scholarly journals Mechanic's hands in a patient with isolated anti‐Ro52 antibodies: antisynthetase syndrome without antisynthetase antibodies

2019 ◽  
Vol 18 (2) ◽  
pp. 147-148
Author(s):  
Peter Korsten ◽  
Jens Schmidt ◽  
Jörg Larsen ◽  
Cornelia S. Seitz
Keyword(s):  
Antisynthetase Syndrome ◽  
Antisynthetase Antibodies ◽  
Mechanic’S Hands

scholarly journals AB0092 ANTISYNTHETASE SYNDROME: CLINICAL PROFILE, SEROLOGIC AND TREATMENTS USED IN A COHORT OF PATIENTS FOLLOWED AT THE VIRGEN MACARENA HOSPITAL

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1075.1-1075
Author(s):  
P. Muñoz Reinoso ◽  
I. García Hernández ◽  
M. Ferrer Galván ◽  
F. J. Toyos Sáenz de Miera ◽  
L. Fernández de la Fuente Bursón ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Biological Therapy ◽  
Diagnostic Delay ◽  
Respiratory Function Tests ◽  
Mean Value ◽  
Frequent Pattern ◽  
Antisynthetase Syndrome ◽  
Antisynthetase Antibodies ◽  
Classical Triad

Background:The antisynthetase syndrome (SAS) is characterized by the presence of antisynthetase antibodies, anti-JO1, PL7 y PL12 are the most common; and the classic triad of myositis, arthritis, and diffuse interstitial lung disease (ILD)1. Most patients present incomplete forms and the severity of the ILD determines the prognosis of the disease2.Objectives:to analyze epidemiological, clinical and serological characteristics and treatments used in a cohort of patients with SAS.Methods:descriptive study of review of medical records. Data were collected from 15 patients with SAS followed in the Rheumatology and Pneumology consultations of the Virgen Macarena Hospital (Seville) in the last 10 years. The analysis was carried out using the R software.Results:15 patients were included, 8 men and 7 women. The median age was 56 years (33-77). Seven patients (47%) used to smoke. Four patients (27%) met the classical triad. All of them presented ILD and 8 patients (53%) had arthritis and / or myositis. Five (33%) had mechanic’s hands and six of them (40%) presented Raynaud. Seven (47%) suffered from dyspnea before the SAS diagnosis. The median diagnostic delay was 1 month (0-43). Seven (47%) patients had anti-JO1, 1 (7%) anti-PL7, 2 (13%) anti-PL12 and 2 (13%) patients anti-Ro52. Radiological patterns detected by HRCT were: 5 (33%) NINE, 4 (37%) NIU and 6 (40%) others. The initial treatment included mostly (66%) glucocorticoids (GC) and one or more cFAME. In maintenance, mycophenolate was used in 7 patients (47%), cyclosporine 5 (33%), cyclophosphamide in 3 cases (20%), azathioprine in 3 patients (20%) and methotrexate in 3 of them (20%). Four (37%) patients required a combination of DMARDs and 2 cases needed (13%) biological therapy, Rituximab and Tocilizumab. Changes in the mean value of the initial respiratory function tests (FVC1 and DLCO1) and during follow-up (FVC2 and DLCO2) were not relevant (FVC1 81.5% [42-110], FVC2 81% [59-115]; DLCO1 83% [10-112], DLCO2 80.5% [47-108]). Nine patients (60%) remained clinically stable and 3 patients (20%) progressed radiologically. Four patients died from ILD progression.Conclusion:In this study, the incomplete diagnosis of SAS predominated. The most detected antibody was anti-JO1. ILD is present in all cases, with NINE being the most frequent pattern so multidisciplinary management is necessary. Most used treatments were GC and FAMES combined, some cases required biological therapy.References:[1]Irazoque F, et al. Epidemiology, etiology and classification. Reumatol Clin. 2009;5:2-5.[2]Johnson C, et al. Clinical and pathologic differences in interstitial lung disease based on antisynthetase antibody type. Respir Med. 2014; 108(10):1542-8.Disclosure of Interests:None declared

scholarly journals POS1229 ANTI-MDA5 AND ANTISYNTHETASE ANTIBODIES SCREENING IN SEVERE SARS-COV-2 PNEUMONIA. BE AWARE OF FALSE POSITIVE RESULTS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 898.1-898
Author(s):  
A. Gil-Vila ◽  
J. Perurena-Prieto ◽  
C. Nolla-Fontana ◽  
O. Orozco-Galvez ◽  
M. Miarons-Font ◽  
...  
Keyword(s):  
Intensive Care Unit ◽  
Immune Response ◽  
Severe Acute Respiratory Syndrome ◽  
Chest Ct ◽  
Antisynthetase Syndrome ◽  
Antisynthetase Antibodies ◽  
Group A ◽  
Group B ◽  
Positive Results ◽  
Glass Pattern

