Idiopathic pulmonary hemosiderosis
The etiology is poorly understood, but it is thought to result from immune dysregulation. IPH is very rare with an unknown prevalence. Most cases are described in children (80%) or young adults; however, patients of all ages have been diagnosed with IPH. Increased incidence among related individuals is suggestive of either environmental or genetic risk factors. To date, no known environmental or inheritable factor has been identified.
Although the body is able to reabsorb most of the blood from the lungs, a large amount of iron is often left behind. Over time, this iron can lead to pulmonary fibrosis and chronic lung disease. Severe anemia can also occur.
J84.03 – Idiopathic pulmonary hemosiderosis
40527005 – Idiopathic Pulmonary Hemosiderosis
- Infectious – pneumonia (bacterial, viral, or fungal), leptospirosis
- Rheumatic – systemic lupus erythematosus, antiphospholipid antibody syndrome, granulomatosis with polyangiitis, microscopic polyangiitis, mixed cryoglobulinemia, antiglomerular basement membrane antibody disease
- Cystic fibrosis
- Thoracic endometriosis
- Inhalation exposures
- Pulmonary alveolar hemorrhage
- Pulmonary embolism with pulmonary infarction
Last Updated: 05/17/2019