C Bergua1,2, H Chiavelli1,2, J P Simon3, O Boyer1,2,4, F Jouen1,4, W Stenzel5, J Martinet6,7,8. 1. Normandie University, IRIB, Rouen, France. 2. INSERM, U905, Rouen, France. 3. Department of Neuropathology, Caen University Hospital, Caen, France. 4. Department of Immunology, Rouen University Hospital, 22 bd Gambetta, 76000, Rouen, France. 5. Department of Neuropathology, Charité - Universitätsmedizin, Berlin, Germany. 6. Normandie University, IRIB, Rouen, France. Jeremie.Martinet@chu-rouen.fr. 7. INSERM, U905, Rouen, France. Jeremie.Martinet@chu-rouen.fr. 8. Department of Immunology, Rouen University Hospital, 22 bd Gambetta, 76000, Rouen, France. Jeremie.Martinet@chu-rouen.fr.
Abstract
BACKGROUND: Immune-mediated necrotizing myopathy (IMNM) is a newly identified subgroup of idiopathic inflammatory myopathies. It is defined as a rare and severe disease, with symmetrical and proximal muscle weakness and a characteristic histology. An autoimmune aspect of IMNM is suggested by its association with autoantibodies directed against signal recognition particle (SRP) and 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) in the majority of patients. Statin use is strongly associated with anti-HMGCR-positive IMNM. The pathophysiological mechanisms of this disease are still poorly understood, and as a result, no therapeutic strategy has been validated to date. OBJECTIVE: The aim of this article is to provide an overview of the current knowledge about epidemiology, clinical features, and pathophysiology of IMNM, as well as treatment strategies. RESULTS AND CONCLUSION: IMNM is a subject of widespread interest, with quick and meaningful advances being made. In recent years, huge progress has been made in terms of diagnosis and patient management. However, the understanding of pathophysiological mechanisms and treatment strategies still requires further investigation.
BACKGROUND: Immune-mediated necrotizing myopathy (IMNM) is a newly identified subgroup of idiopathic inflammatory myopathies. It is defined as a rare and severe disease, with symmetrical and proximal muscle weakness and a characteristic histology. An autoimmune aspect of IMNM is suggested by its association with autoantibodies directed against signal recognition particle (SRP) and 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) in the majority of patients. Statin use is strongly associated with anti-HMGCR-positive IMNM. The pathophysiological mechanisms of this disease are still poorly understood, and as a result, no therapeutic strategy has been validated to date. OBJECTIVE: The aim of this article is to provide an overview of the current knowledge about epidemiology, clinical features, and pathophysiology of IMNM, as well as treatment strategies. RESULTS AND CONCLUSION: IMNM is a subject of widespread interest, with quick and meaningful advances being made. In recent years, huge progress has been made in terms of diagnosis and patient management. However, the understanding of pathophysiological mechanisms and treatment strategies still requires further investigation.
Authors: Jessica E Hoogendijk; Anthony A Amato; Bryan R Lecky; Ernest H Choy; Ingrid E Lundberg; Michael R Rose; Jiri Vencovsky; Marianne de Visser; Richard A Hughes Journal: Neuromuscul Disord Date: 2004-05 Impact factor: 4.296
Authors: Werner Stenzel; Corinna Preuße; Yves Allenbach; Debora Pehl; Reimar Junckerstorff; Frank L Heppner; Kay Nolte; Eleonora Aronica; Veronika Kana; Elisabeth Rushing; Udo Schneider; Kristl G Claeys; Olivier Benveniste; Joachim Weis; Hans H Goebel Journal: Neurology Date: 2015-03-06 Impact factor: 9.910
Authors: Jessie L Werner; Lisa Christopher-Stine; Sharon R Ghazarian; Katherine S Pak; Jordan E Kus; Natalie R Daya; Thomas E Lloyd; Andrew L Mammen Journal: Arthritis Rheum Date: 2012-12
Authors: Andrew L Mammen; Katherine Pak; Emma K Williams; Diane Brisson; Joe Coresh; Elizabeth Selvin; Daniel Gaudet Journal: Arthritis Care Res (Hoboken) Date: 2012-02 Impact factor: 4.794