acute interstitial pneumonia

急性間質性肺炎 AIP

WordNet

1. of critical importance and consequence; "an acute (or critical) lack of research funds"
2. having or demonstrating ability to recognize or draw fine distinctions; "an acute observer of politics and politicians"; "incisive comments"; "icy knifelike reasoning"; "as sharp and incisive as the stroke of a fang"; "penetrating insight"; "frequent penetrative observations" (同)discriminating, incisive, keen, knifelike, penetrating, penetrative, piercing, sharp
3. extremely sharp or intense; "acute pain"; "felt acute annoyance"; "intense itching and burning" (同)intense
4. having or experiencing a rapid onset and short but severe course; "acute appendicitis"; "the acute phase of the illness"; "acute patients"
5. of an angle; less than 90 degrees
6. of or relating to interstices
7. respiratory disease characterized by inflammation of the lung parenchyma (excluding the bronchi) with congestion caused by viruses or bacteria or irritants

PrepTutorEJDIC

1. (先の)『鋭い』,とがった / (痛み・感情などが)『激しい』,強い / (知力・感覚などが)『鋭い』,鋭敏な / (事態が)重大な / (病気が)急性の / (音が)高い,鋭い / 鋭角の
2. 肺炎

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2017/06/02 14:55:23」(JST)

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HR syndrome
Micrograph of diffuse alveolar damage, the histologic correlate of acute interstitial pneumonitis. H&E stain.
Classification and external resources
Specialty	pulmonology
ICD-10	J84.1
ICD-9-CM	516.3
OMIM	178500
DiseasesDB	4815
MeSH	D011658

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.

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.

Symptoms

The most common symptoms of acute interstitial pneumonitis are highly productive cough with expectoration of thick mucus, fever, and 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).

Acute interstitial pneumonitis 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. 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. The clinical picture is similar to ARDS, but AIP differs from ARDS in that the cause for AIP is not known.

Treatment

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.

Epidemiology

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, smoking is not associated with increased risk.

Prognosis

Sixty percent of people with acute interstitial pneumonitis will die in the first six months of illness. The median 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

Acute interstitial pneumonitis was first described in 1935 by Louis Hamman and Arnold Rich, and given the name Hamman–Rich syndrome.

External links

• synd/3010 at Who Named It?
• 00181 at CHORUS

UpToDate Contents

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• 1. 急性間質性肺炎（ハマンリッチ症候群） acute interstitial pneumonia hamman rich syndrome
• 2. 特発性間質性肺炎：臨床症状および病理 idiopathic interstitial pneumonias clinical manifestations and pathology
• 3. 幼児および小児における間質性肺疾患分類 classification of interstitial lung disease in infants and children
• 4. 急性呼吸窮迫症候群：成人における臨床的特徴および診断 acute respiratory distress syndrome clinical features and diagnosis in adults
• 5. 特発性器質化肺炎 cryptogenic organizing pneumonia

English Journal

• Usual interstitial pneumonia end-stage features from explants with radiologic and pathological correlations.

• Rabeyrin M1, Thivolet F2, Ferretti GR3, Chalabreysse L2, Jankowski A3, Cottin V4, Pison C5, Cordier JF4, Lantuejoul S6.
• Annals of diagnostic pathology.Ann Diagn Pathol.2015 Aug;19(4):269-76. doi: 10.1016/j.anndiagpath.2015.05.003. Epub 2015 May 13.
• Idiopathic pulmonary fibrosis (IPF) is the most frequent and severe idiopathic interstitial pneumonia, with typical high-resolution computed tomography (HRCT) features and histologic pattern of usual interstitial pneumonia (UIP); its main differential diagnosis is fibrotic nonspecific interstitial p
• PMID 26025258

• Histopathology of the idiopathic interstitial pneumonias (IIP): A review.

• Tabaj GC1, Fernandez CF2, Sabbagh E3, Leslie KO4.
• Respirology (Carlton, Vic.).Respirology.2015 Aug;20(6):873-83. doi: 10.1111/resp.12551. Epub 2015 May 22.
• The 2013 American Thoracic Society/European Respiratory Society consensus classification update of the idiopathic interstitial pneumonias (IIP) included several important modifications to the organization and spectrum of the diseases that were proposed in an earlier multidisciplinary consensus docum
• PMID 25998242

• HRCT of fibrosing lung disease.

