bronchiolitis obliterans organizing pneumonia

閉塞性細気管支炎性器質化肺炎

WordNet

1. respiratory disease characterized by inflammation of the lung parenchyma (excluding the bronchi) with congestion caused by viruses or bacteria or irritants
2. inflammation of the membranes lining the bronchioles

PrepTutorEJDIC

1. 肺炎

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/07/17 17:00:25」(JST)

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Not to be confused with Bronchiolitis obliterans.

Bronchiolitis obliterans organizing pneumonia
Classification and external resources
Micrograph showing a Masson body (off center left/bottom of the image - pale circular and paucicellular), as may be seen in bronchiolitis obliterans organizing pneumonia. The Masson body plugs the airway. The artery associated with the obliterated airway is also seen (far left of the image). H&E stain.
ICD-10	J84.8
ICD-9	516.8
DiseasesDB	31684
eMedicine	radio/117
MeSH	D018549

Bronchiolitis obliterans organizing pneumonia (BOOP) is a non-infectious pneumonia; specifically, an inflammation of the bronchioles (bronchiolitis) and surrounding tissue in the lungs. It is often a complication of an existing chronic inflammatory disease such as rheumatoid arthritis, or it can be a side effect of certain medications such as amiodarone. BOOP was first described by Gary Epler in 1985.

Some authors have recommended the use of an alternate name, cryptogenic organizing pneumonia (COP), to reduce confusion with bronchiolitis obliterans, a distinct and unrelated disease.

The clinical features and radiological imaging resemble infectious pneumonia. However, diagnosis is suspected after there is no response to multiple antibiotics, and blood and sputum cultures are negative for organisms.

Terminology

"Organizing" refers to unresolved pneumonia (in which the alveolar exudate persists and eventually undergoes fibrosis) in which fibrous tissue forms in the alveoli. The phase of resolution and/or remodeling following bacterial infections is commonly referred to as organizing pneumonia, both clinically and pathologically.

Signs and symptoms

The classic presentation of COP is the development of nonspecific systemic (e.g. fevers, chills, night sweats, fatigue, weight loss) and respiratory (e.g. dyspnea, cough) symptoms in association with filling of the lung alveoli that is visible on chest x-ray. This presentation is usually so suggestive of an infection that the majority of patients with COP have been treated with at least one failed course of antibiotics by the time the true diagnosis is made.

Causes

• Pulmonary infection by bacteria, viruses and parasites
• drugs: antineoplastic drugs, erlotinib
• toxic fumes
• Ionizing radiations
• inflammatory diseases
  • systemic lupus
  • rheumatoid arthritis (RA-associated BOOP)
  • scleroderma
• Bronchial obstruction
  • Proximal bronchial squamous cell carcinoma

It was identified in 1985, although its symptoms had been noted before but not recognised as a separate lung disease. The risk of BOOP is higher for people with inflammatory diseases like lupus, rheumatoid arthritis, and scleroderma.

Diagnosis

On clinical examination, crackles are common, and more rarely, patients may have clubbing (<5% of cases). Laboratory findings are nonspecific.

Almost 75% of people have symptoms for less than two months before seeking medical attention. A flu-like illness, with a cough, fever, a feeling of illness (malaise), fatigue, and weight loss heralds the onset in about 40% of patients. Doctors do not find any specific abnormalities on routine laboratory tests or on a physical examination, except for the frequent presence of crackling sounds (called rales) when the doctor listens with a stethoscope. Pulmonary function tests usually show that the amount of air the lungs can hold is below normal. The amount of oxygen in the blood is often low at rest and is even lower with exercise.

Imaging

CT scan showing bronchiolitis obliterans organizing pneumonia (biopsy-proven)

The chest x-ray is distinctive with features that appear similar to an extensive pneumonia, with both lungs showing widespread white patches. The white patches may seem to migrate from one area of the lung to another as the disease persists or progresses. Computed Tomography (CT) may be used to confirm the diagnosis. Often the findings are typical enough to allow the doctor to make a diagnosis without ordering additional tests. To confirm the diagnosis, a doctor may perform a lung biopsy using a bronchoscope. Many times, a larger specimen is needed and must be removed surgically.

Plain chest radiography shows normal lung volumes, with characteristic patchy unilateral or bilateral consolidation. Small nodular opacities occur in up to 50% of patients and large nodules in 15%. On high resolution computed tomography, airspace consolidation with air bronchograms is present in more than 90% of patients, often with a lower zone predominance A subpleural or peribronchiolar distribution is noted in up to 50% of patients. Ground glass appearance or hazy opacities associated with the consolidation are detected in most patients.

