特發性間質性肺炎 Idiopathic interstitial pneumonia | |
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Micrograph of usual interstitial pneumonia (UIP). UIP is the most common pattern of idiopathic interstitial pneumonia and usually represents 特发性肺纤维化. 苏木精-伊红染色. 驗屍 specimen. | |
症状 | 肺炎 |
类型 | 肺炎、間質性肺病、特發性、疾病 |
分类和外部资源 | |
醫學專科 | 胸腔醫學 |
ICD-11 | CB03 |
Orphanet | 98300 |
特發性間質肺炎(Idiopathic interstitial pneumonia,簡稱IIP),又稱非感染性肺炎(noninfectious pneumonia)[1],屬於一種間質性肺病。該疾病通常侵襲肺間質,有些也會侵襲氣道(例如Cryptogenic organizing pneumonitis),IIP可分為七個亞型。
IIP的分類相當複雜[2],須結合臨床觀察、影像學,以及病理学[3][4]才能確診。
組織學 | 臨床相關意義 |
---|---|
Desquamative interstitial pneumonia (DIP) | DIP |
Diffuse alveolar damage (DAD) | 急性呼吸窘迫症候群、急性間質性肺炎、輸血相關急性肺損傷 |
非特異性間質性肺炎(NSIP) | NSIP |
Respiratory bronchiolitis | 呼吸性細支氣管相關之間質性肺病(RB-ILD) |
尋常性間質性肺炎 (UIP) | 自體免疫性疾病、特发性肺纤维化、致死量、肺塵病 |
Organizing pneumonia | 隱源性機化性肺炎 |
淋巴樣間質性肺炎 (LIP) | LIP |
其中尋常性間質性肺炎為最常見的一種亞型[7]。
Table 1: Development of the (histologic) idiopathic interstitial pneumonia classification
Leibow et al. (1969) | Katzenstein (1998)[8] | ATS/ERS (2002)[6] | |||||||||||||||||||||||||||
UIP | UIP | ||||||||||||||||||||||||||||
UIP | DAD | DAD | |||||||||||||||||||||||||||
NSIP | NSIP | ||||||||||||||||||||||||||||
DIP | DIP/RB | DIP | |||||||||||||||||||||||||||
RB | |||||||||||||||||||||||||||||
BIP | OP | OP | |||||||||||||||||||||||||||
LIP | (LPD) | LIP | |||||||||||||||||||||||||||
GIP | (HMF) | (HMF) | |||||||||||||||||||||||||||
UIP=usual interstitial pneumonia; DAD=diffuse alveolar damage; NSIP=non-specific interstitial pneumonia; DIP=desquamative interstitial pneumonia; RB=respiratory bronchiolitis; BIP=bronchiolitis obliterans interstitial pneumonia; OP=organizing pneumonia; LIP=lymphoid interstitial pneumonia; LPD=lymphoproliferative disease (not considered a diffuse lung disease); GIP=giant cell interstitial pneumonia; HMF=heavy metal fibrosis, no longer grouped with diffuse lung disease
Lymphoid interstitial pneumonia was originally included in this category, then excluded, then included again.[9]
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