Background:Several reports have shown that severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection may trigger a vigorous immune response that could lead to the appearance of various autoantibodies such as antinuclear antibodies, antiphospholipid antibodies or anti-neutrophil cytoplasmic antibodies, among others. Moreover, the pulmonary involvement in SARS-CoV-2 may resemble that of patients with anti-MDA5 positive syndrome or acute form of antisynthetase syndrome.Objectives:Our aim was to analyse the presence of anti-MDA5 and other myositis-specific autoantibodies such as the antisynthetase antibodies in patients diagnosed with severe acute respiratory syndrome caused by SARS-CoV-2.Methods:Retrospective observational study performed in a tertiary care center. We included 28 patients admitted to the intensive care unit with severe acute respiratory syndrome, 14 at the onset of the disease (group A) and 14 after 30 days of being treated in an intensive care unit (group B). Chest CT was performed at the admission. We analyzed the presence of anti-MDA5 and antisynthetase antibodies by immunoblot (Euroimmune®) and in those who were positive we performed a confirmatory test by immunoprecipitation.Results:All chest CT showed bilateral ground glass pattern. Three out of 14 patients of group A (12 males, 86%, mean ± SD age 67.1 ± 12.2) were positive for antisynthetase antibodies (2 anti-PL7, 1 anti-Jo1), and 6 out of 14 patients of the group B (6 males, 48%, mean ± SD age 68.7 ± 8.1) were positive to antisynthetase antibodies (2 anti-PL7, 2 anti-PL-12, 1 anti-EJ, 1 anti-OJ+PL7). Immunoblots also show positivity for other myositis-specific or associated antibodies, such as anti-TIF1g, anti-PM75, anti-SAE and anti-SRP. All of these results found by immunoblotting were negative by immunoprecipitation. None of the 28 patients were positive for anti-MDA5 antibodies.Conclusion:Severe SARS-CoV-2 pneumonia is characterized by ground glass pattern in chest CT, as it is found in anti-MDA5 or antisynthetase syndrome. The positivity of several myositis related autoantibodies showed in immunoblot appears to be more related to the vigorous immune response producing polyclonal immunoglobulins than triggering a real myositis-associated interstitial lung disease. Clinicians must be aware about these false positive results in patients with severe COVID-19 acute respiratory syndrome.References:[1]Xu Q. MDA5 should be detected in severe COVID-19 patients. Med Hypotheses. 2020; 143:109890.[2]Giannini M, Ohana M, Nespola B, Zanframundo G, Geny B, Meyer A. Similarities between COVID-19 and anti-MDA5 syndrome: what can we learn for better care? Eur Respir J. 2020; 56:2001618.[3]Vlachoyiannopoulos PG, Magira E, Alexopoulos H, Jahaj E, Theophilopoulou K, Kotanidou A, Tzioufas AG. Autoantibodies related to systemic autoimmune rheumatic diseases in severely ill patients with COVID-19. Ann Rheum Dis. 2020 Dec;79(12):1661-1663Disclosure of Interests:None declared

scholarly journals Influence of Antisynthetase Antibodies Specificities on Antisynthetase Syndrome Clinical Spectrum Time Course

2019 ◽  
Vol 8 (11) ◽  
pp. 2013 ◽  
Author(s):  
Cavagna ◽  
Trallero-Araguás ◽  
Meloni ◽  
Cavazzana ◽  
Rojas-Serrano ◽  
...  
Keyword(s):  
Time Course ◽  
Clinical Presentation ◽  
Reference Group ◽  
Trna Synthetase ◽  
Antisynthetase Syndrome ◽  
Aminoacyl Trna Synthetase ◽  
Trna Synthetases ◽  
Aminoacyl Trna Synthetases ◽  
Antisynthetase Antibodies ◽  
Clinical Triad

