scholarly journals Anti-signal recognition particle autoantibody in patients with and patients without idiopathic inflammatory myopathy

2004 ◽  
Vol 50 (1) ◽  
pp. 209-215 ◽  
Author(s):  
Amy H. Kao ◽  
David Lacomis ◽  
Mary Lucas ◽  
Noreen Fertig ◽  
Chester V. Oddis
Keyword(s):  
Inflammatory Myopathy ◽  
Signal Recognition Particle ◽  
Idiopathic Inflammatory Myopathy ◽  
Signal Recognition

Clinical Characteristics of Immune-Mediated Necrotizing Myopathy with Autoantibodies Recognizing the Signal Recognition Particle: A Retrospective Study of a Chinese Cohort

2021 ◽  
Author(s):  
Qi Tang ◽  
Jinshen He ◽  
Feng Li ◽  
Jinwei Chen ◽  
Jing Tian ◽  
...  
Keyword(s):  
Logistic Regression ◽  
Inflammatory Myopathy ◽  
Signal Recognition Particle ◽  
Idiopathic Inflammatory Myopathy ◽  
Lower Limbs ◽  
Hunan Province ◽  
Signal Recognition ◽  
Necrotizing Myopathy ◽  
Immune Mediated ◽  
Chinese Cohort

Abstract Objective: Immune-mediated necrotizing myopathy (IMNM) with autoantibodies recognizing the signal recognition particle (SRP) patients tend to have prominent proximal weakness and infrequent extra-muscular involvement, especially interstitial lung disease (ILD). However, we reported a Chinese cohort of anti-SRP IMNM patients with relatively more frequent ILD.Methods: Anti-SRP IMNM patients from September 2016 to November 2019 were included according to the most recent European Neuromuscular Center criteria for IMNM. All sera for anti-SRP autoantibody and other myositis-related autoantibodies detection were obtained before the treatment initiation. Muscle strength, coexisting autoimmunity, complications including ILD, treatment and follow-up outcomes were also recorded. Univariate logistic regression was performed to determine variables predicting bad outcomes.Results: Of 271 patients with idiopathic inflammatory myopathy tested, we diagnosed 23 (8.5%) patients with anti-SRP IMNM. Muscle weakness was presented in 23 patients (100%) and generally worse in the lower limbs. ILD was observed in 50% anti-SRP IMNM patients. Predictor of bad outcomes identified by univariate logistic regression analysis was complicated ILD (odds ratio, 3.8).Conclusion: ILD tends to be more frequent in this Chinese anti-SRP IMNM cohort from Hunan province. Complicated ILD represents a risk factor for bad outcomes for anti-SRP IMNM.

scholarly journals Anti-MDA5 juvenile idiopathic inflammatory myopathy with second-degree heart block but no skin or lung involvement: a case report

2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Meghan E. Ryan ◽  
Daniel Cortez ◽  
Kelly R. Dietz ◽  
Peter Karachunski ◽  
Bryce A. Binstadt
Keyword(s):  
Heart Block ◽  
High Titer ◽  
Inflammatory Myopathy ◽  
Signal Recognition Particle ◽  
Idiopathic Inflammatory Myopathy ◽  
Cardiac Conduction ◽  
Signal Recognition ◽  
Pulmonary Abnormalities ◽  
Degree Heart Block ◽  
Second Degree

Abstract Background Patients with idiopathic inflammatory myopathy and autoantibodies directed against melanoma differentiation-associated protein 5 (MDA5) characteristically have interstitial lung disease, severe cutaneous involvement, arthritis, and relatively mild myositis. Cardiac involvement in idiopathic inflammatory myopathy can occur and has been associated with anti-signal recognition particle and anti-polymyositis-scleroderma autoantibodies, but not with anti-MDA5 autoantibodies. Case presentation A 14-year-old male presented with weakness, second-degree heart block, arthritis, and hematologic cytopenias. Imaging and biopsies confirmed the diagnosis of juvenile idiopathic inflammatory myopathy, and he had high titer anti-MDA5 autoantibodies. There were no cutaneous or pulmonary abnormalities. While on prednisone and methotrexate, the patient’s heart block improved from second- to first-degree and the cytopenias resolved. Persistent myositis prompted the addition of intravenous immunoglobulin. Seven months into the disease course, the arthritis and myositis are in remission and the patient is no longer taking corticosteroids. Conclusions We report a novel case of a patient with juvenile idiopathic myositis who lacked the typical cutaneous and pulmonary findings associated with anti-MDA5 positivity, but who had cardiac conduction defects. This report broadens the clinical spectrum of anti-MDA5-associated inflammatory myopathy.

