial lung disease (ILD) with anti-aminoacyl-tRNA synthetases (anti-ARS) autoantibody-associated ILD.Methods.We retrospectively studied 21 patients with ILD from a department of respiratory medicine, including 9 with anti-PM/Scl autoantibodies (6 women, median age 55 yrs, followup 5.5 yrs) and 12 with anti-ARS autoantibodies (6 women, median age 59 yrs, followup 2.3 yrs).Results.Pulmonary manifestations in patients with anti-PM/Scl autoantibody-associated ILD usually followed the extrapulmonary manifestations of the connective tissue disease (CTD) (7/9 cases). The predominant imaging features on initial high resolution computed tomography were ground-glass attenuation and reticular opacities, and mainly suggested nonspecific interstitial pneumonia in both groups. CTD was classified as dermatomyositis (DM; 2), undifferentiated CTD (2), cutaneous limited systemic sclerosis (2), rheumatoid arthritis (RA; 1), and overlap syndrome (1) in the anti-PM/Scl group; and polymyositis (4), undifferentiated CTD (5), DM (1), amyopathic DM (1), and RA (1) in the anti-ARS group. Frequencies of arthralgia, Raynaud phenomenon, cutaneous rash, and mechanic’s hands were comparable in both groups. Myalgia or muscle weakness was present in 0/9 PM/Scl and 5/12 ARS patients (p < 0.05). More than 1 autoantibody was present in 11 patients. ILD worsened despite treatment in 4 patients with anti-PM/Scl autoantibodies and 2 with anti-ARS autoantibodies, and included 1 death.Conclusion.Anti-PM/Scl and anti-ARS antibodies are associated with similar clinical manifestations, with the exception only of more overt myositis in the latter, therefore challenging the clinical specificity of the antisynthetase syndrome.

scholarly journals Biomarkers and Autoantibodies of Interstitial Lung Disease with Idiopathic Inflammatory Myopathies

2015 ◽  
Vol 9s1 ◽  
pp. CCRPM.S36748 ◽  
Author(s):  
Hajime Yoshifuji
Keyword(s):  
Computed Tomography ◽  
High Resolution ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Trna Synthetase ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Aminoacyl Trna Synthetase ◽  
High Resolution Computed Tomography ◽  
Ground Glass Attenuation

Various autoantibodies are seen in idiopathic inflammatory myopathies. Among myositis-specific antibodies, anti-aminoacyl-tRNA synthetase and anti-melanoma differentiation-associated protein 5 (MDA5) antibodies are associated with interstitial lung disease (ILD). Anti-MDA5 antibodies are associated with dermatomyositis (DM) or clinically amyopathic DM complicated with rapidly progressive ILD. In anti-MDA5-positive patients, a random ground-glass attenuation pattern is a characteristic finding of ILD in chest high-resolution computed tomography. Conversely, anti-aminoacyl-tRNA synthetase antibodies are not associated with rapidly progressive ILD but with chronic ILD. DM or clinically amyopathic DM patients with anti-MDA5, and characteristic high-resolution computed tomography findings are highly likely to have devastating ILD and need aggressive treatment.

scholarly journals Anti-tRNA synthetase syndrome interstitial lung disease: A single center experience

2021 ◽  
Author(s):  
Erin M. Wilfong ◽  
Jennifer J. Young-Glazer ◽  
Bret K. Sohn ◽  
Gabriel Schroeder ◽  
Narender Annapureddy ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Medical Center ◽  
Statistical Significance ◽  
Tertiary Care ◽  
Trna Synthetase ◽  
Single Center Experience ◽  
Pulmonary Manifestations ◽  
Chart Abstraction

