interstitial lung disease

間質性肺炎

WordNet

1. an impairment of health or a condition of abnormal functioning
2. of or relating to interstices
3. caused by or altered by or manifesting disease or pathology; "diseased tonsils"; "a morbid growth"; "pathologic tissue"; "pathological bodily processes" (同)morbid, pathologic, pathological
4. 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. 病気にかかった / 病的な,不健全な(morbid)
4. 《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）』「2014/04/01 18:23:36」(JST)

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Interstitial lung disease
Classification and external resources
End-stage pulmonary fibrosis of unknown origin, taken from an autopsy in the 1980s.
ICD-10	J84.9
ICD-9	518.89, 508.1, 515, 516.3, 714.81, 770.7

Interstitial lung disease (ILD), also known as diffuse parenchymal lung disease (DPLD), refers to a group of lung diseases affecting the interstitium (the tissue and space around the air sacs of the lungs). It concerns alveolar epithelium, pulmonary capillary endothelium, basement membrane, perivascular and perilymphatic tissues.

The term ILD is used to distinguish these diseases from obstructive airways diseases.

Prolonged ILD may result in pulmonary fibrosis, but this is not always the case. The term Idiopathic pulmonary fibrosis is used to describe interstitial lung disease for which no obvious cause can be identified (idiopathic), and is associated with typical radiographic (basal and pleural based fibrosis with honeycombing) and pathologic (temporally and spatially heterogeneous fibrosis, histopathologic honeycombing and fibroblastic foci) findings.

Causes

The alveoli

Micrograph of usual interstitial pneumonia (UIP). UIP is the most common pattern of idiopathic interstitial pneumonia (a type of interstitial lung disease) and usually represents idiopathic pulmonary fibrosis. H&E stain. Autopsy specimen.

ILD may be classified according to the cause. One method of classification is as follows: (Mnemonic- Inhaled Drugs Can Infect Idiopathic Malignancy)

1. Inhaled substances
   • Inorganic
     • Silicosis
     • Asbestosis
     • Berylliosis
   • Organic
     • Hypersensitivity pneumonitis
2. Drug induced
   • Antibiotics
   • Chemotherapeutic drugs
   • Antiarrhythmic agents
   • Statins
3. Connective tissue disease
   • Systemic sclerosis
   • Polymyositis
   • Dermatomyositis
   • Systemic lupus erythematosus
   • Rheumatoid arthritis
4. Infection (mnemonic- TB is CRAP)
   • Atypical pneumonia
   • Pneumocystis pneumonia (PCP)
   • Tuberculosis
   • Chlamydia trachomatis
   • Respiratory Syncytial Virus
5. Idiopathic
   • Sarcoidosis
   • Idiopathic pulmonary fibrosis
   • Hamman-Rich syndrome
   • Antisynthetase syndrome
6. Malignancy
   • Lymphangitic carcinomatosis

Investigation

Patients with pneumocystis pneumonia can present with interstitial lung disease, as seen in the reticular markings on this AP chest x-ray

A chest X-ray demonstrating pulmonary fibrosis due to amiodarone.

Investigation is tailored towards the symptoms and signs. A proper and detailed history looking for the occupational exposures, and for signs of conditions listed above is the first and probably the most important part of the workup in patients with interstitial lung disease. Pulmonary function tests usually show a restrictive defect with decreased diffusion capacity (DLCO) .

A lung biopsy is required if the clinical history and imaging are not clearly suggestive of a specific diagnosis or malignancy cannot otherwise be ruled out. In cases where a lung biopsy is indicated, a trans-bronchial biopsy is usually unhelpful, and a surgical lung lung biopsy is often required.

Role of radiology in the diagnosis of interstitial lung disease

Chest radiography is usually the first test to detect interstitial lung diseases, but the chest radiograph can be normal in up to 10% of patients, especially early on the disease process.

High resolution CT of the chest is the preferred modality, and differs from routine CT of the chest. Conventional (regular) CT chest examines 7-10 mm slices obtained at 10 mm intervals; high resolution CT examines 1-1.5 mm slices at 10 mm intervals using a high spatial frequency reconstruction algorithm. The HRCT therefore provides approximately 10 times more resolution than the conventional CT chest, allowing the HRCT to elicit details that cannot otherwise be visualized.

Radiologic appearance alone however is not adequate and should be interpreted in the clinical context, keeping in mind the temporal profile of the disease process.