Antisynthetase syndrome (ASSD) is a rare clinical condition that is characterized by the occurrence of a classic clinical triad, encompassing myositis, arthritis, and interstitial lung disease (ILD), along with specific autoantibodies that are addressed to different aminoacyl tRNA synthetases (ARS). Until now, it has been unknown whether the presence of a different ARS might affect the clinical presentation, evolution, and outcome of ASSD. In this study, we retrospectively recorded the time of onset, characteristics, clustering of triad findings, and survival of 828 ASSD patients (593 anti-Jo1, 95 anti-PL7, 84 anti-PL12, 38 anti-EJ, and 18 anti-OJ), referring to AENEAS (American and European NEtwork of Antisynthetase Syndrome) collaborative group’s cohort. Comparisons were performed first between all ARS cases and then, in the case of significance, while using anti-Jo1 positive patients as the reference group. The characteristics of triad findings were similar and the onset mainly began with a single triad finding in all groups despite some differences in overall prevalence. The “ex-novo” occurrence of triad findings was only reduced in the anti-PL12-positive cohort, however, it occurred in a clinically relevant percentage of patients (30%). Moreover, survival was not influenced by the underlying anti-aminoacyl tRNA synthetase antibodies’ positivity, which confirmed that antisynthetase syndrome is a heterogeneous condition and that antibody specificity only partially influences the clinical presentation and evolution of this condition.

scholarly journals Immune myopathies with perimysial pathology

2018 ◽  
Vol 5 (2) ◽  
pp. e434 ◽  
Author(s):  
Robert C. Bucelli ◽  
Alan Pestronk
Keyword(s):  
Inflammatory Arthritis ◽  
Antinuclear Antibodies ◽  
Vascular Pathology ◽  
Comorbid Conditions ◽  
Raynaud Phenomenon ◽  
Regular Screening ◽  
Increased Risk ◽  
Antisynthetase Antibodies ◽  
Pathology Review ◽  
Mechanic’S Hands

ObjectiveImmune myopathies with perimysial pathology (IMPP) have a combination of damage to perimysial connective tissue and muscle fiber necrosis, more prominent near the perimysium. We studied the clinical and laboratory correlates of patients with pathologically defined IMPP.MethodsThis is a retrospective chart and pathology review of 57 consecutive patients with IMPP myopathology and, for comparison, 20 patients with dermatomyositis with vascular pathology (DM-VP).ResultsCompared with DM-VP, IMPP patients more commonly had interstitial lung disease (ILD) (p < 0.01), Raynaud phenomenon (p < 0.05), mechanic's hands (p < 0.05), arthralgias (p < 0.001), and a sustained response to immunomodulatory therapy (p < 0.05), and less frequently had a concurrent malignancy (p < 0.01). IMPP patients had higher serum creatine kinase values (p < 0.05), more frequent serum Jo-1 (p < 0.03) or SSA/SSA52 autoantibodies (p < 0.05), and less frequent antinuclear antibodies (p < 0.01). IMPP patients with serum Jo-1/antisynthetase antibodies were more likely to have ILD (p < 0.05) and inflammatory arthritis (p < 0.05) than IMPP patients without these antibodies.ConclusionsIMPP myopathology is associated with an increased risk of ILD, Raynaud phenomenon, mechanic's hands, and inflammatory arthritis when compared with another immune myopathy (DM-VP). IMPP patients require regular screening for ILD, particularly those with antisynthetase antibodies. The absence of myositis-specific autoantibodies in a large percentage of IMPP patients emphasizes the important role for myopathology in identifying patients at higher risk of severe comorbid conditions such as ILD.

Anti-Ro52 antibodies, antisynthetase antibodies, and antisynthetase syndrome

2007 ◽  
Vol 27 (4) ◽  
pp. 521-523 ◽  
Author(s):  
Vidya S. Limaye ◽  
John Cassidy ◽  
Grace Scott ◽  
Peter J. Roberts-Thomson ◽  
David Gillis
Keyword(s):  
Antisynthetase Syndrome ◽  
Antisynthetase Antibodies

Idiopathic inflammatory myopathies and antisynthetase syndrome: contribution of antisynthetase antibodies to improve current classification criteria