AbstractBackgroundRecognition of Anti tRNA synthetase (ARS) related interstitial lung disease (ILD) is key to ensuring patients have prompt access to immunosuppressive therapies. The purpose of this retrospective cohort study was to identify factors that may delay recognition of ARS-ILD.MethodsPatients seen at Vanderbilt University Medical Center (VUMC) between 9/17/2017-10/31/2018 were included in this observational cohort. Clinical and laboratory features were obtained via chart abstraction. Kruskal-Wallis ANOVA, Mann-Whitney U, and Fisher’s exact t tests were utilized to determine statistical significance.ResultsPatients with ARS were found to have ILD in 51.9% of cases, which was comparable to the frequency of ILD in systemic sclerosis (59.5%). The severity of FVC reduction in ARS (53.2%) was comparable to diffuse cutaneous systemic sclerosis (56.8%, p=0.48) and greater than dermatomyositis (66.9%, p=0.005) or limited cutaneous systemic sclerosis (lcSSc, 71.8%, p=0.005). Frank honeycombing was seen with ARS antibodies but not other myositis autoantibodies. ARS patients were more likely to first present to a pulmonary provider in a tertiary care setting (53.6%), likely due to fewer extrapulmonary manifestations. Only 33% of ARS-ILD were anti-nuclear antibody, rheumatoid factor, or anti-cyclic citrullinated peptide positive. Patients with ARS-ILD had a two-fold longer median time to diagnosis compared to other myositis-ILD patients (11.0 months, IQR 8.5 to 43 months vs. 5.0 months, IQR 3.0 to 9.0 months, p=0.003).ConclusionsARS patients without prominent extra-pulmonary manifestations are at high risk for not being recognized as having a connective tissue disease related ILD and miscategorized as UIP/IPF without comprehensive serologies.

scholarly journals Interstitial Lung Disease in Connective Tissue Disease: A Common Lesion With Heterogeneous Mechanisms and Treatment Considerations

2021 ◽  
Vol 12 ◽  
Author(s):  
Tihong Shao ◽  
Xiaodong Shi ◽  
Shanpeng Yang ◽  
Wei Zhang ◽  
Xiaohu Li ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Blood Vessels ◽  
Connective Tissue Disease ◽  
Clinical Manifestations ◽  
Respiratory Muscles ◽  
Diagnostic Process ◽  
Imaging Features ◽  
Laboratory Assessment

Connective tissue disease (CTD) related interstitial lung disease (CTD-ILD) is one of the leading causes of morbidity and mortality of CTD. Clinically, CTD-ILD is highly heterogenous and involves rheumatic immunity and multiple manifestations of respiratory complications affecting the airways, vessels, lung parenchyma, pleura, and respiratory muscles. The major pathological features of CTD are chronic inflammation of blood vessels and connective tissues, which can affect any organ leading to multi-system damage. The human lung is particularly vulnerable to such damage because anatomically it is abundant with collagen and blood vessels. The complex etiology of CTD-ILD includes genetic risks, epigenetic changes, and dysregulated immunity, which interact leading to disease under various ill-defined environmental triggers. CTD-ILD exhibits a broad spectra of clinical manifestations: from asymptomatic to severe dyspnea; from single-organ respiratory system involvement to multi-organ involvement. The disease course is also featured by remissions and relapses. It can range from stability or slow progression over several years to rapid deterioration. It can also present clinically as highly progressive from the initial onset of disease. Currently, the diagnosis of CTD-ILD is primarily based on distinct pathology subtype(s), imaging, as well as related CTD and autoantibodies profiles. Meticulous comprehensive clinical and laboratory assessment to improve the diagnostic process and management strategies are much needed. In this review, we focus on examining the pathogenesis of CTD-ILD with respect to genetics, environmental factors, and immunological factors. We also discuss the current state of knowledge and elaborate on the clinical characteristics of CTD-ILD, distinct pathohistological subtypes, imaging features, and related autoantibodies. Furthermore, we comment on the identification of high-risk patients and address how to stratify patients for precision medicine management approaches.