Interstitial lung diseases can be classified according to radiologic patterns

Pattern of opacities

Consolidation

Acute: Alveolar hemorrhage syndromes, acute eosinophilic pneumonia, acute interstitial pneumonia, cryptogenic organizing pneumonia

Chronic: Chronic eosinophilic pneumonia, cryptogenic organizing pneumonia, lymphoproliferative disorders, pulmonary alveolar proteinosis, sarcoidosis

Linear or reticular opacities

Acute: Pulmonary edema

Chronic: Idiopathic pulmonary fibrosis, connective tissue associated interstitial lung diseases, asbestosis, sarcoidosis, hypersensitivity pneumonitis, drug induced lung disease

Small nodules

Acute: Hypersensitivity pneumonitis

Chronic: Hypersensitivity pneumonitis, sarcoidosis, silicosis, coal workers pneumoconiosis, respiratory bronchiolitis, alveolar microlithiasis

Cystic airspaces

Chronic: Pulmonary langerhans cell histiocytosis, pulmonary lymphangioleiomyomatosis, honeycomb lung caused by IPF or other diseases

Ground glass opacities

Acute: Alveolar hemorrhage syndromes, pulmonary edema, hypersensitivity pneumonitis, acute inhalational exposures, drug induced lung diseases, acute interstitial pneumonia

Chronic: Nonspecific interstitial pneumonia, respiratory bronchiolitis associated interstitial lung disease, desquamative interstitial pneumonia, drug induced lung diseases, pulmonary alveolar proteinosis

Thickened alveolar septa

Acute: Pulmonary edema

Chronic: Lymphangitic carcinomatosis, pulmonary alveolar proteinosis, sarcoidosis, pulmonary veno occlusive disease

Distribution

Upper lung predominance

Pulmonary Langerhans cell histiocytosis, silicosis, coal workers pneumoconiosis, carmustine related pulmonary fibrosis

Lower lung predominance

Idiopathic pulmonary fibrosis, pulmonary fibrosis associated with connective tissue diseases, asbestosis, chronic aspiration

Central predominance (perihilar)

Sarcoidosis, berylliosis

Peripheral predominance

Idiopathic pulmonary fibrosis, chronic eosinophilic pneumonia, cryptogenic organizing pneumonia

Associated findings

Pleural effusion or thickening

Pulmonary edema, connective tissue diseases, asbestosis, lymphangitic carcinomatosis, lymphoma, lymphangioleiomyomatosis, drug induced lung diseases

Lymphadenopathy

Sarcoidosis, silicosis, berylliosis, lymphangitic carcinomatosis, lymphoma, lymphocytic interstitial pneumonia

Treatment

ILD is not a single disease, but encompasses many different pathological processes. Hence treatment is different for each disease.

If a specific occupational exposure cause is found, the person should avoid that environment. If a drug cause is suspected, that drug should be discontinued.

Many idiopathic and connective tissue-based causes of ILD are treated with corticosteroids, such as prednisolone. Some patients respond to immunosuppressant treatment. Patients with hypoxemia may be given supplemental oxygen.

Lung transplantation is an option if the ILD progresses despite therapy in appropriately selected patients with no other contraindications.

References

External links

For more information and resources on ILD, please visit the links below:

• AIMIP Onlus - Italian Interstitial Lung Diseases No-Profit Organization
• 00736 at CHORUS
• 1476788304 at GPnotebook
• Pulmonary Fibrosis at the US National Library of Medicine Medical Subject Headings (MeSH)
• MedlinePlus Overview pulmonaryfibrosis
• coalitionforpf.org - Coalition for Pulmonary Fibrosis: Pulmonary Fibrosis Patient Services, Education; Funding Research to Find a Cure for PF
• GeneReview/NCBI/NIH/UW entry on Pulmonary Fibrosis, Familial
• UCSF Interstitial Lung Disease Program at University of California San Francisco
• Interstitial Lung Disease Program at University of Chicago Medical Center
• Interstitial Lung Disease Center at University of Cincinnati
• Interstitial Lung Diseases Program at Northwestern Medicine in Chicago
• PA-IPF - The Pennsylvania Idiopathic Pulmonary Fibrosis State Registry at University of Pittsburgh
• WASOG - World Association for Sarcoidosis and Other Granulomatous Disorders

UpToDate Contents

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• 1. 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
• 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. 間質性肺疾患を有する成人に対するアプローチ：診断的検査 approach to the adult with interstitial lung disease diagnostic testing
• 4. 間質性肺疾患を有する幼児および小児に対するアプローチ approach to the infant and child with interstitial lung disease
• 5. 非特異的間質性肺炎 nonspecific interstitial pneumonia

English Journal

• Focused cardiopulmonary ultrasound for assessment of dyspnea in a resource-limited setting.

• Shah SP1, Shah SP2, Fils-Aime R3, Desir W3, Joasil J3, Venesy DM2, Muruganandan KM4.
• Critical ultrasound journal.Crit Ultrasound J.2016 Dec;8(1):7. doi: 10.1186/s13089-016-0043-y. Epub 2016 Jun 3.
• BACKGROUND: The diagnosis and management of acutely dyspneic patients in resource-limited developing world settings poses a particular challenge. Focused cardiopulmonary ultrasound (CPUS) may assist in the emergency diagnosis and management of patients with acute dyspnea by identifying left ventricu
• PMID 27260349

• Fibrotic gene expression coexists with alveolar proteinosis in early indium lung.

