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| {{Infobox disease |
| | #REDIRECT [[Hamman-Rich syndrome]] |
| Name = Hamman–Rich syndrome |
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| ICD10 = {{ICD10|J|84|1|j|80}} |
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| ICD9 = {{ICD9|516.3}} |
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| ICDO = |
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| Image = Hyaline membranes - very high mag.jpg |
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| Caption = [[Micrograph]] of [[diffuse alveolar damage]], the histologic correlate of acute interstitial pneumonitis. [[H&E stain]]. |
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| OMIM = 178500 |
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| MedlinePlus = |
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| eMedicineSubj = |
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| eMedicineTopic = |
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| DiseasesDB = 4815 |
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| MeshID = D011658 |
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| }}
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| __NOTOC__
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| {{CMG}}
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| {{SK}} Acute interstitial pneumonia, Hamman–Rich syndrome
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| ==Overview==
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| '''Acute interstitial pneumonitis''' (also known as '''acute interstitial pneumonia''' or '''Hamman–Rich syndrome''') is a rare, severe [[lung]] disease that usually affects otherwise healthy individuals. There is no known cause or cure.
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| Acute interstitial pneumonitis is often categorized as both an [[interstitial lung disease]] and a form of [[acute respiratory distress syndrome]] (ARDS) but it is distinguished from the ''chronic'' forms of interstitial pneumonia such as [[idiopathic pulmonary fibrosis]].<ref>{{cite journal |author=Hamman L., Rich A.R. |title=Acute diffuse interstitial fibrosis of the lungs |journal=Bull. Johns Hopkins Hosp. |volume=74 |pages=177–212 |year=1944 }}</ref>
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| ==Symptoms==
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| The most common symptoms of acute interstitial pneumonitis are [[highly productive cough with expectoration of thick mucus]], [[fever]], and [[shortness of breath|difficulties breathing]]. These often occur over a period of one to two weeks before medical attention is sought. The presence of fluid means the person experiences a feeling similar to 'drowning'. Difficulties breathing can quickly progress to an inability to breathe without support ([[respiratory failure]]).
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| Acute interstitial pneumonitis typically progresses rapidly, with hospitalization and mechanical ventilation often required only days to weeks after initial symptoms of [[cough]], [[fever]], and [[shortness of breath|difficulties breathing]] develop.
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| ==Diagnosis==
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| Rapid progression from initial symptoms to respiratory failure is a key feature. An x-ray that shows ARDS is necessary for diagnosis (fluid in the small air sacs ([[alveoli]]) in both lungs). In addition, a [[biopsy]] of the lung that shows organizing [[diffuse alveolar damage]] is required for diagnosis. Other diagnostic tests are useful in excluding other similar conditions, but history, x-ray, and biopsy are essential. These other tests may include basic blood work, blood cultures, and [[bronchoalveolar lavage]].
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| The clinical picture is similar to ARDS, but AIP differs from ARDS in that the cause for AIP is not known.
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| ==Treatment==
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| Treatment 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. The only treatment that has met with success to date is a [[lung transplant]].
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| ==Epidemiology==
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| Acute interstitial pneumonitis occurs most frequently among people older than forty years old. It affects men and women equally. There are no known risk factors; in particular, [[tobacco smoking|smoking]] is not associated with increased risk.
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| ==Prognosis==
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| Sixty percent of people with acute interstitial pneumonitis will die in the first six months of illness.<ref>{{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}}</ref> The median survival is 1½ months.
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| However, most people who have one episode do not have a second. People who survive often recover lung function completely.
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| ==History==
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| Acute interstitial pneumonitis was first described in 1935 by [[Louis Hamman]] and [[Arnold Rice Rich|Arnold Rich]], and given the name Hamman–Rich syndrome.<ref>{{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 | year=1935 }}</ref>
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| ==References==
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| {{Reflist|2}}
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| ==External links==
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| * {{WhoNamedIt|synd|3010}}
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| * {{Chorus|00181}}
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| * {{GPnotebook|1241907254}}
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| {{Respiratory pathology}}
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| [[Category:Respiratory diseases principally affecting the interstitium]]
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