Pulmonary physiology is restrictive with a reduced diffusion capacity of the lung for carbon monoxide (D_LCO). Airflow limitation is uncommon; gas exchange is usually abnormal and mild hypoxemia is common. Bronchoscopy with bronchoalveolar lavage reveals up to 40% lymphocytes, along with more subtle increases in neutrophils and eosinophils. In patients with typical clinical and radiographic features, a transbronchial biopsy that shows the pathologic pattern of organizing pneumonia and lacks features of an alternative diagnosis is adequate to make a tentative diagnosis and start therapy. On surgical lung biopsy, the histopathologic pattern is organizing pneumonia with preserved lung architecture; this pattern is not exclusive to BOOP and must be interpreted in the clinical context.

Histologically, cryptogenic organizing pneumonia is characterized by the presence of polypoid plugs of loose organizing connective tissue (Masson bodies) within alveolar ducts, alveoli, and bronchioles.

Consequence

Rare cases of BOOP have induced with lobar cicatricial atelectasis.

Treatment

Most patients recover with corticosteroid therapy. A standardized approach to dosing starting at 0.75 mg/kg and weaning over 24 weeks has been shown to reduce total corticosteroid exposure without affecting outcome.

About two thirds of patients recover with corticosteroid therapy: the usual steroid administered is prednisolone in Europe and prednisone in the USA; these differ by only one functional group and have the same clinical effect. The steroid is initially administered in high dosage, typically 50 mg per day tapering down to zero over a six-month to one-year period. If the steroid treatment is halted too quickly the disease may return. Other drugs must be taken to counteract side effects of the steroid.

References

External links

• Support & Information for COP & BOOP
• "Idiopathic Interstitial Pneumonias". Merck Manual Professional. May 2008. 

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

• 1. 特発性器質化肺炎 cryptogenic organizing pneumonia
• 2. 間質性肺疾患における肺生検結果の解釈 interpretation of lung biopsy results in interstitial lung disease
• 3. 特発性間質性肺炎：臨床症状および病理 idiopathic interstitial pneumonias clinical manifestations and pathology
• 4. 成人における細気管支炎 bronchiolitis in adults
• 5. 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

English Journal

• Organizing Pneumonia and Non-Necrotizing Granulomata on Transbronchial Biopsy: Co-Existence or Bronchiolitis Obliterans Organizing Pneumonia Secondary to Mycobacterium kansasii Disease.

• Starobin D, Guller V, Gurevich A, Fink G, Huszar M, Tal S.AbstractMycobacterium kansasii disease was diagnosed in an 85-year-old woman admitted to the hospital due to cough and gradually worsening breathlessness. The transbronchial biopsy specimen demonstrated either non-necrotizing granulomata or organizing pneumonia. The patient has been cured with combined therapy of specific anti-mycobacterial medications and systemic steroids. This is a first case of M. kansasii non-tuberculous mycobacterium disease with a BOOP-like pattern in the lung biopsy.
• Respiratory care.Respir Care.2011 Jun 17. [Epub ahead of print]
• Mycobacterium kansasii disease was diagnosed in an 85-year-old woman admitted to the hospital due to cough and gradually worsening breathlessness. The transbronchial biopsy specimen demonstrated either non-necrotizing granulomata or organizing pneumonia. The patient has been cured with combined ther
• PMID 21682987

• Chronic exposure to particles caused bronchioloalveolar carcinoma in a patient with cryptogenic organizing pneumonia evaluated by elemental analysis.

• Shimizu Y, Matsuzaki S, Mori M, Dobashi K.SourceDepartment of Medicine and Molecular Science, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan.
• Immunopharmacology and immunotoxicology.Immunopharmacol Immunotoxicol.2011 Jun 16. [Epub ahead of print]
• An 81-year-old Japanese man had organizing pneumonia (OP), and he had worked as a painter and had a history of exposure of various paints over 20 years. The major features on computed tomography (CT) in patients were cryptogenic organizing pneumonia (COP) showing airspace consolidation, and air bron
• PMID 21675931

Japanese Journal

• 乳房温存術後放射線治療後に発症したBOOPの3例

• 李 信男,野原 淳,坂口 泰人,寺田 泰二,大野 暢宏
• 気管支学 : 日本気管支研究会雑誌 33(3), 187-193, 2011-05-25
• 背景.乳房温存術後放射線療法後にBOOPを呈することがある.今回我々が経験した3例を報告する.症例.全例放射線療法後2ヵ月から6ヵ月後に咳嗽,発熱にて発症し,胸部X線上照射側の肺に浸潤陰影を認めた.1例はステロイドを使用せず改善,残りの2例においてはステロイドで治療し改善した.臨床経過や組織学的所見より放射線に関連したBOOPと診断した.結論.病態の成因は明らかでないものの,同様の報告が増加してお …
• NAID 110008686113

• リンパ球刺激試験で内分泌療法薬に陽性を示した乳癌温存手術後の bronchiolitis obliterans organizing pneumonia (BOOP) の2例

• 堀内 喜代美,鈴木 留美,川真田 明子,飯原 雅季,岡本 高宏
• 日本臨床外科学会雑誌 = The journal of the Japan Surgical Association 72(3), 613-618, 2011-03-25
• NAID 10028121763

Related Links

• Bronchiolitis Obliterans Organizing Pneumonia (BOOP ...

