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Madisons Foundation - Moms And Dads In Search Of Needed Support
Sarcoidosis
Wednesday, 16 July 2003
Last Updated Wednesday, 04 February 2004
What
Sarcoidosis is a disease in which small lumps of inflamed cells, or granulomas, form in body organs or tissues. In children, there are two different forms of sarcoidosis. The first is an early-onset childhood sarcoidosis that starts before age 4. The other form occurs in later childhood and more closely resembles the sarcoidosis seen in adults. In this group of children, the lung is the organ that is most often affected. The prognosis for sarcoidosis in children is highly variable depending on the age of onset and the severity of the disease.Who
Sarcoidosis primarily affects adults between 20-40 years of age, but is also present in children. Most children manifest the disease between ages 10-20 with the most common time being 13-15 years old. However, an apparently distinct form of sarcoidosis affects children under age 4. Sarcoidosis occurs in both male and female children with neither sex being disproportionately affected. In the United States, the disease is 2-10 times more common in Hispanic and black children than it is in white children. However, children under age 4 who are affected are more likely to be white. The incidence in the adult population of the U.S. is 30-80 people per 100,000 with African Americans being much more likely to be affected than whites. The frequency in children is unknown but significantly less than adult incidence. Sarcoidosis is present throughout the world and affects all races and ethnic groups.Signs and Symptoms
Signs and symptoms of sarcoidosis vary from one child to the next. A small number of children have granulomas without demonstrating any outward symptoms. The following is a list of signs and symptoms that are associated with sarcoidosis in children but it is important to remember that not all of these apply to every child. Enlarged lymph nodes in the chest (hilar lymph nodes) or other areas throughout the body. Many children have no symptoms at all other than the lymph nodes. Inflammation in the front part of the eye (known as anterior uveitis) that may or may not be accompanied by pain, increased sensitivity to light, or blurry vision. This may ultimately lead to blindness, even if there is no pain or light sensitivity. Granulomas in the lungs with or without cough and shortness of breath. The lungs are the most common site for granuloma formation in children over age 4. Sarcoidosis affecting the lungs can impair lung function by limiting the volume capacity of the lungs (restrictive lung disease) or causing constriction of the airways (obstructive lung disease). Arthritis or other joint pain Fatigue Weight loss Fever Headache Enlarged liver Enlarged spleen Several types of rash that can be found on essentially any area of the body. Bone pain Muscle pain Sarcoidoisis that affects kidney function directly or indirectly by raising calcium levels Enlarged parotid gland (one of the glands that produces saliva) Sarcoidosis that affects the heart, brain, or any other organ is rare, but also possible. Children under 4 years old demonstrate a different form of sarcoidosis that primarily involves a red rash, inflammation of the eye (uveitis), and arthritis. Lung involvement may also occur, but the lung is not the primary target as it is in older children. Other organs may also be affected in these young children.Possible Causes
The cause of sarcoidosis is unknown. It is thought that genetics as well as environmental factors such as toxins or infections might contribute to the development of sarcoidosis. Whatever the cause, the result is an inappropriate immune response. The immune system is the body’s defensive force against invading substances and infections. The immune system helps mediate inflammation, the body’s natural response to injury and germs. In sarcoidosis, something triggers the immune system to instigate the formation of clusters of inflammatory cells, known as granulomas, in body organs and tissues. These granulomatous lumps often resolve on their own over months to years, but they can also lead to scarring and functional impairment of vital organs.Diagnosis
A chest X-ray can demonstrate granulomas in the lungs and enlarged lymph nodes. However, there are disorders other than sarcoidosis that produce similar X-ray results. Blood may be drawn for several tests including an evaluation of calcium levels and a measurement of the level of an enzyme known as ACE. Both ACE and calcium may be elevated in sarcoidosis. The lungs may also be washed with water to quantify immune cells present (known as a bronoalveolar lavage). The cells in the wash help determine whether there is inflammation typical of sarcoidosis. A biopsy (in which a tiny tissue sample is removed) is generally needed to more definitively diagnose sarcoidosis. Biopsies are performed at sites of active sarcoidosis including the lymph nodes, lungs, or liver.Treatment
Sarcoidosis may resolve on its own without treatment. Therefore, some doctors choose not to treat milder cases of sarcoidosis. Other children receive prednisone (corticosteroid) pills, which reduce inflammation. Length of treatment can vary from months to years and may need to be reinitiated after previous cessation. The variability in the duration of treatment depends on patient response and the severity of the disease. Treatment cannot cure sarcoidosis, but it usually reduces symptoms and can lessen complications in certain organs. If there is eye disease, this can often be treated with steroid drops, which is useful if the eye is the only place significantly affected.Prognosis
Because sarcoidosis occurs infrequently in children, data regarding prognosis is scarce and therefore often speculative. However, it is known that after a period of months to years, sarcoidosis may simply resolve on its own. In a study related to lung function, about 30-40 percent of patients had symptoms of lung sarcoidosis for many years. However, for most children, sarcoidosis symptoms lessen over time. Corticosteroids may help prevent serious complications in organs including the eye, but have been less effective in preventing scarring in the lungs. Permanent lung or other organ damage may occur, but fatal complications are rare. In children under 4 who have early-onset sarcoidosis, the prognosis may be less positive. These children are more prone to a longer disease course with the potential for serious complications including blindness. It is imperative that these children be closely monitored by an ophthalmologist and other specialists. Fortunately, much research on sarcoidosis is being carried out with the hope for better therapy in the future.Connect with other parents
In the spirit of community and support, Madisons Foundation offers the unique service of connecting parents of children with rare diseases. If you would like to be connected to other parents of children with this disease, please fill out this brief form.Weblinks
Sarcoidosis Family Foundation
A nice link with information about the disease as well as links to support networks, pain management issues and other useful sites.
National Heart, Lung, and Blood Institute: “Sarcoidosis”
A good description of Sarcoidosis.
National Sarcoidosis Resource Center
A great website with information about the disease as well as links to ongoing clinical trials and many other useful resources.
Sarcoidosis Worldwide Support Group
A well organized website with information about support groups around the world as well as links to other useful resources.