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Abstract Number: 2314

Anti-Mi2 Dermatomyositis Revisited: Pure DM Phenotype with Muscle Fiber Necrosis and High Risk of Malignancy

Océane Landon-Cardinal1, Grégoire Monseau2, Yoland Schoindre3, Aude Rigolet1, Nicolas Champtiaux1, Baptiste Hervier1, Agathe Masseau4, Eric Hachulla5, Thomas Papo6, Benjamin Terrier7, Alain Meyer8, Jean-Emmanuel Kahn3, François Maurier9, Francis Gaches10, Emmanuelle Salort-Campana11, Thierry Zenone12, Nathalie Costedoat-Chalumeau7, Florian Perez13, Maxime Samson14, Anne-Marie Piette3, Guillaume Moulis10, Sylvain Audia15, Séverine Genot16, Nicolas Schleinitz17, Guillaume Lefevre5, Laurence Verneuil18, Olivier Benveniste19, Yves Allenbach1 and Boris Bienvenu20, 1Internal Medicine, Pitié-Salpêtrière University Hospital, Paris, France, 2Internal Medicine, Caen University Hospital, Caen, France, 3Internal Medicine, Foch Hospital, Suresnes, France, 4Internal Medicine, Nantes University Hospital, Nantes, France, 5Internal Medicine, Lille University Hospital, Lille, France, 6Bichat University Hospital - Internal Medicine, Paris, France, 7Internal Medicine, Cochin University Hospital, Paris, France, 8Rheumatology, Strasbourg University Hospital, Strasbourg, France, 9Internal Medicine, Sainte-Blandine de Metz Hospital, Metz, France, 10Internal Medicine, Toulouse University Hospital, Toulouse, France, 11Neurology, La Timone University Hospital, Marseille, France, 12Internal Medicine, Valence Hospital, Valence, France, 13Neurology, Albi Hospital, Albi, France, 14Dijon University Hospital, Dijon, France, 15Internal Medicine, Dijon University Hospital, Dijon, France, 16Internal Medicine, Martigues Hospital, Martigues, France, 17La Timone University Hospital, Marseille, France, 18Dermatology, Caen University Hospital, Caen, France, 19Pitié-Salpêtrière University Hospital, Paris, France, 20Caen University Hospital, Caen, France

Meeting: 2016 ACR/ARHP Annual Meeting

Date of first publication: September 28, 2016

Keywords: Cancer, dermatomyositis and myositis

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Session Information

Date: Tuesday, November 15, 2016

Session Title: Muscle Biology, Myositis and Myopathies - Poster II: Clinical

Session Type: ACR Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose:  Anti-Mi2 autoantibodies (Aabs) have been proposed to be highly specific for dermatomyositis (DM) and to be associated with a DM classical phenotype consisting of typical skin rashes and low extra-muscular features. Cancer has been estimated in about 30% of all DM patients. Patients with anti-Mi2 DM are considered having a good prognosis, possibly related to a markedly lower risk of malignancy reported in this subset. Nonetheless, there has been only a few and small cohort descriptions of this DM subgroup. Our objective was therefore to describe the phenotype of anti-Mi-2 DM in a large French cohort.

Methods:  A national multicenter retrospective cohort study was performed (15 medical centers) including all patients with a clinical phenotype suggestive of DM (cutaneous manifestations and/or muscle involvement) and a positive anti-Mi2 Aabs. Medical records were retrospectively reviewed. Muscle strength was assessed using the Medical Research Council (MRC) scale and cancer-associated myositis (CAM) was defined as a cancer occurring ± 3 years of diagnosing myositis.

Results: A total of 65 patients were identified, 62% were female and mean age at diagnosis was 54 years old (yo) (±17 yo). DM skin rash was reported in 88% of patients, most frequently Gottron papules and/or sign (68%), periungueal erythema (51%) and heliotrope rash (40%). Peripheral muscle weakness was reported in 92% of patients and dysphagia was reported in 34% of patients. At diagnosis, patients displayed severe muscle weakness (MRC 3/5, ±1/5) with mean CK level of 5085 UI/L (±5535 UI/L). Systematic review of muscle biopsies (n=11) showed marked inflammatory infiltrates. Strikingly, necrosis and regeneration were identified in all patients (n=11/11). C5b-9 deposition was found in all patients mainly on non-necrotic fibers but only sparsely on capillaries and without prominent capillary loss. Arthritis, Raynaud phenomenon and interstitial lung disease were reported in less than 20 % of patients. CAM was identified in 20% of patients and detected within one year and a half of DM diagnosis in most patients (n=11/12). All CAM patients, but one (38 yo), were diagnosed over 50 yo. There was no predominant histological subtype of malignancy (gastro-intestinal, urological, gynecological and pulmonary) and cancer was metastatic in a third of patients. Survival rate was 83% after a mean follow-up of 4.9 years from cancer diagnosis. Ninety-eight percent of patients were initially treated with corticosteroids (CS), in combination with immunosuppressant (IS) in 60% of cases. Patients treated with CS monotherapy (n=14), needed second-line agents upon follow-up in 60% of cases. In all, 53% of patients relapsed upon CS and/or IS tapering.

Conclusion: In this large French cohort, patients with anti-Mi2 DM displayed a phenotype with 3 main characteristics (i) pure DM phenotype (low overlap features) (ii) necrotizing myositis (severe weakness, high CK level and muscle fiber necrosis) and (iii) higher than expected malignancy rate. 


Disclosure: O. Landon-Cardinal, None; G. Monseau, None; Y. Schoindre, None; A. Rigolet, None; N. Champtiaux, None; B. Hervier, None; A. Masseau, None; E. Hachulla, None; T. Papo, None; B. Terrier, None; A. Meyer, None; J. E. Kahn, None; F. Maurier, None; F. Gaches, None; E. Salort-Campana, None; T. Zenone, None; N. Costedoat-Chalumeau, None; F. Perez, None; M. Samson, None; A. M. Piette, None; G. Moulis, None; S. Audia, None; S. Genot, None; N. Schleinitz, None; G. Lefevre, None; L. Verneuil, None; O. Benveniste, None; Y. Allenbach, None; B. Bienvenu, None.

To cite this abstract in AMA style:

Landon-Cardinal O, Monseau G, Schoindre Y, Rigolet A, Champtiaux N, Hervier B, Masseau A, Hachulla E, Papo T, Terrier B, Meyer A, Kahn JE, Maurier F, Gaches F, Salort-Campana E, Zenone T, Costedoat-Chalumeau N, Perez F, Samson M, Piette AM, Moulis G, Audia S, Genot S, Schleinitz N, Lefevre G, Verneuil L, Benveniste O, Allenbach Y, Bienvenu B. Anti-Mi2 Dermatomyositis Revisited: Pure DM Phenotype with Muscle Fiber Necrosis and High Risk of Malignancy [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/anti-mi2-dermatomyositis-revisited-pure-dm-phenotype-with-muscle-fiber-necrosis-and-high-risk-of-malignancy/. Accessed January 24, 2021.
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