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类风湿关节炎患者肺部、肝脏和肾脏同时发生结节性增生病,间质性肺炎急性加重:尸检病例报告。

Simultaneous occurrence of accelerated nodulosis in lungs, liver, and kidneys, and acute exacerbation of interstitial pneumonia in a patient with rheumatoid arthritis: an autopsy case report.

机构信息

Department of Rheumatology, Mitsui Memorial Hospital, 1 Kanda-izumi-cho Chiyoda-ku, Tokyo, 101-8643, Japan.

Center for Rheumatology and Joint Surgery, Kawakita General Hospital, Tokyo, Japan.

出版信息

BMC Pulm Med. 2022 Jan 5;22(1):10. doi: 10.1186/s12890-021-01806-x.

Abstract

BACKGROUND

Accelerated nodulosis (ARN) is a rare variant of rheumatoid nodules (RNs) that is characterized by a rapid onset or the worsening of RNs. It generally develops at the fingers in patients with rheumatoid arthritis (RA) receiving methotrexate (MTX). Few case reports have described ARN at an extracutaneous location.

CASE PRESENTATION

An elderly patient with long-standing RA was admitted to our hospital with acute respiratory failure. Computed tomography upon admission showed diffuse ground-glass opacities superimposed with subpleural reticular shadowing and honeycombing and multiple nodules in the lungs and liver. Despite the discontinuation of MTX and introduction of an immunosuppressive regimen with pulse methylprednisolone followed by a tapering dose of prednisolone and intravenous cyclophosphamide, the patient died due to the acute exacerbation (AE) of RA-related interstitial lung disease (ILD) following the parallel waxing and waning of a diffuse interstitial shadow and pulmonary and liver nodules. At autopsy, RNs were scattered throughout both lung fields in addition to extensive interstitial changes. RNs were also detected in the liver and kidneys. The foci of cryptococcosis were mainly identified in alveolar spaces. Based on the clinical and pathological findings, these nodules were most consistent with ARN because of acute increases in the size and number of previously detected pulmonary nodules.

CONCLUSION

The present case is noteworthy because ARN was concurrently detected in multiple internal organs and may be associated with the AE of RA-related ILD.

摘要

背景

加速结节形成(ARN)是类风湿结节(RNs)的一种罕见变异,其特征为 RNs 的快速发作或恶化。它通常发生在接受甲氨蝶呤(MTX)治疗的类风湿关节炎(RA)患者的手指上。少数病例报告描述了位于皮肤外的 ARN。

病例介绍

一位患有长期 RA 的老年患者因急性呼吸衰竭而入院。入院时的计算机断层扫描显示弥漫性磨玻璃影伴胸膜下网状影和蜂巢影,肺部和肝脏有多发性结节。尽管停用了 MTX,并采用脉冲甲基强的松龙联合逐渐减量的泼尼松龙和静脉环磷酰胺的免疫抑制方案治疗,但由于 RA 相关间质性肺病(ILD)的急性加重(AE),患者最终死亡,ILD 的表现为弥漫性间质影以及肺部和肝脏结节的平行消长。尸检时,除了广泛的间质改变外,双肺野还散布着 RNs。肝脏和肾脏也发现了 RNs。隐球菌病的病灶主要位于肺泡腔。基于临床和病理发现,这些结节最符合 ARN,因为先前发现的肺部结节的大小和数量急性增加。

结论

本病例值得注意,因为在多个内脏器官中同时检测到 ARN,并且可能与 RA 相关 ILD 的 AE 有关。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2e53/8728930/2f6952b6536c/12890_2021_1806_Fig1_HTML.jpg

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