Plasma exchange therapy in refractory inflammatory myopathy with anti-signal recognition particle antibody: a case series

Rheumatology ◽  
2021 ◽  
Author(s):  
Hao Zhang ◽  
Yue Sun ◽  
Honglei Liu ◽  
Xiaobing Cheng ◽  
Junna Ye ◽  
...  
Keyword(s):  
Lower Extremity ◽  
Plasma Exchange ◽  
Immunosuppressive Agents ◽  
Inflammatory Myopathy ◽  
Signal Recognition Particle ◽  
Case Series ◽  
Chest Ct ◽  
Signal Recognition ◽  
Muscle Testing ◽  
Global Activity

Abstract Objectives To explore the efficacy of plasma exchange (PE) therapy in refractory idiopathic inflammatory myopathy (IIM) patients with positive anti-signal recognition particle (SRP) antibody. Methods Nine refractory IIM patients with positive anti-SRP antibody were enrolled, who received PE therapy at Ruijin Hospital from Octobor 2017 to December 2020. The clinical manifestations, laboratory tests, chest CT and lower extremity MRI images before and after PE therapy were compared. The treatment response was evaluated by the 2016 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) myositis response criteria. Results 88.9% (8/9) subjects had achieved improvement by 3 weeks after PE therapy, with 55.6% (5/9) minimal improvement and 33.3% (3/9) moderate improvement. There were statistically significant improvements between baseline and after PE therapy at 3 weeks on the core set measures: physician global activity, patient global activity, Health Assessment Questionnaire (HAQ), manual muscle testing (MMT), extramuscular disease activity, and muscle enzymes activity including creatine kinase (CK), lactate dehydrogenase (LDH), aspartate transaminase (AST), except for alanine transaminase (ALT). Moreover, the chest CT showed regression of ground glass opacities and irregular linear opacities after PE therapy in four patients with interstitial lung disease. The MRI images of lower extremity in four patients showed reduction of muscle oedema after the therapy. Conclusion PE therapy is effective for refractory IIM patients with positive anti-SRP antibody. It should be considered as an alternative treatment for those patients who are resistant to the combined therapy of glucocorticoids and immunosuppressive agents.

scholarly journals Inflammatory myopathy with anti-signal recognition particle antibodies: case series of 100 patients

2015 ◽  
Vol 10 (1) ◽  
Author(s):  
Shigeaki Suzuki ◽  
Atsuko Nishikawa ◽  
Masataka Kuwana ◽  
Hiroaki Nishimura ◽  
Yurika Watanabe ◽  
...  
Keyword(s):  
Inflammatory Myopathy ◽  
Signal Recognition Particle ◽  
Case Series ◽  
Signal Recognition

In vivo pathogenicity of IgG from patients with anti-SRP or anti-HMGCR autoantibodies in immune-mediated necrotising myopathy

2018 ◽  
Vol 78 (1) ◽  
pp. 131-139 ◽  
Author(s):  
Cécile Bergua ◽  
Hélène Chiavelli ◽  
Yves Allenbach ◽  
Louiza Arouche-Delaperche ◽  
Christophe Arnoult ◽  
...  
Keyword(s):  
Inflammatory Myopathy ◽  
Signal Recognition Particle ◽  
Idiopathic Inflammatory Myopathy ◽  
Complement C3 ◽  
Active Immunisation ◽  
Immune Mediated ◽  
Coa Reductase ◽  
Grip Test ◽  
Antibody Levels

ObjectivesIn autoimmunity, autoantibodies (aAb) may be simple biomarkers of disease or true pathogenic effectors. A form of idiopathic inflammatory myopathy associated with anti-signal recognition particle (SRP) or anti-3-hydroxy-3-methylglutaryl-CoA reductase (HMGCR) aAb has been individualised and is referred to as immune-mediated necrotising myopathy (IMNM). The level of aAb correlates with IMNM activity and disease may respond to immunosuppression, suggesting that they are pathogenic. We aimed to evaluate the pathogenicity of IgG from patients with anti-SRP or anti-HMGCR aAb in vivo by developing the first mouse model of IMNM.MethodsIgG from patients suffering from anti-SRP or anti-HMGCR associated IMNM were passively transferred to wild-type, Rag2-/- or complement C3-/- mice. Muscle deficiency was evaluated by muscle strength on electrostimulation and grip test. Histological analyses were performed after haematoxylin/eosin staining or by immunofluorescence or immunohistochemistry analysis. Antibody levels were quantified by addressable laser bead assay (ALBIA).ResultsPassive transfer of IgG from patients suffering from IMNM to C57BL/6 or Rag2-/- mice provoked muscle deficiency. Pathogenicity of aAb was reduced in C3-/- mice while increased by supplementation with human complement. Breakage of tolerance by active immunisation with SRP or HMGCR provoked disease.ConclusionThis study demonstrates that patient-derived anti-SRP+ and anti-HMGCR+ IgG are pathogenic towards muscle in vivo through a complement-mediated mechanism, definitively establishing the autoimmune character of IMNM. These data support the use of plasma exchanges and argue for evaluating complement-targeting therapies in IMNM.

Sign in / Sign up

Export Citation Format

Share Document