Idiopathic pulmonary hemosiderosis

What causes idiopathic pulmonary hemosiderosis?

The exact cause of idiopathic pulmonary hemosiderosis is not well-understood. The term "idiopathic" means that there is not a known cause of a disease. Some researchers suspect that the disease is caused by damage to the capillaries (small blood vessels) of the alveoli (small air sacs in the lungs). It is not known exactly what causes the capillaries to become damaged, but it could be due to an autoimmune response.

When the capillaries of the alveoli are damaged, blood may collect in the alveoli. While the body can expel most of the collection of blood in the lungs, the frequent bleeds leave behind deposits of iron in storage complexes known as hemosiderin. This accumulation of iron leads to the development of anemia because the blood does not have enough iron. Additionally, the deposits can cause damage to the lungs (pulmonary fibrosis).

Last updated on 05-01-20

How is idiopathic pulmonary hemosiderosis diagnosed?

Idiopathic pulmonary hemosiderosis is typically diagnosed by a combination of laboratory tests, imaging, and sometimes a lung biopsy. Diagnosis of the disease is based on ruling out other possible causes of the symptoms, including other types of pulmonary hemosiderosis. The diagnosis of idiopathic pulmonary hemosiderosis may include procedures such as:

Last updated on 05-01-20

Is idiopathic pulmonary hemosiderosis inherited?

There is not a single gene that is known to cause idiopathic pulmonary hemosiderosis. In most cases, there are not multiple affected individuals in a family. However, more than one family member having idiopathic pulmonary hemosiderosis has been reported in some families. In some cases, people who have idiopathic pulmonary hemosiderosis are reported to have family members with other types of autoimmune diseases. Most autoimmune diseases are thought to be caused by a combination of genetic and environmental factors.

Last updated on 05-01-20

What is the prognosis for idiopathic pulmonary hemosiderosis?

The clinical course of idiopathic pulmonary hemosiderosis varies widely, and it is hard to predict how long symptoms will last. Treatment of the disease may help to alleviate symptoms. Potential factors that could influence the long-term outlook include:

  • Gender: females tend to have a better prognosis then men
  • Age at diagnosis: individuals who were older when they were diagnosed may have a better prognosis

In general, the severity of the disease at the original diagnosis does not necessarily correlate with the time associated with survival. Some people who have idiopathic pulmonary hemosiderosis eventually achieve complete remission. Adults who have the disease are likely to have a better prognosis in terms of the severity of symptoms and the survival time. Adults may also have a better response to treatment, especially corticosteroids. Potential complications of the disease that may be life-threatening include acute massive hemorrhage, progressive pulmonary insufficiency, and right heart failure.

Last updated on 05-01-20

How common is pulmonary hemosiderosis?

Idiopathic pulmonary hemosiderosis is very rare, but exact prevalence numbers are not known. It is estimated that somewhere between 0.24 and 1.26 in one million people are affected by the disease.

Last updated on 05-01-20

How might idiopathic pulmonary hemosiderosis be treated?

The treatment of idiopathic pulmonary hemosiderosis is aimed at managing acute crises and providing long-term therapy. Potential therapies may include:

Some individuals with idiopathic pulmonary hemosiderosis may also have celiac disease. For these individuals, a gluten-free diet is recommended in addition to other therapies.

More detailed information about the treatment of idiopathic pulmonary hemosiderosis can be accessed through Medscape. Click on the following links to learn more about Treatment & Management and Medications.

Last updated on 05-01-20

Name: Pulmonary Fibrosis Foundation 230 East Ohio Street, Suite 340
Chicago, IL, 60611, United States
Phone: +1-312-587-9272 Toll Free: 1-844-825-5733 (844.TalkPFF) Fax : 1-866-587-9158 Email: pcc@pulmonaryfibrosis.org Url: https://www.pulmonaryfibrosis.org
Name: Children's Interstitial Lung Disease Foundation 6539 Harrison Ave #1045
Cincinnati, OH, 45247, United States
Email: info@child-foundation.org Url: http://child-foundation.org/
Name: Idiopathic Pulmonary Hemosiderosis Facebook Group Url: https://www.facebook.com/IPH.Community/?ref=br_rs
Name: Idiopathic Pulmonary Hemosiderosis Support Group Url: https://www.facebook.com/PulmonaryHemosiderosisSupport/?ref=br_rs
Taytard J, Nathan N, de Blic J, Fayon M, Epaud R, Deschildre A, Troussier F, Lubrano M, Chiron R, Reix P, Cros P, Mahloul M, Michon D, Clement A, and Corvol H. New insights into pediatric idiopathic pulmonary hemosiderosis: the French RepsiRare cohort Orphanet Journal of Rare Diseases. 2013; 8. 161. Reference Link Schwarz MI. Idiopathic Pulmonary Hemosiderosis Merck Manual. February 2017; Reference Link Pulmonary Hemosiderosis Online Mendelian Inheritance in Man. February 1, 1996; Reference Link Milman N, King TE, and Hollingsworth H. Idiopathic pulmonary hemosiderosis UpToDate. January 26, 2017; Reference Link Abbdallah Fatma CB, Amel C, Ridha M, Olfa S, Sophia T, Faouzi M, and Ali BK. Idiopathic pulmonary hemosiderosis in adult Respiratory Medicine CME. 2010; 3(4). 238-240. Reference Link Chen XY, Sun JM, and Huang XJ. Idiopathic pulmonary hemosiderosis in adults: review of cases reported in the latest 15 years The Clinical Respiratory Journal. March 14, 2016; Reference Link

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