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Distal inflammatory myopathy: unusual presentation of polymyositis or new entity?

Distal inflammatory myopathy: unusual presentation of polymyositis or new entity?

Authors :
Dimitri D
Dubourg O
Maisonobe T
Fournier E
Ranque B
Laforêt P
Mussini JM
Pagnoux C
Béhin A
Papo T
Benveniste O
Eymard B
Herson S
Source :
Neuromuscular disorders : NMD [Neuromuscul Disord] 2008 Jun; Vol. 18 (6), pp. 493-500. Date of Electronic Publication: 2008 Jun 04.
Publication Year :
2008

Abstract

New classification of idiopathic inflammatory myopathy (IIM) defined three major entities, polymyositis (PM), dermatomyositis (DM) and sporadic inclusion body myositis (s-IBM). We report the clinical, electrophysiological and pathological characteristics of three patients with a rare form of IIM not fulfilling the diagnostic criteria for any of these three major entities. The three patients presented with a subacute, distal asymmetrical weakness in upper limbs. Muscle biopsy showed an active myositis, with necrosis and regeneration, T cell infiltrates with invasion of non-necrotic fibers, without rimmed vacuoles, and diffuse major histocompatibility complex-I (MHC-I) immunostaining in muscle fibers. All patients responded to immunosuppressive agents. Seven others cases were identified in the literature. It is important to recognize this atypical presentation as it seems to respond to immunosuppressive agents.

Details

Language :
English
ISSN :
0960-8966
Volume :
18
Issue :
6
Database :
MEDLINE
Journal :
Neuromuscular disorders : NMD
Publication Type :
Academic Journal
Accession number :
18534849
Full Text :
https://doi.org/10.1016/j.nmd.2008.04.015