ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 388

Treating Statin-induced Anti-HMGCR Myopathy with Normal Muscle Strength: A New Window of Opportunity

Alain Meyer1, Yves Troyanov 2, Julie Drouin 3, Josiane Bourre-Tessier 4, Genevieve Oligny-Longpre 5, Océane Landon-Cardinal 6, Baptiste Hervier 7, Sabrina Hoa 8, Anne-Marie Mansour 9, Eric Rich 4, Jean-Richard Goulet 4, Sandra Chartrand 10, Marie Hudson 11, Jessica Nehme 9, Jean-Paul Makhzoum 12, Farah Zarka 9, Sara Hussein 13, Vincent Morin 14, Edith Villeneuve 15, Jean-Pierre Raynauld 16, Marianne Landry 17, Erin O'Ferrall 18, Jose Ferreira 19, Benjamin Ellezam 20, Jason Karamchandani 18, Sandrine Larue 21, Rami Massie 18, Catherine Isabelle 21, isabelle Deschênes 5, Valérie Leclair 22, Helene Couture 23, Ira Targoff 24, Marvin Fritzler 25 and Jean-Luc Senecal 4, 1Centre de Reference des Maladies Autoimmunes Rares, Hôpitaux Universitaires de Strasbourg, Strasbourg, France, 2Division of Rheumatology, Department of Medicine, Hôpital du Sacre-Coeur de Montreal, Montreal, QC, Canada, 3Centre hospitalier regional de Trois-Rivieres, Trois-Rivieres, QC, Canada, 4Centre hospitalier de l'Universite de Montreal, Montreal, QC, Canada, 5Hôpital du Haut-Richelieu, Saint-Jean-sur-Richelieu, QC, Canada, 6Division of Rheumatology, Centre hospitalier de l'Université de Montréal; Department of Medicine, Université de Montréal, Montreal, QC, Canada, 7Hopital Pitie-Salpetriere, Paris, France, 8University of Montreal, Montreal, QC, Canada, 9Hôpital du Sacre-Coeur de Montreal, Montreal, QC, Canada, 10Hôpital Maisonneuve-Rosemont, Montreal, QC, Canada, 11Jewish General Hospital, Lady Davis Institute for Medical Research, and Department of Medicine, McGill University, Montreal, QC, Canada, 12Vasculitis Clinic, Hopital du Sacre-Coeur de Montreal, Montreal, QC, Canada, 13Centre hospitalier regional de Lanaudiere, Saint-Charles-Borromée, QC, Canada, 14Universite Laval, Quebec, QC, Canada, 15Institut de Recherche en Rhumatologie de Montréal, Montreal, QC, Canada, 16Institut de Rhumatologie de Montréal, Montreal, QC, Canada, 17Hôpital LaSalle, Montreal, QC, Canada, 18Montreal Neurological Institute, Montreal, QC, Canada, 19Hopital Maisonneuve-Rosemont, Montreal, QC, Canada, 20Centre hospitalier universitaire Sainte-Justine, Montreal, QC, Canada, 21Hôpital Charles-Le Moyne, Greenfield Park, QC, Canada, 22Division of Rheumatology, Department of Medicine, Jewish General Hospital; Clinical Epidemiology Unit, Department of Medicine, Karolinska Institutet, Montreal, QC, Canada, 23Hôpital Enfant-Jesus, Quebec, QC, Canada, 24University of Oklahoma Health Sciences Center, Oklahoma City, OK, 25Cumming School of Medicine, University of Calgary, Calgary, AB, Canada

Meeting: ACR/ARP Annual Meeting 2019

Keywords:

Session Information

Date: Sunday, November 10, 2019

Title: Muscle Biology, Myositis & Myopathies Poster I

Session Type: Poster Session (Sunday)

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

Background/Purpose: Anti-HMGCR myopathy has recently been defined as a subset of immune-mediated necrotizing myopathies characterized by proximal muscle weakness, elevated CK levels and the presence of anti-HMGCR autoantibodies (aAbs). Statin-induced anti-HMGCR myopathy with elevated serum CK but normal muscle strength has scarcely been reported. Our objective was to describe the natural history of this subset.

Methods: All our patients with elevated serum CK, anti-HMGCR aAbs and statin exposure were studied retrospectively for demographics, myologic features, chronology of events leading to induction as well as maintenance therapy. Early treatment was defined as treatment when muscle strength was still normal, whereas delayed treatment was defined as treatment when proximal weakness was apparent. Remission was defined by serum CK ≤ 500 UI/L, and successful steroid sparing immunosuppressant (SSI) maintenance as remission for 1 year with ≤ 5 mg/day of prednisone. Anti-HMGCR aAbs were detected by ALBIA or ELISA.

