Idiopathic pulmonary haemosiderosis
| Idiopathic pulmonary haemosiderosis | |
| ICD-9 | 516.1 |
|---|---|
| OMIM | 178550 |
| DiseasesDB | 29717 |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Idiopathic pulmonary haemosiderosis is a condition affecting the lungs which results in bleeding from tiny alveolar capillaries.
Idiopathic pulmonary haemosiderosis 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 celluar respiration to occur and for life to continue.
Other names
- Idiopathic pulmonary hemosiderosis
- IPH
- Hemosiderin accumulation
- Pulmonary hemosiderosis (also spelled Pulmonary haemosiderosis)
- Essential brown induration of lung
- Brown induration of lung
- Ceelen-Gellerstedt syndrome
Related or similar 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
Etiology
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-GMB antibodies.
- PH2 involves PH with immune complex disease such as systemic lupus erythematosus, SLE.
- PH3 involves no demonstrable immune system involvement.
Prevalence
One study indicated an annual incidence of 0.24 case per million.[1]
Survival
Death may occur rapidly with acute, massive pulmonary bleeding or over longer periods as the result of continued pulmonary failure and left heart failure.
See also
References
External links
Template:Respiratory pathology