restrictive lung disease

拘束性肺疾患

WordNet

1. an impairment of health or a condition of abnormal functioning
2. serving to restrict; "teenagers eager to escape restrictive home environments"
3. (of tariff) protective of national interests by restricting imports
4. caused by or altered by or manifesting disease or pathology; "diseased tonsils"; "a morbid growth"; "pathologic tissue"; "pathological bodily processes" (同)morbid, pathologic, pathological
5. either of two saclike respiratory organs in the chest of vertebrates; serves to remove carbon dioxide and provide oxygen to the blood

PrepTutorEJDIC

1. (体の)『病気』,疾患 / (精神・道徳などの)病気,病弊
2. 女性の話術芸人 =diseur
3. 制限する,限定的な / (文法で)制限的な
4. 病気にかかった / 病的な,不健全な(morbid)
5. 《しばしば複数形で》『肺』,肺臓

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2017/06/09 14:48:29」(JST)

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Restrictive lung disease
Classification and external resources
ICD-9-CM	518.89
eMedicine	article/301760

Restrictive lung diseases (or restrictive ventilatory defects) are a category of extrapulmonary, pleural, or parenchymal respiratory diseases that restrict lung expansion, resulting in a decreased lung volume, an increased work of breathing, and inadequate ventilation and/or oxygenation. Pulmonary function test demonstrates a decrease in the forced vital capacity.

Pathophysiology

In disorders that are intrinsic to the lung parenchyma, the underlying process is usually pulmonary fibrosis (scarring of the lung). As the disease progresses, the normal lung tissue is gradually replaced by scar tissue interspersed with pockets of air. This can lead to parts of the lung having a honeycomb-like appearance.

Presentation

The main symptoms are shortness of breath and cough.

Diagnosis

In restrictive lung disease, both forced expiratory volume in one second (FEV1) and forced vital capacity (FVC) are reduced, however, the decline in FVC is more than that of FEV1, resulting in a higher than 80% FEV1/FVC ratio. In obstructive lung disease however, the FEV1/FVC is less than 0.7, indicating that both FEV1 and FVC are reduced. This indicates that the FVC is also reduced, but not by the same ratio as FEV1.

One definition requires a total lung capacity which is 80% or less of the expected value.

Causes and classification

Restrictive lung diseases may be due to specific causes which can be intrinsic to the parenchyma of the lung, or extrinsic to it.

Intrinsic

• Pneumoconiosis caused by long-term exposure to dusts, especially in mining. For example, Asbestosis.
• Radiation fibrosis, usually from the radiation given for cancer treatment.
• Certain drugs such as amiodarone, bleomycin and methotrexate.
• As a consequence of another disease such as rheumatoid arthritis.
• Hypersensitivity pneumonitis due to an allergic reaction to inhaled particles.
• Acute respiratory distress syndrome (ARDS), a severe lung condition occurring in response to a critical illness or injury.
• Infant respiratory distress syndrome due to a deficiency of surfactant in the lungs of a baby born prematurely.
• Tuberculosis

Many cases of restrictive lung disease are idiopathic (have no known cause). Still, there is generally pulmonary fibrosis. Examples are:

• Idiopathic pulmonary fibrosis
• Idiopathic interstitial pneumonia, of which there are several types
• Sarcoidosis
• Eosinophilic pneumonia
• Lymphangioleiomyomatosis
• Pulmonary Langerhans' cell histiocytosis
• Pulmonary alveolar proteinosis

Conditions specifically affecting the interstitium are called interstitial lung diseases.

Extrinsic

• Nonmuscular diseases of the upper thorax such as kyphosis, pectus carinatum and pectus excavatum.
• Diseases restricting lower thoracic/abdominal volume (e.g. obesity, diaphragmatic hernia, or the presence of ascites).
• Pleural thickening.

See also

• Chronic obstructive pulmonary disease
• Obstructive lung disease

UpToDate Contents

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• 1. 成人における肺機能検査の概要 overview of pulmonary function testing in adults
• 2. フローボリューム曲線 flow volume loops
• 3. 間質性肺疾患を有する成人に対するアプローチ：臨床的評価 approach to the adult with interstitial lung disease clinical evaluation
• 4. 小児における肺機能検査の概要 overview of pulmonary function testing in children
• 5. 一酸化炭素拡散能 diffusing capacity for carbon monoxide

English Journal

• Lung capillary injury and repair in left heart disease: a new target for therapy?