Bronchiolitis obliterans with organizing pneumonia (BOOP) is a rare lung condition in which the small airways (bronchioles) and air exchange sac (alveoli) become inflamed with connective tissue. ... Bronchiolitis Obliterans Organizing ...

• Imaging in Bronchiolitis Obliterans Organizing Pneumonia

Organizing pneumonia is characterized by the presence of granulation tissue in the distal air spaces. When organizing pneumonia is associated with granulation tissue in the bronchiolar lumen, the qualifying term bronchiolitis obliterans (BO) is added. (See the image below.) Standard nonenhanced axial ...

Related Pictures

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★リンクテーブル★

リンク元	「閉塞性細気管支炎性器質化肺炎」「COP」
関連記事	「obliterans」

「閉塞性細気管支炎性器質化肺炎」

　　[★]

英

bronchiolitis obliterans organizing pneumonia、BOOP

同

ブープ、特発性器質化肺炎 cryptogenic organizing pneumonia COP

特徴

臨床診断名	IPF	NSIP	BOOP/COP
病理組織像	UIP		OP
発症様式	慢性	慢性/亜急性	亜急性
BALF所見	リンパ球±	CD8↑	CD8↑
予後	不良	良好(時に不良)	良好
分布	斑状,不均質, 胸膜下･小葉辺縁	びまん性,均質	小葉中心性
時相	多様	均質	均質
間質への細胞浸潤	少ない	通常多い	やや多い
胞隔の炎症	軽度,斑状	びまん性,多彩	軽度
線維芽細胞巣	多数	まれ	なし
肺胞内マクロファージ集積	巣状	巣状	なし
肺胞腔内線維化	まれ	しばしば	多数
顕微鏡的蜂巣肺(肺胞虚説)	高頻度	通常なし(一部に認める)	なし
硝子膜	なし	なし	なし

概念

• 細気管支領域内腔がポリープ状肉芽により閉塞され、細気管支胞隔炎を呈した病態
• 病理学的な定義：

• 1. 閉塞性細気管支炎および器質化肺炎の所見が斑状に分布する、
• 2. 種々の程度の単核球細胞浸潤が間質にみられる、
• 3. 肺胞腔内に泡沫細胞が集簇する、
• 4. 肺の既存構造は保たれており、蜂窩肺形成や広範な間質性線維化は認められない

疫学

• 50歳代の発症が多い
• 喫煙との関連なし

症状

• 咳、発熱、咳嗽

身体所見

• 肺の副雑音
• バチ指は稀

病因

• 膠原病、感染症、移植後など　←　特発性BOOPとは病態や治療に対する反応も異なる
• 原因不明　→　特発性BOOP

検査

血液検査

• 赤沈亢進、CRP陽性
• 白血球数増加もあり売る

胸部単純X線写真

• びまん性、下肺野優位の両側斑状陰影
• 肺炎様の浸潤、陰影はしばしば移動

=胸部CT写真

• 2パターン：エアブロンコグラムを伴う肺炎様の恋浸潤英(肺胞性パターン)、びまん性の微細粒状影(間質性パターン)
• 主として胸膜下に沿うすりガラス陰影(コンソリデーション)
• 蜂窩肺　は　ない

BALF所見

• リンパ球%↑、CD8%増加(50%の症例)、好中球%・好酸球%増加(50%の症例)

肺機能検査

• 拘束性換気障害、拡散障害
• 閉塞性換気障害は稀

診断

鑑別診断

臨床症状・胸部X線写真上での鑑別疾患

• 感染性肺炎
• ウェゲナー肉芽腫症
• 特発性肺線維症 IPF：発症時期が明確でない、慢性経過をたどっている。BOOPで認められない蜂巣肺所見がX線・CTで認められる。BALF所見がCD8↑でない。リンパ球数±
• 非特異性間質性肺炎 NSIP：一部に蜂巣肺所見。
• 慢性好酸急性肺炎：肺病理所見を除けば、臨床所見・検査所見で区別できない。リンパ球%↑は同様

BALFでの鑑別疾患

• サルコイドーシス、慢性ベリリウム肺：胸部X線写真、CD8↓から鑑別可能 (SPU.274)
• 過敏性肺臓炎：胸部X線写真上で鑑別可能

治療

• 自然軽快が少なくないので、軽症例では経過観察
• ステロイド薬

予後

• 特発性BOOPはステロイド薬が有効で予後良好。時に再燃する。

「COP」

　　[★]

• 閉塞性細気管支炎性器質化肺炎 bronchiolitis obliterans organizing pneumonia BOOP = 閉塞性細気管支炎性器質化肺炎 cryptogenic organizing pneumonia
• 膠質浸透圧 colloid osmotic pressure

「obliterans」

　　[★]

• adj.

• 閉塞性の

関

constrictive、obliterative、obstructive、occlusive、occlusively

bronchiolitis obliterans、thromboangiitis obliterans、arteriosclerosis obliterans