Idiopathic interstitial pneumonias (IIPs)
(AJRCCM 2013;188:733)
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Definition: ILD of unknown cause; dx by radiographic, histologic, and clinical features
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Rx for IPF: suppl O2, pulm rehab, Rx for GERD, PHT screening, lung tx referral;
medications
- pirfenidone (antifibrotic)
- nintedanib (tyrosine kinase inhib mediating fibrogenic growth factors)
- uptodate: Treatment of idiopathic pulmonary fibrosis
↓ rate of FVC decline (Lancet 2021;398:1450) 參考➡️ Treatment of fibrotic interstitial lung disease: current approaches and future directions
Steroids use of Idiopathic interstitial pneumonias (IIPs)
- high-dose steroids may be used for acute exacerbations, but no RCT data
- Steroids for other IIPs:
- NSIP (esp. cellular type) and COP (AJRCCM 2000;162:571);
- ? benefit for AIP and DIP/RB-ILD (for which Pts should stop smoking)
Explain by ChatGPT
Idiopathic interstitial pneumonias (IIPs) are a type of ILD of unknown cause. They are diagnosed by radiographic, histologic, and clinical features. The treatment for IPF is to give the patient supplemental oxygen, pulmonary rehabilitation, treatment for GERD, PHT screening, and referral for lung transplant. The medications used for idiopathic interstitial pneumonias are pirfenidone, which is an antifibrotic, and nintedanib, which is a tyrosine kinase inhibitor mediating fibrogenic growth factors. Steroids may be used for acute exacerbations, but there is no RCT data. Steroids are used for other IIPs such as NSIP (especially the cellular type) and COP. There may be some benefit for AIP and DIP/RB-ILD, but for these patients, they should stop smoking.
窩的英文不太好
- 特發性間質性肺炎 (IIP) 是一種原因不明的 ILD。它們通過放射學、組織學和臨床特徵來診斷。 IPF 的治療是給患者補充氧氣、肺復健、GERD 治療、PHT 篩查和轉診肺移植。
- 用於特發性間質性肺炎的藥物是抗纖維化藥物 吡非尼酮 和介導纖維化生長因子的酪氨酸激酶抑製劑 您他的您。
- 類固醇可用於急性加重，但尚無 RCT 數據。
- 類固醇用於其他 IIP，例如 NSIP（尤其是細胞類型）和 COP。
- AIP 和 DIP/RB-ILD 可能有一些益處，但對於這些患者，他們應該戒菸。