Hamman-Rich syndrome

DiseaseDisorder infobox
Name = HR syndrome
ICD10 = ICD10|J|84|1|j|80
ICD9 = ICD9|516.3
ICDO =


Caption =
OMIM = 178500
MedlinePlus =
eMedicineSubj =
eMedicineTopic =
DiseasesDB = 4815
MeshID = D011658

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.

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. [cite journal | last=Bouros | first=D | coauthors=Nicholson AC, Polychronopoulos V, du Bois RM | title=Acute interstitial pneumonia |journal=Eur. Respir. J. |volume=15 |issue=2 |pages=412–8 |year=2000 |pmid=10706513 |doi=10.1034/j.1399-3003.2000.15b31.x |url=http://erj.ersjournals.com/cgi/pmidlookup?view=long&pmid=10706513] The mean survival is 1½ months.

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. [cite journal | last=Hamman | first=L | coauthors=Rich AR | title=Fulminating diffuse interstitial fibrosis of the lungs | journal=Transactions of the American Clinical and Climatological Association | volume=51 | pages=154–163 | publisher=European Respiratory Society | date=1935 ]

References

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