SAT0288 CHARACTERIZATION OF ANTI-AMINOACYL TRNA SYNTHETASE AUTOANTIBODIES IN PATIENTS WITH IDIOPATHIC INFLAMMATORY MYOPATHIES

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1088.1-1089
Author(s):  
C. Preger ◽  
A. Notarnicola ◽  
C. Hellström ◽  
E. Wigren ◽  
C. Cerqueira ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Inflammatory Myopathy ◽  
Clinical Manifestations ◽  
Trna Synthetase ◽  
University Hospital ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Research Support ◽  
Mortality And Morbidity

Background:Idiopathic inflammatory myopathies (IIM) are rare chronic inflammatory diseases associated with high mortality and morbidity [1]. One sub-group of IIM, anti-synthetase syndrome (ASS), is characterized by the presence of autoantibodies that target aminoacyl transfer(t) RNA synthetases (aaRS), together with specific clinical manifestations such as myositis, interstitial lung disease (ILD), arthritis, mechanic’s hand, Raynaud’s syndrome and fever [2]. The most common anti-aaRS autoantibody, anti-Jo1 targeting histidyl tRNA synthetase (HisRS), is present in up to 20-30% of patients with IIM, and up to 90% of patients with myositis and ILD [3, 4]. Besides Jo1, there are today seven other identified autoantigens within the aaRS family.Objectives:A large part of patients with IIM, including individuals with clinical manifestations indicating ASS, test seronegative to all known myositis specific autoantibodies. However, these patients could potentially harbor autoantibodies against targets not tested for in clinic. In this study, we aimed at extending the detection of autoantibodies by including all cytoplasmic aaRS in the analysis of patients with IIM. We hypothesized the existence of new potential autoantigens within this protein family.Methods:The presence of anti-aaRS autoantibodies was determined using a multiplex suspension bead array assay on 242 IIM patients from the Karolinska University Hospital myositis cohort. A panel of 186 recombinant constructs, representing 57 proteins that included full-length or partial sequence overlaps between constructs of all cytoplasmic aaRS as well as other myositis related proteins, were coupled to magnetic color-coded beads and each plasma sample was tested against the complete antigen panel.Results:By the use of this multiplex method we identified patients with autoantibodies against many of the tested aaRS. Autoantibodies binding to HisRS have previously been shown to bind with higher reactivity to the WHEP domain of HisRS and this was also confirmed in this study. We confirmed reactivity against three of the other aaRS tested for in the clinic (PL-12, PL-7, and EJ). In addition, we identified patients positive for anti-Zo, -KS and -HA, autoantibodies usually not screened for in routine. Finally, our data indicates that there are autoantibodies binding to other aaRS than the previously known eight autoantigens, which will be presented.Conclusion:In this study, we could detect autoantibodies in plasma from patients with IIM, both against the most common aaRS autoantigens, but also against other aaRS that are usually not tested for in clinic. We conclude that it is important to continue the studies of anti-aaRS autoantibodies, and their correlation to clinical manifestations, and in the long run also include more aaRS autoantigens in clinical practice.References:[1]Dobloug, G.C., et al., Mortality in idiopathic inflammatory myopathy: results from a Swedish nationwide population-based cohort study. Ann Rheum Dis, 2018. 77(1): p. 40-47.[2]Barsotti, S. and I.E. Lundberg, Myositis an evolving spectrum of disease. Immunol Med, 2018. 41(2): p. 46-54.[3]Vencovsky, J., H. Alexanderson, and I.E. Lundberg, Idiopathic Inflammatory Myopathies. Rheum Dis Clin North Am, 2019. 45(4): p. 569-581.[4]Richards, T.J., et al., Characterization and peripheral blood biomarker assessment of anti-Jo-1 antibody-positive interstitial lung disease. Arthritis Rheum, 2009. 60(7): p. 2183-92.Disclosure of Interests:Charlotta Preger: None declared, Antonella Notarnicola: None declared, Cecilia Hellström: None declared, Edvard Wigren: None declared, Catia Cerqueira: None declared, Peter Nilsson: None declared, Ingrid E. Lundberg Grant/research support from: Bristol Meyer Squibb, Corbus Pharmaceuticals, Inc and Astra Zeneca, Helena Persson: None declared, Susanne Gräslund: None declared, Per-Johan Jakobsson Shareholder of: Gesynta Pharma, Grant/research support from: Gesynta Pharma, AstraZeneca,

Interstitial lung lesion in systemic scleroderma (literature review and personal observation)

2020 ◽  
Vol 30 (1) ◽  
pp. 102-108
Author(s):  
M. S. Nashatyreva ◽  
I. N. Trofimenko ◽  
B. A. Chernyak
Keyword(s):  
Computed Tomography ◽  
Respiratory Failure ◽  
Clinical Study ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Clinical Manifestations ◽  
Lung Lesion ◽  
Systemic Scleroderma ◽  
Personal Observation ◽  
High Resolution Computed Tomography

