4A44.1

ICD-11 MMS 2026-01

Aortic arch syndrome

Description

Arteritis, often granulomatous, predominantly affecting the aorta and/or its major branches. Onset usually in patients younger than 50.

Index terms

  • Aortic arch syndrome
  • Martorell 2 syndrome
  • Raeder-Harbitz syndrome
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  • Takayasu aortitis
  • Takayasu arteritis
  • Takayasu disease
  • Takayasu syndrome
  • aortic arch arteritis
  • chronic subclavian-carotid obstruction syndrome
  • idiopathic aortitis
  • idiopathic medial aortopathy and arteriopathy
  • nonspecific aortoarteritis
  • obliterative aortitis
  • obliterative brachiocephalic arteritis syndrome
  • pulseless disease
  • sclerosing aortitis and arteritis
  • subclavian-carotid obstruction syndrome
  • young female aortic arch arteritis syndrome

Exclusions from above levels

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

Sibling codes

  • 4A44.0 – Rhizomelic pseudopolyarthritis
  • 4A44.2 – Giant cell arteritis
  • 4A44.3 – Single organ vasculitis
  • 4A44.4 – Polyarteritis nodosa
  • 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

Aortic arch syndrome – http://id.who.int/icd/entity/1327645131

Allowed postcoordination axes

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