224th ENMC International Workshop:

Stenzel, Werner; Allenbach, Yves; Amato, Anthony A.; Aussey, Audrey; Benveniste, Olivier; Bleecker, Jan De; Groot, Ingrid de; Visser, Marjolein; Goebel, Hans H.; Hervier, B.; Fischer, N.; Hilton‐Jones, David; Lamb, J. Melvin; Lundberg, Ingrid E.; Mammen, Andrew L.; Mozaffar, Tahseen; Nishino, Ichizo; Pestronk, Alan; Schara, Ulrike; Stenzelr, Werner

doi:10.1016/j.nmd.2017.09.016

Cited by 371 publications

(208 citation statements)

References 57 publications

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“…CK elevations can be used for disease prognostication in that they correspond with increased disease severity and necrosis [2,5]. Median CK values in IMNM are between 4,000-7,000 U/L [7,12]. In this case, the patient's initial CK level was 13,510 U/L.…”

Section: Discussionmentioning

confidence: 87%

“…Several risk factors have been associated with IMNM such as statin exposure, malignancy, and viral infections, two of which stand out as attributable risks in this patient [2,7,12]. First, the patient had a prior history of statin-induced myopathy following three years of atorvastatin use.…”

Section: Discussionmentioning

confidence: 99%

“…A comprehensive paraneoplastic panel and CT of the chest, abdomen, and pelvis were negative. However, it is well documented that IMNM, particularly autoantibody-negative IMNM, has the highest risk of concomitant neoplastic disease [8,12].…”

Section: Discussionmentioning

confidence: 99%

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Autoantibody-Negative Immune-Mediated Necrotizing Myopathy Responds to Early and Aggressive Treatment: A Case Report

Marotta¹,

Zadourian²,

Jabaay³

et al. 2020

Cureus

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Immune-mediated necrotizing myopathy (IMNM) is a rare idiopathic disease that is further classified by the presence of serum antibodies. A modicum of patients lack serum autoantibodies. Significantly elevated creatine kinase (CK) is highly characteristic of IMNM. The pathophysiology of IMNM is partially understood, and effective treatment options are limited, particularly in patients without serum autoantibodies. In this case, we report a 76year-old male presenting with a four-month history of proximal muscle weakness. Muscle biopsy and serology confirmed the diagnosis of autoantibody-negative IMNM. Early and aggressive treatment with high-dose steroids and a course of intravenous immunoglobulin significantly reduced the patient's symptoms and CK within three months. This case serves as an example of an effective treatment outcome in a patient with this rare idiopathic necrotizing myopathy.

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Section: Discussionmentioning

confidence: 87%

Section: Discussionmentioning

confidence: 99%

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Autoantibody-Negative Immune-Mediated Necrotizing Myopathy Responds to Early and Aggressive Treatment: A Case Report

Marotta¹,

Zadourian²,

Jabaay³

et al. 2020

Cureus

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“…American College of Rheumatology/European League Against Rheumatism diagnosis criteria defined a novel probabilityscore model to diagnose patients with IIM from among a group of patients with myositis and without myositis. If they also aimed to classify the major subgroups of patients with IIM using a classification tree approach, they acknowledged a limited number of IMNM cases in their study, 27 which explains why they only predicted 3 subgroups of IIMs: DM (including juvenile and amyopathic subgroups), PM, and IBM. Our study completed the American College of Rheumatology/European League Against Rheumatism classification criteria by showing the presence of 4 entities comprising the IIM group.…”

Section: Discussionmentioning

confidence: 99%

“…We recommend the use of the established diagnosis criteria for these 4 IIM subgroups. 9,23,27,28 The algorithm showed that MSA were crucial for IIM classification and were probably more relevant than morphologic data in a large number of cases (eFigure 5 in the Supplement).…”

