Hamman-Rich syndrome
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Synonyms and keywords: Acute interstitial pneumonia; HR syndrome
Overview=
Hamman-Rich syndrome is a rare, severe lung disease which usually affects otherwise healthy individuals.
HR syndrome is often categorized as both an interstitial lung disease and a form of acute respiratory distress syndrome (ARDS).
Symptoms
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 breathing develop.
Diagnosis
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 biopsy of the lung which shows organizing diffuse alveolar damage is 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
Treatment of HR syndrome is primarily supportive. Management in an intensive care unit is required and the need for mechanical ventilation is common. Therapy with corticosteroids is generally attempted, though their usefulness has not been established as of 2005.
Epidemiology
HR syndrome occurs most frequently among people older than forty years old. It affects men and women equally. There are no known risk factors; in particular, smoking is not associated with increased risk.
Prognosis
Sixty percent of people with HR syndrome will die in the first six months of illness. However, most people who have one episode do not have a second. People who survive often recover lung function completely.
History
HR syndrome was first described in 1935 by Louis Hamman and Arnold Rich.
References
- ^ HR, AR. Fulminating diffuse interstitial fibrosis of the lungs. Trans Am Clin Climatol Assoc 1935; 51:154.
External links
Template:Respiratory pathology