CB07.1

ICD-11 MMS 2026-01

Sporadic lymphangioleiomyomatosis

Description

This is a rare lung disease that results in a proliferation of disorderly smooth muscle growth (leiomyoma) throughout the lungs, in the bronchioles, alveolar septa, perivascular spaces, and lymphatics, resulting in the obstruction of small airways (leading to pulmonary cyst formation and pneumothorax) and lymphatics (leading to chylous pleural effusion). LAM occurs in a sporadic form, which affects only females, usually of childbearing age; LAM also occurs in patients who have tuberous sclerosis.

Index terms

  • Sporadic lymphangioleiomyomatosis

Exclusions from above levels

  • 01 – Certain infectious or parasitic diseases
  • 05 – Endocrine, nutritional or metabolic diseases
  • 18 – Complications of pregnancy, childbirth and the puerperium
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  • 19 – Certain conditions originating in the perinatal period
  • 20 – Congenital malformations, deformations and chromosomal abnormalities
  • 22 – Injury, poisoning or certain other consequences of external causes

Sibling codes

  • CB07.0 – Lymphangioleiomyomatosis associated with tuberous sclerosis complex
  • CB07.Y – Other specified lymphangioleiomyomatosis
  • CB07.Z – Lymphangioleiomyomatosis, unspecified

Foundation entity

Sporadic lymphangioleiomyomatosis – http://id.who.int/icd/entity/1251780231

Part of: CB07: Lymphangioleiomyomatosis › 12: Diseases of the respiratory system