Section: Discussionmentioning

confidence: 99%

Development of a New Classification System for Idiopathic Inflammatory Myopathies Based on Clinical Manifestations and Myositis-Specific Autoantibodies

et al. 2018

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myopathies are heterogeneous in their pathophysiologic features and prognosis. The emergence of myositis-specific autoantibodies suggests that subgroups of patients exist.OBJECTIVE To develop a new classification scheme for idiopathic inflammatory myopathies based on phenotypic, biological, and immunologic criteria.DESIGN, SETTING, AND PARTICIPANTS An observational, retrospective cohort study was performed using a database of the French myositis network. Patients identified from referral centers for neuromuscular diseases were included from January 1, 2003, to February 1, 2016. Of 445 initial patients, 185 patients were excluded and 260 adult patients with myositis who had complete data and defined historical classifications for polymyositis, dermatomyositis, and inclusion body myositis were enrolled. All patients were tested for anti-histidyl-ARN-tsynthetase (Jo1), anti-threonine-ARN-t-synthetase (PL7), anti-alanine-ARN-t-synthetase (PL12), anti-complex nucleosome remodeling histone deacetylase (Mi2), anti-Ku, anti-polymyositis/systemic scleroderma (PMScl), anti-topoisomerase 1 (Scl70), and anti-signal recognition particle (SRP) antibodies. A total of 708 variables were collected per patient (eg, cancer, lung involvement, and myositis-specific antibodies). MAIN OUTCOMES AND MEASURESUnsupervised multiple correspondence analysis and hierarchical clustering analysis to aggregate patients in subgroups. RESULTS Among 260 participants (163 [62.7%] women; mean age, 59.7 years; median age [range], 61.5 years [48-71 years]), 4 clusters of patients emerged. Cluster 1 (n = 77) included patients who were male, white, and older than 60 years and had finger flexor and quadriceps weakness and findings of vacuolated fibers and mitochondrial abnormalities. Cluster 1 regrouped patients who had inclusion body myositis (72 of 77 patients [93.5%]; 95% CI, 85.5%-97.8%; P < .001). Cluster 2 (n = 91) regrouped patients who were women and had high creatine phosphokinase levels, necrosis without inflammation, and anti-SRP or anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) antibodies corresponding to immune-mediated necrotizing myopathy (53 of 91 [58.2%]; 95% CI, 47.4%-68.5%; P < .001). Cluster 3 (n = 52) regrouped patients who had dermatomyositis rash and anti-Mi2, anti-melanoma differentiation-associated protein 5 (MDA5), or anti-transcription intermediary factor-1γ (TIF1γ) antibodies, mainly corresponding with patients who had dermatomyositis (43 of 52 [82.7%]; 95% CI, 69.7%-91.8%; P < .001). Cluster 4 (n = 40) was defined by the presence of anti-Jo1 or anti-PL7 antibodies corresponding to antisynthetase syndrome (36 of 40 [90.0%]; 95% CI, 76.3%-97.2%; P < .001). The classification of an independent cohort (n = 50) confirmed the 4 clusters (Cohen κ light, 0.8; 95% CI, 0.6-0.9). CONCLUSIONS AND RELEVANCEThese findings suggest a classification of idiopathic inflammatory myopathies with 4 subgroups: dermatomyositis, inclusion body myositis, immune-mediated necrotizing myopathy, and antisynthetase syndrome. This classification...

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Autoantibodies at the Center of (sub)Classification—Issues of Detection—Reply

2019

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HAL is a multi-disciplinary open access archive for the deposit and dissemination of scientific research documents, whether they are published or not. The documents may come from teaching and research institutions in France or abroad, or from public or private research centers. L'archive ouverte pluridisciplinaire HAL, est destinée au dépôt et à la diffusion de documents scientifiques de niveau recherche, publiés ou non, émanant des établissements d'enseignement et de recherche français ou étrangers, des laboratoires publics ou privés.

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224th ENMC International Workshop:

Cited by 371 publications

References 57 publications

Autoantibody-Negative Immune-Mediated Necrotizing Myopathy Responds to Early and Aggressive Treatment: A Case Report

Autoantibody-Negative Immune-Mediated Necrotizing Myopathy Responds to Early and Aggressive Treatment: A Case Report

Development of a New Classification System for Idiopathic Inflammatory Myopathies Based on Clinical Manifestations and Myositis-Specific Autoantibodies

Autoantibodies at the Center of (sub)Classification—Issues of Detection—Reply

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