4A44.4

ICD-11 MMS 2026-01

Polyarteritis nodosa

Description

Polyarteritis nodosa is an immunologically mediated systemic necrotising vasculitis affecting medium-sized vessels. In a few cases, the disease appears after viral infection but in the majority of cases there is no known triggering event. The clinical manifestations involve numerous organs and lead to a general alteration in the health status including rapid weight loss, paralysis of the peripheral nerves, renal disease, and digestive problems such as haemorrhages, perforation, appendicitis and pancreatitis. Arthralgia is almost always present and myalgia occurs in half of patients. Cardiac and cerebral anomalies (cephalalgia) are also reported, as well as ocular and genital (orchitis) manifestations.

Index terms

  • Polyarteritis nodosa
  • Kussmaul disease
  • PAN - [polyarteritis nodosa]
Show allHide
  • arteritis nodosa
  • panangiitis
  • panarteritis nodosa
  • periarteritis nodosa
  • polyangiitis
  • Polyneuropathy in polyarteritis nodosa
  • Periarteritis nodosa with necrosis

Exclusions from above levels

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

Sibling codes

  • 4A44.0 – Rhizomelic pseudopolyarthritis
  • 4A44.1 – Aortic arch syndrome
  • 4A44.2 – Giant cell arteritis
  • 4A44.3 – Single organ vasculitis
  • 4A44.5 – Mucocutaneous lymph node syndrome
  • 4A44.6 – Sneddon syndrome
  • 4A44.7 – Primary angiitis of the central nervous system
  • 4A44.8 – Thromboangiitis obliterans
  • 4A44.9 – Immune complex small vessel vasculitis
  • 4A44.A – Antineutrophil cytoplasmic antibody-associated vasculitis
  • 4A44.B – Leukocytoclastic vasculitis
  • 4A44.Y – Other specified vasculitis
  • 4A44.Z – Vasculitis, unspecified

Foundation entity

Polyarteritis nodosa – http://id.who.int/icd/entity/1419332129

Allowed postcoordination axes

Part of: 4A44: Vasculitis › 04: Diseases of the immune system