• Azarbar S1, Dupuis J.Author information 1*Department of Medicine, Montreal Heart Institute, Université de Montréal, 5000 Belanger Street, Montreal, Quebec, Canada, H1T 1C8.AbstractThe lungs are the primary organs affected in LHD (left heart disease). Increased left atrial pressure leads to pulmonary alveolar-capillary stress failure, resulting in cycles of alveolar wall injury and repair. The reparative process causes the proliferation of MYFs (myofibroblasts) with fibrosis and extracellular matrix deposition, resulting in thickening of the alveolar wall. Although the resultant reduction in vascular permeability is initially protective against pulmonary oedema, the process becomes maladaptive causing a restrictive lung syndrome with impaired gas exchange. This pathological process may also contribute to PH (pulmonary hypertension) due to LHD. Few clinical trials have specifically evaluated lung structural remodelling and the effect of related therapies in LHD. Currently approved treatment for chronic HF (heart failure) may have direct beneficial effects on lung structural remodelling. In the future, novel therapies specifically targeting the remodelling processes may potentially be utilized. In the present review, we summarize data supporting the clinical importance and pathophysiological mechanisms of lung structural remodelling in LHD and propose that this pathophysiological process should be explored further in pre-clinical studies and future therapeutic trials.
• Clinical science (London, England : 1979).Clin Sci (Lond).2014 Jul 1;127(2):65-76. doi: 10.1042/CS20130296.
• The lungs are the primary organs affected in LHD (left heart disease). Increased left atrial pressure leads to pulmonary alveolar-capillary stress failure, resulting in cycles of alveolar wall injury and repair. The reparative process causes the proliferation of MYFs (myofibroblasts) with fibrosis a
• PMID 24678967

• The degree of skin involvement identifies distinct lung disease outcomes and survival in systemic sclerosis.

• Cottrell TR1, Wise RA, Wigley FM, Boin F.Author information 1Johns Hopkins University School of Medicine, , Baltimore, Maryland, USA.AbstractOBJECTIVE: To determine whether the pattern of skin involvement can predict clinical features, risk of restrictive lung disease (RLD) and survival in a large scleroderma (SSc) cohort.
• Annals of the rheumatic diseases.Ann Rheum Dis.2014 Jun 1;73(6):1060-1066. doi: 10.1136/annrheumdis-2012-202849. Epub 2013 Apr 20.
• OBJECTIVE: To determine whether the pattern of skin involvement can predict clinical features, risk of restrictive lung disease (RLD) and survival in a large scleroderma (SSc) cohort.METHODS: Demographic and clinical data collected over 30 years from 2205 patients with SSc were retrospectively analy
• PMID 23606705

• Pulmonary function abnormalities in childhood cancer survivors treated with bleomycin.

• De A1, Guryev I, Lariviere A, Kato R, Wee CP, Mascarenhas L, Keens TG, Venkatramani R.Author information 1Division of Pulmonology, Children's Hospital Los Angeles, Los Angeles, California.AbstractBACKGROUND: Bleomycin is associated with pulmonary toxic side effects including pneumonitis and pulmonary fibrosis. We evaluated the prevalence of long-term pulmonary function abnormalities in children receiving bleomycin therapy in the context of current chemotherapeutic regimens.
• Pediatric blood & cancer.Pediatr Blood Cancer.2014 May 2. doi: 10.1002/pbc.25098. [Epub ahead of print]
• BACKGROUND: Bleomycin is associated with pulmonary toxic side effects including pneumonitis and pulmonary fibrosis. We evaluated the prevalence of long-term pulmonary function abnormalities in children receiving bleomycin therapy in the context of current chemotherapeutic regimens.METHODS: A retrosp
• PMID 24789770

