Hemosiderosis pathophysiology: Difference between revisions

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== Pathophysiology ==
== Pathophysiology ==
The exact pathogenesis of idiopathic pulmonary hemosiderosis (IDH) is not fully understood. However, it is thought that idiopathic pulmonary hemosiderosis is the result of abnormal immune response towards alveolar capillaries.
The exact pathogenesis of idiopathic pulmonary hemosiderosis (IDH) is not fully understood. However, it is thought that idiopathic pulmonary hemosiderosis is the result of abnormal immune response towards alveolar capillaries.
* IDH is more commonly seen in patients with underlying structural defect in the alveolar capillaries.
* IDH is more commonly seen in patients with underlying structural defect in alveolar capillaries.
* Immune response against  
* Immune response against the alveolar basement membrane or alveolar endothelial cell results in rupture and alveolar hemorrhage.
**Over time, alveolar hemorrhage results in breakdown of hemoglobin into heme and globin chains which leads to recruitment of neutrophil and monocyte inflammatory response.
* Patients with prolonged duration of idiopathic pulmonary hemosiderosis have been associated with development of other autoimmune disorders such as celiac disease.
* Patients with prolonged duration of idiopathic pulmonary hemosiderosis have been associated with development of other autoimmune disorders such as celiac disease.
* It is not yet clear but  
* It is not yet clear but  

Revision as of 14:55, 16 March 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Pathophysiology

The exact pathogenesis of idiopathic pulmonary hemosiderosis (IDH) is not fully understood. However, it is thought that idiopathic pulmonary hemosiderosis is the result of abnormal immune response towards alveolar capillaries.

  • IDH is more commonly seen in patients with underlying structural defect in alveolar capillaries.
  • Immune response against the alveolar basement membrane or alveolar endothelial cell results in rupture and alveolar hemorrhage.
    • Over time, alveolar hemorrhage results in breakdown of hemoglobin into heme and globin chains which leads to recruitment of neutrophil and monocyte inflammatory response.
  • Patients with prolonged duration of idiopathic pulmonary hemosiderosis have been associated with development of other autoimmune disorders such as celiac disease.
  • It is not yet clear but


Hemosiderosis is an uncommon or rare condition in which bleeding in the lungs causes additional problems especially a collection of iron (FE) which, in itself causes additional lung damage. Iron is an essential component of a hemoglobin molecule. This molecule is responsible for transporting Oxygen through the blood stream and to the individual cells. Such transport is essential for cellular respiration to occur and for life to continue.


Hemosiderosis 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-GMB antibodies.
  • PH2 involves PH with immune complex disease such as systemic lupus erythematosus, SLE.
  • PH3 involves no demonstrable immune system involvement.

Associated conditions

There are many pulmonary problems that may seem to mimic hemosiderosis but do not necessarily include the deposits of iron into the lung. The deposition of iron in the lungs, occurring in the form of hemosiderin, is the defining characteristic of this illness. These other conditions may occur separately or together with hemosiderosis.

  • Pulmonary Fibrosis
  • Adult Respiratory Distress Syndrome (ARDS)
  • Immune Complex Disease
  • intra-alveolar bleeding

References

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