• Noguchi S1, Eitoku M1, Kiyosawa H1, Suganuma N1.
• Inhalation toxicology.Inhal Toxicol.2016 Aug;28(9):421-8. doi: 10.1080/08958378.2016.1193573.
• Occupational inhalation of indium compounds can cause the so-called "indium lung disease". Most affected individuals show pulmonary alveolar proteinosis (PAP) and fibrotic interstitial lung disease. In animal experiments, inhalation of indium tin oxide or indium oxide has been shown to cause lung da
• PMID 27308969

• Dynamic changes of mononuclear phagocytes in circulating, pulmonary alveolar and interstitial compartments in a mouse model of experimental silicosis.

• Xiang GA1, Zhang YD1, Su CC1, Ma YQ2, Li YM2, Zhou X2, Wei LQ1, Ji WJ1,2.
• Inhalation toxicology.Inhal Toxicol.2016 Aug;28(9):393-402. doi: 10.1080/08958378.2016.1188186. Epub 2016 May 31.
• CONTEXT: Silicosis is a devastating, irreversible lung fibrosis condition exposed to crystalline silica. The mononuclear phagocyte system plays an important role in the pathogenesis of silicosis.OBJECTIVE: The present study was aimed to explore the dynamic changes of mononuclear phagocytes in circul
• PMID 27240636

Japanese Journal

• びまん性肺疾患の画像診断 : 間質性肺炎(第1回)特発性間質性肺炎 IPF/UIP

• 日本胸部臨床 75(6), 655-665, 2016-06
• NAID 40020870194

• 間質性肺炎に対するNPPV (特集 『NPPV(非侵襲的陽圧換気療法)ガイドライン改訂第2版』をめぐって)

• 日本胸部臨床 75(6), 600-606, 2016-06
• NAID 40020870099

• Increased Serum KL-6 Levels Induced by Pulmonary Mycobacterium Avium Complex Infection in a Patient with RA-associated Lung Disease

• Acta Medica Okayama 70(3), 217-221, 2016-06
• NAID 120005770691

Related Links

• Interstitial Lung Disease - American Thoracic Society - We ...

Interstitial Lung Disease Chapter 10 102 What are we learning about this disease? Pathophysiology, causes, genetic, environment, microbes Inflammation is a characteristic cellular and molecular response to injury, nox-ious ...

• Interstitial lung disease | インターネット内の検索結果 ...

ウィキペディアの英語版でInterstitial lung diseaseという用語が挙げてありますが、そこでは、以下の意味で使われています。Interstitial lung disease (ILD), also known as diffuse parenchymal lung disease (DPLD), refers to a group of lung ...

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★リンクテーブル★

リンク元	「間質性肺炎」「間質性肺疾患」
関連記事	「disease」「interstitial」「lung disease」

「間質性肺炎」

　　[★]

英

interstitial pneumonitis, interstitial lung disease, pneumonitis, interstitial pneumonia

同

びまん性間質性肺炎 diffuse interstitial pneumonia、びまん性線維性肺胞炎 diffuse fibrosisalveolitis、肺臓炎 pneumonitis

関

肺炎、間質性肺疾患

間質性肺炎と実質性肺炎

• 実質性肺炎・・・いわゆる肺炎。実質(肺胞上皮細胞および肺胞腔)の炎症。区域性の分布
• 間質性肺炎・・・実質の間を埋める間質が炎症の場。胞隔炎。びまん性の分布

定義

• 間質(肺胞壁や細気管支、細動静脈周囲など)を病変の主座とする炎症性疾患に対する病理組織学的総称。大葉性・小葉性肺炎(肺胞、肺胞道などの気腔内への滲出性病変)に対比して用いられる。

検査

• 拘束性換気障害(%VC、%肺活量が80%以下)としてとらえられる。

病因による分類

• 特発性間質性肺炎

• 特発性間質性肺炎 idiopathic interstitial pneumonia IIP

• 続発性間質性肺炎

間質性肺炎を来す疾患として、塵肺(無機塵・有機塵、エアロゾル？)、膠原病(PSS, SLE, RAなど)、感染症(ウイルス感染、細菌感染)、薬物誘起性肺炎、放射線肺炎など

• 無機塵肺(アスベスト、ベリリウム、珪酸)
• 薬剤(メトトレキサート、金製剤、シクロホスファミド、IFN-α、小柴胡湯、ブシラミン、アミオダロン、ゲフィチニブなど)
• 放射線
• 感染(サイトメガロウイルス、EBウイルス、C型肝炎ウイルス、マイコプラズマ、ニューモシスチス、水痘・帯状疱疹ウイルス、麻疹ウイルスなど)
• アレルギー(サルコイドーシス、過敏性肺臓炎)
• そのた、パラコート

• 膠原病に続発

• 強皮症、皮膚筋炎/多発筋炎、関節リウマチ、シェーグレン症候群、全身性エリテマトーデス、混合性結合組織病

「間質性肺疾患」

　　[★]

英

interstitial lung disease

関

間質性肺炎

「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

　 　 　

「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

　

「lung disease」

　　[★]

関

Respiratory disease

関

respiratory disease

関

respiratory disease