4A41.21

ICD-11 MMS 2026-01

Noninflammatory inclusion body myopathy

Description

Noninflammatory inclusion body myopathy (IBM) is an idiopathic muscle disorder without inflammatory exudates and expression of class I major histocompatibility complex. Rimmed vacuoles and “IBM-like” filaments without inflammatory cells are described in muscle biopsy.

Index terms

  • Noninflammatory inclusion body myopathy
  • Hereditary inclusion body myopathy
  • HIBM - [hereditary inclusion body myositis]
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  • Desmin-related myopathy with Mallory body-like inclusions
  • Hereditary inclusion body myopathy - joint contractures - ophthalmoplegia
  • Inclusion body myopathy with Paget disease of bone and frontotemporal dementia
  • IBMPFD - [inclusion body myopathy with Paget disease of bone and frontotemporal dementia]
  • X-linked myopathy with excess autophagy
  • XMEA - [x-linked myopathy with excess autophagy]
  • Sporadic inclusion body myopathy

Excludes

  • http://id.who.int/icd/entity/1425591047 – Myasthenia gravis or certain specified neuromuscular junction disorders

Exclusions from above levels

  • 02 – Neoplasms
  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 20 – Developmental anomalies

Sibling codes

  • 4A41.20 – Inflammatory inclusion body myositis
  • 4A41.2Z – Inclusion body myopathy, unspecified

Foundation entity

Noninflammatory inclusion body myopathy – http://id.who.int/icd/entity/404396176

Part of: 4A41.2: Inclusion body myopathy › 4A41: Idiopathic inflammatory myopathy › 04: Diseases of the immune system