Familial inflammatory inclusion body myositis
نویسندگان
چکیده
منابع مشابه
Familial inflammatory inclusion body myositis.
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, whic...
متن کاملInclusion-body myositis
Inclusion-body myositis is the most frequent and disabling myopathy seen in patients over 50 years of age. The distinct clinical features that lead to correct diagnosis and inclusion-body myositis mimics are highlighted. Inclusionbody myositis has a complex pathogenesis in which autoimmune and inflammatory features coexist with elements of degeneration and abundant accumulations of various stre...
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Inclusion body myositis is an insidious, slowly progressive myopathy of middle-aged and older individuals. Because of these characteristics, diagnosis is often delayed. Affected muscle is marked by the presence of rimmed vacuoles, inclusions, and an inflammatory infiltrate largely made up of CD8 T lymphocytes and macrophages. The inclusions contain beta-amyloid and phosphorylated tau protein, a...
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The histochemical and ultrastructural study of muscle biopsies of two patients with a chronic muscle weakness and wasting showed particular changes in muscle fibers: (1) peripheral lined vacuoles, containing whorls of membranes and cytoplasmic debris; (2) collections of intranuclear and intrasarcoplasmic tubular filaments (16-18 nm in external diameter and 6.5 nm in inner diameter). These chang...
متن کاملInclusion body myositis: update.
PURPOSE OF REVIEW To examine new developments in sporadic inclusion body myositis (IBM), including updated clinical and prognostic factors, novel autoantibody associations, unique histopathologic findings, proposed new clinical diagnostic criteria, and novel therapeutic agents. RECENT FINDINGS IBM is a slowly progressive disease, leading to wheelchair use, on average, 12-20 years after onset ...
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ژورنال
عنوان ژورنال: Annals of the Rheumatic Diseases
سال: 2005
ISSN: 0003-4967
DOI: 10.1136/ard.2004.025494