8C81

ICD-11 MMS 2026-01

Autoimmune myopathy

Description

Autoimmune myopathy is a subgroup of idiopathic inflammatory myopathies, which, despite diverse causes, have the common histopathological features of myocyte necrosis without significant inflammation. Patients present with a subacute severe symmetrical proximal myopathy, associated with a markedly elevated creatine kinase level. These are most likely immune-mediated, as they respond to immunotherapy. It is often accompanied by statin therapy, connective tissue diseases, cancer, and autoantibodies specific for signal recognition particle (SRP) or 3-hydroxy-3-methylglutaryl–coenzyme A reductase (HMGCR).

Index terms

  • Autoimmune myopathy

Excludes

  • http://id.who.int/icd/entity/1425591047 – Myasthenia gravis or certain specified neuromuscular junction disorders
  • http://id.who.int/icd/entity/833816211 – Primary disorders of muscles

Exclusions from above levels

  • FB32.2 – Ischaemic infarction of muscle
  • LD26.41 – Arthrogryposis multiplex congenita
  • 05 – Endocrine, nutritional or metabolic diseases
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  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 19 – Certain conditions originating in the perinatal period
  • 22 – Injury, poisoning or certain other consequences of external causes

Sibling codes

  • 8C80 – Drug-induced myopathy
  • 8C82 – Myopathy in certain specified infectious or parasitic disease
  • 8C83 – Myopathy in certain specified endocrine disease
  • 8C84 – Secondary rhabdomyolysis
  • 8C8Y – Other specified secondary myopathies
  • 8C8Z – Secondary myopathies, unspecified

Foundation entity

Autoimmune myopathy – http://id.who.int/icd/entity/1227790808

Allowed postcoordination axes

Part of: 08: Diseases of the nervous system