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史蒂文斯-约翰逊综合征后出现的严重气流阻塞和嗜酸性粒细胞性肺病。

Severe airflow obstruction and eosinophilic lung disease after Stevens-Johnson syndrome.

作者信息

Shah A P, Xu H, Sime P J, Trawick D R

机构信息

Department of Medicine, Pulmonary and Critical Care Unit, University of Rochester Medical Center, Rochester, NY, USA.

出版信息

Eur Respir J. 2006 Dec;28(6):1276-9. doi: 10.1183/09031936.00036006.

Abstract

Respiratory involvement is a frequent complication of Stevens-Johnson syndrome (SJS). However, there are very few convincing reports of persistent pulmonary sequelae, as demonstrated by spirometry, radiology and pathology. The current study presents a case of a 13-yr-old female with T-cell acute lymphocytic leukaemia who developed persistent, severe, obstructive lung disease following an episode of SJS. A lung biopsy demonstrated bronchiolar submucosal fibrosis consistent with constrictive bronchiolitis, as well as eosinophilic micro-abscesses, which, to the current authors' knowledge, has not been previously described. The present study illustrates specific histopathological features that highlight a possible association between Stevens-Johnson syndrome, constrictive bronchiolitis and eosinophilic micro-abscesses. The eosinophils may be associated with permanent mucosal damage, as seen in the present case, by releasing mediators that have a pro-fibrogenetic role. However, further investigation is warranted.

摘要

呼吸系统受累是史蒂文斯-约翰逊综合征(SJS)的常见并发症。然而,通过肺活量测定、放射学和病理学证实,持续性肺部后遗症的令人信服的报告非常少。本研究报告了1例13岁患T细胞急性淋巴细胞白血病的女性,在发生SJS后出现持续性、严重的阻塞性肺疾病。肺活检显示细支气管黏膜下纤维化符合缩窄性细支气管炎,以及嗜酸性微脓肿,据作者所知,此前尚未有过此类描述。本研究阐明了特定的组织病理学特征,突出了史蒂文斯-约翰逊综合征、缩窄性细支气管炎和嗜酸性微脓肿之间可能存在的关联。嗜酸性粒细胞可能通过释放具有促纤维化作用的介质,如本病例所见,与永久性黏膜损伤有关。然而,仍需进一步研究。

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