2019 ◽  
Vol 78 (9) ◽  
pp. 1291-1292 ◽  
Author(s):  
Martin Greco ◽  
María Jesus García de Yébenes ◽  
Inmaculada Alarcón ◽  
Anahy María Brandy-García ◽  
Íñigo Rúa-Figueroa ◽  
...  
Keyword(s):  
Classification Criteria ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Antisynthetase Syndrome ◽  
Antisynthetase Antibodies ◽  
Current Classification

scholarly journals FRI0306 IDIOPATHIC INFLAMMATORY MYOPATHIESAND ANTISYNTHETASE SYNDROME: CONTRIBUTION OF ANTISYNTHETASE ANTIBODIES TO IMPROVE CURRENT CLASSIFICATION CRITERIA

2019 ◽  
Author(s):  
Martín Greco ◽  
María Jesús García de Yébenes ◽  
Inmaculada Alarcón ◽  
Anahy Brandy-Garcia ◽  
Iñigo Rua-Figueroa ◽  
...  
Keyword(s):  
Classification Criteria ◽  
Antisynthetase Syndrome ◽  
Antisynthetase Antibodies ◽  
Current Classification

scholarly journals Mechanic’s hands and hiker’s feet in antisynthetase syndrome

2017 ◽  
Vol 189 (44) ◽  
pp. E1365-E1365 ◽  
Author(s):  
Madelaine Wernham ◽  
Steven J. Montague
Keyword(s):  
Antisynthetase Syndrome ◽  
Mechanic’S Hands

scholarly journals Rituximab in the Treatment of Interstitial Lung Disease Associated with Antisynthetase Syndrome: A Multicenter Retrospective Case Review

2018 ◽  
Vol 45 (6) ◽  
pp. 841-850 ◽  
Author(s):  
Tracy J. Doyle ◽  
Namrata Dhillon ◽  
Rachna Madan ◽  
Fernanda Cabral ◽  
Elaine A. Fletcher ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Pulmonary Function ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Clinical Investigation ◽  
Medical Center ◽  
Usual Interstitial Pneumonia ◽  
Antisynthetase Syndrome ◽  
Cryptogenic Organizing Pneumonia ◽  
Antisynthetase Antibodies

Objective.To assess clinical outcomes including imaging findings on computed tomography (CT), pulmonary function testing (PFT), and glucocorticoid (GC) use in patients with the antisynthetase syndrome (AS) and interstitial lung disease (ILD) treated with rituximab (RTX).Methods.We retrospectively identified all patients at 2 institutions with AS-ILD who were treated with RTX. Baseline demographics, PFT, and chest CT were assessed before and after RTX. Two radiologists independently evaluated CT using a standardized scoring system.Results.Twenty-five subjects at the Brigham and Women’s Hospital (n = 13) and University of Pittsburgh Medical Center (n = 12) were included. Antisynthetase antibodies were identified in all patients (16 Jo1, 6 PL-12, 3 PL-7). In 21 cases (84%), the principal indication for RTX use was recurrent or progressive ILD, owing to failure of other agents. Comparing pre- and post-RTX pulmonary variables at 12 months, CT score and forced vital capacity were stable or improved in 88% and 79% of subjects, respectively. Total lung capacity (%) increased from 56 ± 13 to 64 ± 13 and GC dose decreased from 18 ± 9 to 12 ± 12 mg/day. Although DLCO (%) declined slightly at 1 year, it increased from 42 ± 17 to 70 ± 20 at 3 years. The most common imaging patterns on CT were nonspecific interstitial pneumonia (NSIP; n = 13) and usual interstitial pneumonia/fibrotic NSIP (n = 5), of which 5 had concurrent elements of cryptogenic organizing pneumonia.Conclusion.Stability or improvement in pulmonary function or severity of ILD on CT was seen in most patients. Use of RTX was well tolerated in the majority of patients. RTX may play a therapeutic role in patients with AS-ILD, and further clinical investigation is warranted.

'Mechanic's hands': a misleading cutaneous sign of the antisynthetase syndrome

2007 ◽  
Vol 156 (1) ◽  
pp. 192-194 ◽  
Author(s):  
C. Bachmeyer ◽  
I. Tillie-Leblond ◽  
A. Lacert ◽  
J. Cadranel ◽  
S. Aractingi
Keyword(s):  
Antisynthetase Syndrome ◽  
Mechanic’S Hands
Sign in / Sign up

Export Citation Format

Share Document