Results: Fifty-five patients with statin-induced anti-HMGCR myopathy were identified. Twenty-two patients (40%) presented with normal muscle strength, with median CK of 1501 IU/L (range 500-5613) and median age of 64.7 years. Of these 22 patients, 9 (41%) had early treatment and 13 (59%) had delayed treatment. All patients were treated with at least one SSI, with or without steroids and/or intravenous immunoglobulins (IVIg). All patients achieved remission.

  • Early treatment group (n=9): median time from presentation (defined as first CK elevation) to treatment was 13.4 months (range 2.6-78.4). Median CK at presentation and at treatment initiation were 1501 IU/L (range 617-2132) and 2684 IU/L (554-13339), respectively. Successful steroid-free induction was used in 5 patients (56%), and IVIg induction in 2 patients (22%). Successful SSI monotherapy maintenance was possible in 5 patients (56%), with only one patient still on IVIg and steroids. At last follow-up, all patients had normal strength.
  • Delayed treatment group (n=13): median time from presentation to treatment was 21.6 months (range 7-95). Median CK at presentation and at treatment initiation were 1700 IU/L (range 500-5613) and 6400 IU/L (1556-14098), respectively. Successful steroid-free induction was used in 1 patient (8%), and IVIg induction in 8 patients (62%). Successful SSI monotherapy maintenance was possible in 4 patients (31%) while 6 patients were on maintenance IVIg and 3 (23%) on steroids. At last follow-up, normal strength was present in 9 patients (69%).
  • Thus, steroid-free induction was proposed by the treating physicians and used successfully in 56% of patients in the early treatment group versus only 8% of patients in the delayed treatment group (p=0.023).

Conclusion: In this cohort of statin-induced anti-HMGCR myopathy, 22 patients (40%) presented with normal strength. While successful in all patients, steroid-free induction was proposed in 5 patients (56%) with normal strength, but only once (8%) in patients in whom proximal weakness had ensued (p=0.023). Anti-HMGCR myopathy presenting with normal strength may be an ideal subset for initiating steroid-free induction.

Disclosure: A. Meyer, None; Y. Troyanov, None; J. Drouin, None; J. Bourre-Tessier, None; G. Oligny-Longpre, None; O. Landon-Cardinal, None; B. Hervier, None; S. Hoa, None; A. Mansour, None; E. Rich, None; J. Goulet, None; S. Chartrand, None; M. Hudson, None; J. Nehme, None; J. Makhzoum, None; F. Zarka, None; S. Hussein, None; V. Morin, None; E. Villeneuve, AbbVie Canada, 5, 8, 9, Amgen Canada, 5, BMS Canada, 5, 8, Celgene Canada, 5, Pfizer Canada, 5, 8, Roche Canada, 5, 8, Sanofi-Genzyme Canada, 5, UCB Canada, 5; J. Raynauld, ArthroLab Inc., 5; M. Landry, None; E. O'Ferrall, None; J. Ferreira, None; B. Ellezam, None; J. Karamchandani, None; S. Larue, None; R. Massie, None; C. Isabelle, None; i. Deschênes, None; V. Leclair, None; H. Couture, None; I. Targoff, None; M. Fritzler, Alexion Canada, 7, BioRad, 5, Dr. Fooke Laboratorien GmbH, 5, Euroimmun GmbH, 5, 7, ImmunoConcepts, 7, Inova Diagnostics, 5, 7, 8, Inova Diagnostics Inc. San diego, CA, 5, Inova Dx, Mikrogen GmbH, 5, Werfen International, 5; J. Senecal, None.

To cite this abstract in AMA style:

Meyer A, Troyanov Y, Drouin J, Bourre-Tessier J, Oligny-Longpre G, Landon-Cardinal O, Hervier B, Hoa S, Mansour A, Rich E, Goulet J, Chartrand S, Hudson M, Nehme J, Makhzoum J, Zarka F, Hussein S, Morin V, Villeneuve E, Raynauld J, Landry M, O'Ferrall E, Ferreira J, Ellezam B, Karamchandani J, Larue S, Massie R, Isabelle C, Deschênes i, Leclair V, Couture H, Targoff I, Fritzler M, Senecal J. Treating Statin-induced Anti-HMGCR Myopathy with Normal Muscle Strength: A New Window of Opportunity [abstract]. Arthritis Rheumatol. 2019; 71 (suppl 10). https://acrabstracts.org/abstract/treating-statin-induced-anti-hmgcr-myopathy-with-normal-muscle-strength-a-new-window-of-opportunity/. Accessed .

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