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持续性实变的罕见病例:特发性淋巴细胞间质性肺炎。

An unusual case of persistent consolidation: Idiopathic lymphoid interstitial pneumonia.

作者信息

Caterson Harriet J, Kim Sewon, Zaborowski Matthew, Harden Michael, Hibbert Michael

机构信息

Department of Respiratory Medicine Royal North Shore Hospital St Leonards New South Wales Australia.

Department of Anatomical Pathology Royal North Shore Hospital St Leonards New South Wales Australia.

出版信息

Respirol Case Rep. 2024 Jun 10;12(6):e01408. doi: 10.1002/rcr2.1408. eCollection 2024 Jun.

Abstract

Lymphocytic interstitial pneumonia (LIP) is a rare but largely benign interstitial lung disease, most frequently associated with HIV and autoimmune conditions. It is infrequently found to be an idiopathic condition. Diagnosis is complex and can require numerous invasive tests as evidenced in the case presented. The diagnosis is made from a combination of clinical, radiological, and histological features but the unusual radiological and clinical features meant diagnosis in our case required surgical biopsy. There is minimal evidence around best treatment although largely involves targeting the underlying cause. There is a small risk of transformation to lymphoma and fibrosis. Immunosuppression with steroids is the most common therapeutic strategy however in our case the radiographic changes spontaneously resolved. We present a case of an immunocompetent male presenting with significant radiological and histopathological findings of LIP, without significant symptomatology, that spontaneously resolved without intervention suggesting a monitoring approach may be a valid management strategy.

摘要

淋巴细胞间质性肺炎(LIP)是一种罕见但大多为良性的间质性肺病,最常与HIV和自身免疫性疾病相关。它很少被发现是特发性疾病。诊断很复杂,可能需要进行多项侵入性检查,如本文所述病例所示。诊断是根据临床、放射学和组织学特征综合做出的,但该病例不寻常的放射学和临床特征意味着需要手术活检来确诊。关于最佳治疗的证据很少,尽管主要是针对潜在病因。有小风险会转化为淋巴瘤和纤维化。使用类固醇进行免疫抑制是最常见的治疗策略,然而在我们的病例中,影像学改变自行消退。我们报告一例免疫功能正常的男性,其有LIP显著的放射学和组织病理学表现,但无明显症状,未经干预自行消退,提示监测方法可能是一种有效的管理策略。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eb8b/11164566/4b6ac54562b8/RCR2-12-e01408-g002.jpg

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