ICD9 = 516.3|
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OMIM = 178500 |
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Hamman-Rich syndrome (also known as acute interstitial pneumonia) is a rare, severe lung disease which usually affects otherwise healthy individuals.
As of 2005, there is no known cause or treatment.
Hamman-Rich syndrome is often categorized as both an interstitial lung diseaseand a form of acute respiratory distress syndrome(ARDS).
- 1 Symptoms
- 2 Diagnosis
- 3 Treatment
- 4 Epidemiology
- 5 Prognosis
- 6 History
- 7 References
- 8 External links
The most common symptoms of Hamman-Rich syndrome are cough, fever, and difficulties breathing. These often occur over a period of one to two weeks before medical attention is sought. Difficulties breathing can quickly progress to an inability to breathe without support (respiratory failure).
Hamman-Rich syndrome typically progresses rapidly, with hospitalization and mechanical ventilation often required only days to weeks after initial symptoms of cough, fever, and difficulties breathingdevelop.
Rapid progression from initial symptoms to respiratory failure is a key feature of Hamman-Rich syndrome. An x-ray which shows ARDS is necessary for diagnosis (fluid in the small air sacs (alveoli) in both lungs). In addition, a biopsyof the lung which shows organizing diffuse alveolar damageis required for diagnosis. Other diagnostic tests are useful excluding other similar condition, but history, x-ray, and biopsy are necessary. These other tests may include basic blood work, blood cultures, and bronchoalveolar lavage.
Treatment of Hamman-Rich syndrome is primarily supportive. Management in an intensive care unitis required and the need for mechanical ventilationis common. Therapy with corticosteroidsis generally attempted, though their usefulness has not been established as of 2005.
Hamman-Rich syndrome occurs most frequently among people older than forty years old. It affects men and women equally. There is no known risk factors; in particular, smokingis not associated with increased risk.
Sixty percent of people with Hamman-Rich syndrome will die in the first six months of illness. However, most people who have one episode do not have a second. Indeed people who survive often recover lung function completely.
Hamman-Rich syndrome was first described in 1935by Louis Hamman and Arnold Rich.
- ^ Hamman, L, Rich, AR. Fulminating diffuse interstitial fibrosis of the lungs. Trans Am Clin Climatol Assoc 1935; 51:154.
- Who Named Itsynd/3010
Categories: Pulmonology| Eponymous diseases
This article is licensed under the GNU Free Documentation License.
It uses material from the http://en.wikipedia.org/wiki/Hamman-Rich+syndrome Wikipedia article Hamman-Rich syndrome.