Idiopathic Pulmonary Haemosiderosis - Pathophysiology

Pathophysiology

Being idiopathic, IPH by definition has an unknown cause. It is thought to be an immune-mediated disease. The lung bleeding causes accumulation of iron, which in itself causes additional lung damage. Meanwhile, there is insufficient iron for inclusion into the haemoglobin molecules inside red blood cells which carry oxygen to body tissues for cellular respiration.

Idiopathic pulmonary haemosiderosis can occur either as a primary lung disorder or as the sequela to other pulmonary, cardiovascular or immune system disorder.

  • PH1 involves PH with circulating anti-GBM antibodies.
  • PH2 involves PH with immune complex disease such as systemic lupus erythematosus, SLE.
  • PH3 involves no demonstrable immune system involvement.

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