Interstitial lesion is one of the most common lung pathologies in patients with systemic scleroderma (SS) In most cases, interstitial lung disease (ILD) is formed during the detailed clinical picture of SS, but it can manifest from interstitial lung disease, which significantly complicates early nosological diagnosis. Patterns of non-specific and common interstitial pneumonia are most often found among the variants of interstitial lung lesion at SS Clinical manifestations of ILD-SS are non-specific and vary significantly between patients from asymptomatic to rapidly progressing respiratory failure. Early diagnosis of subclinical interstitial pulmonary lesion at SS is carried out using high-resolution computed tomography. Active immunosuppressive therapy is required for timely diagnosis of progressive forms of ILD-SS The presented clinical study demonstrates a case of late diagnosis of ILD-SS

Bedeutung von Myositis-spezifischen Autoantikörpern bei der Dermatomyositis und Lungenbeteiligung

2021 ◽  
Vol 3 (1) ◽  
pp. 17-18
Author(s):  
Michael Sticherling
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Subcutaneous Emphysema ◽  
Poor Prognosis ◽  
Clinical Characteristics ◽  
Clinical Manifestations ◽  
Imaging Features ◽  
Amyopathic Dermatomyositis ◽  
Serological Tests ◽  
Medical University

<b>Objective:</b> To investigate the clinical characteristics of patients positive for anti-melanoma differentiation-associated gene 5 (MDA5) antibodies, and to analyse the potential pathogenesis of anti-MDA5 antibodies. <b>Methods:</b> The clinical manifestations, serological tests, imaging features, treatments, and prognoses of 32 anti-MDA5 antibody-positive patients diagnosed in the Rheumatology and Immunology Department of the Second Affiliated Hospital of Chongqing Medical University from September 2015 to August 2018 were analysed. <b>Results:</b> Of the 32 anti-MDA5 antibody-positive patients, eleven patients were clinically diagnosed with interstitial pneumonia with autoimmune features (IPAF), ten patients were diagnosed with clinically amyopathic dermatomyositis (CADM), six patients were diagnosed with dermatomyositis (DM) and five patients were diagnosed with anti-synthetase syndrome (ASS). Thirty patients had various degrees of pulmonary interstitial changes. The incidence of mortality, subcutaneous emphysema, hoarseness and dysphagia in patients who were positive for both anti-MDA5 and anti-Ro52 antibodies was significantly higher than in patients positive for only anti-MDA5 antibodies. The anti-MDA5 antibody-positive IPAF patients had a very poor prognosis, and mortality in these patients was as high as 54.55%. <b>Conclusion:</b> Anti-MDA5 antibodies are closely related to interstitial lung disease (ILD). The presence of both anti-MDA5 and anti-Ro52 antibodies indicates poor prognosis.

scholarly journals Interstitial Lung Disease and Diffuse Alveolar Hemorrhage, the Two Key Pulmonary Manifestations in Microscopic Polyangiitis

2021 ◽  
Vol 84 (4) ◽  
pp. 255-262
Author(s):  
Min Jung Kim ◽  
Kichul Shin
Keyword(s):  
Interstitial Lung Disease ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Microscopic Polyangiitis ◽  
Usual Interstitial Pneumonia ◽  
Clinical Manifestations ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Diffuse Alveolar Hemorrhage ◽  
Alveolar Hemorrhage ◽  
Pulmonary Manifestations

Microscopic polyangiitis (MPA) is an antineutrophil cytoplasmic antibody (ANCA)‒associated necrotizing vasculitis, which mainly affects small vessels in various organs, especially the lungs. The two key pulmonary manifestations, interstitial lung disease (ILD) and diffuse alveolar hemorrhage (DAH), increase the morbidity and death rate of patients with MPA. ILD is more common in MPA than in other ANCA-associated vasculitis subsets and is primarily associated with myeloperoxidase-ANCA. Unlike alveolar hemorrhage due to pulmonary capillaritis, ILD can initially manifest as isolated pulmonary fibrosis. Of note, its most frequent radiographic pattern is the usual interstitial pneumonia pattern, similar to the characteristic pattern seen in idiopathic pulmonary fibrosis. In this review we present the pathogenesis, clinical manifestations, and radiographic and histopathologic features of ILD and DAH in MPA. We also briefly summarize the outcome and therapeutic options for the two conditions.