• Jacob J1, Hansell DM1,2.
• Respirology (Carlton, Vic.).Respirology.2015 Aug;20(6):859-72. doi: 10.1111/resp.12531. Epub 2015 Apr 21.
• The use of high-resolution computed tomography (HRCT) has brought increased diagnostic discrimination to the evaluation of lung disease, particularly fibrosing lung diseases. Once the presence of a predominantly fibrosing lung disease has been established on evaluation of a HRCT, a stepwise approach
• PMID 25900734

Japanese Journal

• 症例報告 ステロイドパルス療法,エンドトキシン吸着療法およびシクロスポリンAが著効した急性間質性肺炎の一例

• 高野 智圭,熊澤 文雄,野田 彰浩 [他]
• 日大医学雑誌 = Journal of Nihon University Medical Association 71(6), 455-459, 2012-12
• NAID 40019547362

• 脊髄くも膜下麻酔での虫垂切除術後に急性増悪した間質性肺炎の1症例

• 小寺 厚志,上妻 精二,宮崎 直樹 [他]
• 麻酔 61(12), 1347-1351, 2012-12
• NAID 40019512803

Related Pictures

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★リンクテーブル★

リンク元	「急性間質性肺炎」「AIP」「急性型間質性肺炎」
関連記事	「interstitial」「acute」

「急性間質性肺炎」

　　[★]

英

acute interstitial pneumonitis acute interstitial pneumonia AIP

関

特発性間質性肺炎、diffuse alveolar damage DAD

ハンマン-リッチ症候群

特徴

臨床診断名	AIP
病理組織像	DAD
分布	びまん性,均質
時相	均質
間質への細胞浸潤	少ない
胞隔の炎症	なし
線維芽細胞巣	びまん性,間質
肺胞内マクロファージ集積	なし
肺胞腔内線維化	しばしば
顕微鏡的蜂巣肺(肺胞虚説)	なし
硝子膜	高頻度

概念

• 特発性間質性肺炎の一型
• 発熱、乾性咳、呼吸困難をもって急激に発症する特発性間質性肺炎の急性型。
• 急性発症、数日の経過で呼吸不全、数週間～数ヶ月で重篤な転帰
• 病理的にはびまん性肺胞障害 DAD、臨床的には急性呼吸促迫症候群 ARDSを呈する。

原因

• 不明

病理組織所見

• びまん性肺胞障害 DAD

• 蜂巣肺
• 上肺野から下肺野までびまん性に肺胞障害が見られる。
• (急性期)間質浮腫、肺胞への進出、硝子膜形成、(器質化期)線維化

発症様式

• 急性

検査

BALF所見

• 好中球

胸部X線写真

• 両側びまん性浸潤様陰影

進行性に拡大するスリガラス陰影・肺胞性陰影

• 牽引性気管支拡張
• 蜂巣肺所見はない。

症状

• 臨床的には急性呼吸促迫症候群 ARDSの像を呈する。

*ARDSとは

• 以下の条件を満たす病態

1. 急性発症，

2. 胸部X線写真で両側浸潤影，

3. PaO2/FIO2≦200，

4. 左心不全の否定

治療

治療が奏功するのは少数

• ステロイドホルモン
• 免疫抑制薬

予後

• 不良

• 2ヶ月以内に呼吸不全に至る

「AIP」

　　[★]

• 急性間質性肺炎 acute interstitial pneumonia
• 自己免疫性膵炎 autoimmune pancreatitis
• 急性間欠性ポルフィリン症 acute intermittent porphyria
• アポトーシス抑制蛋白質 apoptosis inhibitory protein
• アルドステロン誘導蛋白 aldosterone induced protein

「急性型間質性肺炎」

　　[★]

英

acute interstitial pneumonia

関

びまん性肺胞障害

「interstitial」

　　[★]

• adj.

• 間質性の、間質内の、侵入型の、組織内の、介在の、介在性の、中間部の、間質の

関

intercalary、intermediate、internuncial、interstitial tissue、interstitium、intervene、intervening、interzonal、invasive、stroma、stromal、stromata

　

「acute」

　　[★]

• adj.

• (疾患)急性の、急性型の、急性的な。(形状が)鋭い、鋭角の。(感覚、才知などが)鋭い。明敏な、鋭い眼識のある。

関

acutely、quick、sharp