Usual interstitial pneumonia is the predominant histopathology in patients with systemic sclerosis receiving a lung transplant.

Usual interstitial pneumonia is the predominant histopathology in patients with systemic sclerosis receiving a lung transplant.
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DOI:
10.55563/clinexprheumatol/icr6hy
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发表时间:
2023-08
影响因子:
3.7
通讯作者:
Lafyatis R
Lafyatis R
中科院分区:
医学4区
文献类型:
--
作者:
Valenzi E;Cody B;Lafyatis R

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非特异性间质性肺炎(NSIP)是系统性硬化症相关性间质性肺病(SSC-ILD)的主要组织病理学特征,研究主要利用外科肺活检来诊断早期疾病。这些病例系列可能只反映了早期疾病的组织病理学,而不同于呼吸衰竭患者晚期疾病的组织病理学。对2000-2021年间在单一中心接受肺移植诊断为SSc的患者进行了回顾性分析。作为常规护理的一部分,所有移植的肺都接受了组织病理学检查。在研究期间,127名SSc患者接受了原生肺移植。普通型间质性肺炎111例(87.4%),非间质性肺炎45例(35.4%),机化性肺炎11例(8.7%),淋巴细胞性支气管炎2例(1.6%)。37个外植体(29.1%)同时存在UIP和NSIP区,只有9个外植体(7.1%)既没有UIP区也没有NSIP区。组织学证实有49例(38.6%)外植体有误吸现象。19例患者的病理结果来自先前的手术肺活检,其中11例患者的活检和外植体保持相同的原发病理(2例NSIP,9例UIP),8例患者在不同的时间点显示不同的病理,所有患者的外植体上都有UIP。大多数患者(101例,79.5%)有肺动脉高压和血管病变的证据。在接受肺移植的SSc患者中,UIP是主要的组织病理学,许多患者同时患有NSIP和UIP,或在移植前随着时间的推移从NSIP进展为UIP。
Studies identifying nonspecific interstitial pneumonia (NSIP) as the predominant histopathology in systemic sclerosis-associated interstitial lung disease (SSc-ILD) have primarily utilized surgical lung biopsies in early disease. These case series may only reflect the histopathology of early disease, and differ from the histopathology of advanced disease in those with respiratory failure. Patients receiving a lung transplant for a diagnosis of SSc at a single center from 2000–2021 were included for retrospective analysis. All explanted lungs underwent histopathology review as part of routine care. 127 patients with SSc received a native lung transplant during the study period. Usual interstitial pneumonia (UIP) was identified in 111 explants (87.4%), NSIP in 45 (35.4%) explants, organizing pneumonia in 11 explants (8.7%), and lymphocytic bronchitis in 2 explants (1.6%). Areas of both UIP and NSIP were identified in 37 explants (29.1%), with only 9 explants (7.1%) showing neither UIP nor NSIP. Aspiration was identified on histology in 49 (38.6%) explants. Pathology results were available from a prior surgical lung biopsy for 19 patients, with 11 patients maintaining the same primary pathology on biopsy and explant (2 NSIP, 9 UIP) and 8 patients showing different pathology at the timepoints, all of whom had UIP on explant. Most patients (101, 79.5%) had evidence of pulmonary hypertension and vasculopathy on explant. UIP is the predominant histopathology in patients with SSc receiving a lung transplant, with many patients concurrently having both NSIP and UIP or showing progression from NSIP to UIP over time before transplant.
线粒体,衰老和细胞衰老:对硬皮病的影响。
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