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Polymyositis

Disease definition

A rare idiopathic inflammatory myopathy (IIM) historically characterized by symmetric proximal muscle weakness, elevated muscle enzymes (creatine kinase), myopathic findings on electromyography, and muscle biopsy showing endomyial infiltration composed mainly of macrophages and lymphocytes. The features are non-specific, thus the disease should be distinguished from similar entities with specific clinical, immunological, histological features, notably dermatomyositis, immune-mediated necrotizing myopathy, anti-synthetase syndrome, inclusion body myositis, and myositis associated with other connective tissue disorder.

ORPHA:732

Classification level: Disorder
  • Synonym(s): -
  • Prevalence: 1-9 / 100 000
  • Inheritance: Not applicable 
  • Age of onset: Adult, Elderly
  • ICD-10: M33.2
  • ICD-11: 4A41.1
  • OMIM: -
  • UMLS: C0085655
  • MeSH: D017285
  • GARD: 7425
  • MedDRA: 10036102

  A summary on this disease is available in Deutsch (2003) Italiano (2003) Español (2022) Français (2022) Nederlands (2022) Suomi (2014, pdf) Polski ()

Detailed information

General public

Guidelines

ERN produced/endorsed by ERN(s)   FSMR produced/endorsed by FSMR(s)

Additional information

Further information on this disease

Patient-centred resources for this disease

Research activities on this disease

Specialised Social Services

Newborn screening

The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.