Inflammatory Myopathies

General information


Inflammatory myopathies are a heterogeneous group of rare diseases that have in common the presence of muscle weakness and a variable increase in serum creatine kinase.

This group of muscle pathologies mainly includes Polymyositis, Dermatomyositis, necrotizing myopathies and inclusion body myositis. Clinically, they may be presented as acute, subacute or chronic with markedly proximal and symmetric weakness, except for the inclusion body myositis, which is asymmetric and distal. The most characteristic histopathological finding is the presence of inflammation in the muscle biopsy, thus being an important test for the definitive diagnosis of all of them.

Although diagnosis by clinics and muscle biopsy can be approached in most cases, the lack of specific biomarkers difficult in some cases the diagnosis, prognosis and the use of more specific drugs.

ONGOING PROJECTS

Research in this area is focused on the study of the physiopathology of inflammatory myopathies to obtain new biomarkers and to develop new more specific therapeutic strategies currently existing.

1) Role of innate immunity in the pathophysiology of dermatomyositis:
Recent studies from our group (J Pathol, 2014) have demonstrated the role of an innate immune receptor, RIG-I, which could keep a local immune response specifically in muscle of patients with dermatomyositis.

The continuation of this line of research focuses on the triggers of impaired innate immunity using in vitro models.

2) Antibodies in inflammatory myopathies:
From sera of patients seek relevant autoantibodies in the pathogenesis of these patients using new technologies and models in vitro. The discovery of antibodies may have important therapeutic implications.

Publications

2020
Suárez-Calvet X, Alonso-Pérez J, Castellví I, Carrasco-Rozas A, Fernández-Simón E, Zamora C, Martínez-Martínez L, Alonso-Jiménez A, Rojas-García R, Turón J, Querol L, de Luna N, Milena-Millan A, Corominas H, Castillo D, Cortés-Vicente E, Illa I, Gallardo E, Díaz-Manera J
Neurol Neuroimmunol Neuroinflamm
2019
Olivier PA, De Paepe B, Aronica E, Berfelo F, Colman R, Amato A, Dimitri D, Gallardo E, Gherardi R, Goebel HH, Hilton-Jones D, Hofer M, Holton J, Schrøder HD, Selcen D, Stenzel W, de Visser M, De Bleecker JL
Neurology
2018
Piñol-Jurado P, Suárez-Calvet X, Fernández-Simón E, Gallardo E, de la Oliva N, Martínez-Muriana A, Gómez-Gálvez P, Escudero LM, Pérez-Peiró M, Wollin L, de Luna N, Navarro X, Illa I, Díaz-Manera J
Cell Death Dis
Ladislau L, Suárez-Calvet X, Toquet S, Landon-Cardinal O, Amelin D, Depp M, Rodero MP, Hathazi D, Duffy D, Bondet V, Preusse C, Bienvenu B, Rozenberg F, Roos A, Benjamim CF, Gallardo E, Illa I, Mouly V, Stenzel W, Butler-Browne G, Benveniste O, Allenbach Y
Brain
2017
De Luna N, Suárez-Calvet X, Lleixà C, Diaz-Manera J, Olivé M, Illa I, Gallardo E
Sci Rep
Piñol-Jurado P, Gallardo E, de Luna N, Suárez-Calvet X, Sánchez-Riera C, Fernández-Simón E, Gomis C, Illa I, Díaz-Manera J
Am J Pathol
Suárez-Calvet X, Gallardo E, Pinal-Fernandez I, De Luna N, Lleixà C, Díaz-Manera J, Rojas-García R, Castellví I, Martínez MA, Grau JM, Selva-O'Callaghan A, Illa I
Arthritis Res Ther
2016
Allenbach Y, Leroux G, Suárez-Calvet X, Preusse C, Gallardo E, Hervier B, Rigolet A, Hie M, Pehl D, Limal N, Hufnagl P, Zerbe N, Meyer A, Aouizerate J, Uzunhan Y, Maisonobe T, Goebel HH, Benveniste O, Stenzel W, French Myositis Network
Am J Pathol
2015
Loureiro Amigo J, Gallardo E, Gallano P, Grau-Junyent JM
Med Clin (Barc)
2014
Suárez-Calvet X, Gallardo E, Nogales-Gadea G, Querol L, Navas M, Díaz-Manera J, Rojas-Garcia R, Illa I
J Pathol
2006
Selva-O'Callaghan A, Labrador-Horrillo M, Gallardo E, Herruzo A, Grau-Junyent JM, Vilardell-Tarres M
Neuromuscul Disord
2003
Bassez G, Authier FJ, Lechapt-Zalcman E, Delfau-Larue MH, Plonquet A, Coquet M, Illa I, Gherardi RK
J Neuropathol Exp Neurol
2001
Gallardo E, Rojas-García R, de Luna N, Pou A, Brown RH Jr, Illa I
Neurology
1999
Fuster M, Negredo E, Cadafalch J, Domingo P, Illa I, Clavé P
Arch Intern Med
1993
Dalakas MC, Illa I, Dambrosia JM, Soueidan SA, Stein DP, Otero C, Dinsmore ST, McCrosky S
N Engl J Med
1990
Dalakas MC, Illa I, Pezeshkpour GH, Laukaitis JP, Cohen B, Griffin JL
N Engl J Med

Ongoing Projects

Influence of immune and non-immune factors in the pathogenesis of inflammatory myopathies
Estado: Activo
Duración: 2016-2018
Investigador Principal: Eduard Gallardo Vigo (Hospital de Sant Pau)
Agencia Financiadora: Instituto de salud Carlos III
Nodal and paranodal cell adhesión molecules as immune targets in inflammatory neuromuscular diseases
Estado: Activo
Duración: 2014-2017
Investigador Principal: Isabel Illa Sendra (nacional)
Agencia Financiadora: AFM- TÉLÉTHON
Role of RLR immunity in dermatomyositis
Estado: Finalizado
Duración: 2013-2015
Investigador Principal: Isabel Illa Sendra (nacional)
Agencia Financiadora: The Myositis Association
Danger signals promoting innate immunity in dermatomyositis
Estado: Finalizado
Duración: 2015-2016
Investigador Principal: Isabel Illa (Hospital de Sant Pau)
Agencia Financiadora: AFM- TÉLÉTHON

Clínical Trials

CL2-78989-010: Randomized, double-blind, placebo-controlled, proof-of-concept, efficacy study of 60 mg of subcutaneous gevokizumab administered every 4 weeks for 24 weeks in the treatment of patients with polymyositis or dermatomyositis
Estado: Finalizado
Ensayo Clínico