Familial inflammatory inclusion body myositis.

Abstract:

OBJECTIVE:To compare familial inflammatory inclusion body myositis (IBM) with hereditary inclusion body myopathies and sporadic IBM. PATIENTS AND METHODS:Clinical, biological, MRI, and histological data were analysed in two siblings with inflammatory IBM and compared with those of patients with sporadic and hereditary IBM. RESULTS:Both patients had a clinical phenotype of sporadic IBM, which differs from hereditary myopathies because of late age of onset--respectively 65 and 66 years, and different pattern of muscular involvement--asymmetric, mainly distal but also involving quadriceps. MRI showed selective fatty infiltration and oedema in the extensor compartment of thigh muscles. The diagnosis of IBM was confirmed by muscle biopsy, showing muscle fibres containing numerous rimmed vacuoles, a characteristic shared by all types of IBM. In contrast with hereditary IBM, histological analysis also showed inflammatory mononuclear infiltrate invading non-necrotic fibres, ragged red and oxidase c negative fibres, and positive Congo red staining. Moreover, HLA class II typing disclosed DR beta 1 0301 haplotype, which is significantly related to sporadic but not to hereditary IBM. With steroid treatment and monthly intravenous immunoglobulins, the disease was stabilised in both patients at protracted follow up. CONCLUSION:Sporadic and familial inflammatory IBM share the same clinical, biological, MRI, and histological features.

journal_name

Ann Rheum Dis

authors

Ranque-Francois B,Maisonobe T,Dion E,Piette JC,Chauveheid MP,Amoura Z,Papo T

doi

10.1136/ard.2004.025494

subject

Has Abstract

pub_date

2005-04-01 00:00:00

pages

634-7

issue

4

eissn

0003-4967

issn

1468-2060

pii

64/4/634

journal_volume

64

pub_type

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