scholarly journals The comparison of nailfold videocapillaroscopy findings between anti-melanoma differentiation-associated gene 5 antibody and anti-aminoacyl tRNA synthetase antibody in patients with dermatomyositis complicated by interstitial lung disease

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Reiko Wakura ◽  
Shogo Matsuda ◽  
Takuya Kotani ◽  
Takeshi Shoda ◽  
Tohru Takeuchi
Keyword(s):  
Interstitial Lung Disease ◽  
Disease Activity ◽  
Lung Disease ◽  
Trna Synthetase ◽  
Aminoacyl Trna Synthetase ◽  
Nailfold Videocapillaroscopy ◽  
High Resolution Computed Tomography ◽  
Survival Group ◽  
Positive Group ◽  
Death Group

Abstract Dermatomyositis (DM) is frequently complicated by interstitial lung disease (ILD), which increases mortality. This study aims to elucidate the clinical significance of nailfold videocapillaroscopy (NVC) on assessing the disease activity and prognosis of DM-ILD. We compared the NVC findings between anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive and anti-aminoacyl tRNA synthetase (anti-ARS) antibody-positive patients, the survival and ILD-related death groups, and examined the association of NVC findings with prognostic factors of DM-ILD. The median scores of microhemorrhage and capillary disorganization in the anti-MDA5 antibody-positive group were significantly higher than those in the anti-ARS antibody-positive group (P = 0.012 and 0.044, respectively). In contrast, the median scores of tortuous capillaries in the anti-ARS antibody-positive group were significantly higher than those in the anti-MDA5 antibody-positive group (P = 0.002). The median scores of microhemorrhage was significantly higher in the ILD-related death group than the survival group (P = 0.02). The scores of microhemorrhage, capillary disorganization, and neoangiogenesis correlated with known poor prognosis factors of DM-ILD. Additionally, the scores of microhemorrhage and capillary loss correlated significantly with the total fibrosis scores of chest high-resolution computed tomography. These findings suggest that NVC is a useful tool for assessing the disease activity and prognosis of DM-ILD.

scholarly journals HRCT imaging features of systemic sclerosis-associated interstitial lung disease

2021 ◽  
Vol 5 (1) ◽  
pp. 035-041
Author(s):  
Zhou Zexuan ◽  
Zhang Guangfeng ◽  
Xu Ting ◽  
Lin Haobo ◽  
Zhang Xiao
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Pulmonary Function Tests ◽  
Imaging Features ◽  
High Resolution Computed Tomography ◽  
Common Pattern ◽  
Nonspecific Interstitial Pneumonia ◽  
Evaluating Methods

Background: The aim of the study was to evaluate radiographic features of systemic sclerosis-associated interstitial lung disease. Patients and methods: 116 patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD) from 2010 to 2019 comprised our retrospective study. All patients were subject to high resolution computed tomography (HRCT). ILD patterns were classified into 7 patterns as IIPs and analyzed with pathology. We chose two staging method and two semi-quantitative score methods to evaluate the HRCT performance and analyzed with pulmonary function tests. Results: Ground-glass opacities were the most common presentation on HRCT, followed by interlobular septal thickening, reticular opacities, intralobular interstitial thickening; honeycombing, traction bronchiectasis and nodules can also be observed. The most common pattern of SSc-ILD was nonspecific interstitial pneumonia (NSIP), secondly was UIP. There was no difference in ILD pattern between HRCT and pathology, and revealed a high congruence. The four HRCT evaluating methods presented in this study all had significant relationships with PETs. Conclusion: The most common pattern of SSc-ILD was nonspecific interstitial pneumonia (NSIP). The ILD patterns of HRCT coincide very well with histology, and will replace pathology as the gold standard for diagnosis and evaluation of SSc-ILD.

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