4A41.20 Inflammatory inclusion body myositis
International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01
Inclusion body myositis (IBM) is the most common idiopathic inflammatory myopathy after age 50. It typically presents with chronic insidious proximal leg and/or distal arm asymmetric muscle weakness leading to recurrent falls and loss of dexterity. Creatine kinase is up to 15 times elevated in IBM and needle electromyography mostly shows a chronic irritative myopathy. Muscle histopathology demonstrates endomysial inflammatory exudates surrounding and invading non-necrotic muscle fibres often times accompanied by rimmed vacuoles and protein deposits. Despite inflammatory muscle pathology, it is likely that IBM has a prominent degenerative component as supported by refractoriness to immunosuppressive therapy.
exclusions
synonyms
- Inflammatory inclusion body myositis
- Inflammatory IBM - [inflammatory inclusion body myositis]
- Sporadic inclusion body myositis
- Hereditary inclusion body myositis
- UPD-GlcNac epimerase/kinase deficiency
- Familial inclusion body myositis
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