Japanese Journal

• 肺動脈性肺高血圧症および混合性呼吸障害合併患者に対する低用量等比重ブピバカインを用いた脊髄くも膜下麻酔

• 鵜澤 康二,箱根 雅子,中澤 春政 [他]
• 麻酔 63(2), 157-160, 2014-02
• NAID 40019965921

• 肺癌術直後の吸引痰サーベイランスは術後肺炎を予測できない：術後肺炎の危険因子の後方視的検討

• 溝渕 輝明,尹 貴正,鈴木 秀海,稲毛 輝長,山本 高義,鎌田 稔子,森本 淳一,中島 崇裕,岩田 剛和,田川 哲三,吉田 成利,吉野 一郎
• 日本呼吸器外科学会雑誌 28(5), 557-563, 2014
• 我々はCDCガイドラインに準じた予防的抗菌薬投与と慢性閉塞性肺疾患に対する周術期の積極的治療介入を行い術後肺炎の予防に努めてきたが未だ術後肺炎を時に経験する．気道内の細菌コロニー形成が術後肺炎に関与するかを検討した．2011年の原発性肺癌手術114例に対し，術直後の気管支鏡吸引痰培養検査で潜在的病原菌100コロニー以上を有意とし，サーベイランスの結果を含む術後肺炎の危険因子を後方視的に検証した．1 …
• NAID 130004678879

• Imatinibにより改善した強皮症型皮膚慢性移植片対宿主病の小児症例

• 高橋 寛吉,康 勝好,加藤 元博,岸本 宏志,小熊 栄二,花田 良二
• 日本造血細胞移植学会雑誌 3(1), 27-31, 2014
• 　早期に骨髄および中枢神経に再発したB 前駆細胞性急性リンパ性白血病の4 歳男児に対し，HLA抗原型8/8一致の母より同種末梢血幹細胞移植を行った。移植102 日後より皮膚乾燥を認め，慢性移植片対宿主病と診断した。Prednisoloneを投与したが移植1年後より強皮症型の皮膚所見となり，成長に伴い脊柱側彎を認めた。移植9年後より胸郭の呼吸運動の制限による肺拘束性障害と，脊柱側彎の進行による右主気 …
• NAID 130003397474

Related Links

• Restrictive Lung Disease - Diseases & Conditions ...

Restrictive lung diseases are characterized by reduced lung volumes, either because of an alteration in lung parenchyma or because of a disease of the pleura, chest wall, or neuromuscular apparatus. Unlike obstructive lung diseases, including asthma and COPD, which show a normal or increased total lung ...

• restrictive lung disease - definition of restrictive lung disease ...

restrictive lung disease Pulmonology A general term that encompasses the functional aspects of interstitial lung disease Etiology-Acute Infections–miliary TB, histoplasmosis, PCP, CMV, fungal; RT; pulmonary edema, inhalation ...

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★リンクテーブル★

リンク元	「拘束性肺疾患」「拘束性」
関連記事	「disease」「lung disease」

「拘束性肺疾患」

　　[★]

英

restrictive lung disease

同

拘束性疾患

関

拘束性換気障害

定義

• 肺活量が正常値の80%以下

• 肺機能検査項目に影響を及ぼす主因

病態

• コンプライアンスC↓　＝　弾性仕事が増加する (TP.353)

関連する疾患

• 肺線維症　←　間質性肺疾患なら拘束性の障害を来すはず。過敏性肺臓炎も加えてよい。

• 肺コンプライアンスCは減少
• FRCが減少

「拘束性」

　　[★]

英

restrictive

• 拘束性肺疾患 restrictive lung disease
• 拘束型心筋症 restrictive cardiomyopathy

「disease」

　　[★]

• n.

疾患：illnessより厳密な概念。「ある臓器に明確な障害が確認され、それによって症状が出ているとはっきり説明できる場合」 (PSY.9)

特定の原因、病態生理、症状、経過、予後、病理組織所見が全てそろった場合 (PSY.9)

• something that is very wrong with people's attitudes, way of life or with society.

関

ail、ailment、disease entity、disorder、ill、illness、malady、sick、sickness

• 注意

disease ≠ illness ≠ disorder

　 　 　

「lung disease」

　　[★]

関

Respiratory disease

関

respiratory disease

関

respiratory disease