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Twenty-year-old patient with polyarthritis since childhood showing cysts and ground glass attenuation on HRCT
Thorax ( IF 10 ) Pub Date : 2024-04-01 , DOI: 10.1136/thorax-2023-220798
Marília da Cruz Fagundes , Thais Bianco , Daniella Porfírio Nunes , Tayza Katelline Danilau Ostroski , Guilherme das Posses Bridi , Alexandre Melo Kawassaki , Carmen Silvia Valente Barbas , Leonardo Oliveira Mendonça , Samar Freschi Barros , Jorge Kalil , Anthony K Shum , Dante Luiz Escuissato

We present a case of a 20-year-old woman reporting polyarthritis, muscle weakness, dyspnoea and multiple intensive care unit admissions due to respiratory failure, without haemoptysis, since 4 months of age. The mother had systemic erythematous lupus. On physical examination, digital clubbing and mild desaturation were found. Laboratory tests demonstrated 1:640 nuclear homogeneous pattern antinuclear antibodies (ANA) and erythrocyte sedimentation rate (ESR) of 61 mm. Spirometry exhibited forced vital capacity of 53%. High-resolution CT (HRCT) showed cysts and ground glass with reticular infiltrates (figure 1A-D). In 2006, she underwent a pulmonary biopsy, which revealed follicular bronchiolitis (figure 2A-D), without features of haemorrhage on biopsy such as haemosiderin laden macrophages. Figure 1 HRCT appearances. (A, B) Multiple well-circumscribed cysts in the parenchyma (arrows) and parenchymal reticulation with architectural distortion (arrowheads); (C, D) scattered ground glass opacities, more extensive in the lower lobes (circles). These findings correspond to an interstitial lung disease of unclassifiable pattern with parenchymal cysts. HRCT, high-resolution CT. Figure 2 Lung biopsy revealing. (A) Pulmonary parenchyma with lymphoid aggregates; (B) peribronchiolar (arrowhead) lymphoid aggregate with prominent germinative centre (arrow); (C) proliferation …

中文翻译:

20岁自幼患有多关节炎的患者HRCT显示囊肿和毛玻璃衰减

我们介绍了一名 20 岁女性的病例,她自 4 个月大起患有多发性关节炎、肌肉无力、呼吸困难,并因呼吸衰竭多次入院重症监护室,但没有咯血。母亲患有系统性红斑狼疮。体检时发现杵状指和轻度饱和度下降。实验室检测显示1:640核同质型抗核抗体(ANA)和红细胞沉降率(ESR)为61毫米。肺活量测定显示用力肺活量为 53%。高分辨率 CT (HRCT) 显示囊肿和毛玻璃伴网状浸润(图 1A-D)。2006年,她接受了肺活检,结果显示滤泡性细支气管炎(图2A-D),活检没有出血特征,例如富含含铁血黄素的巨噬细胞。图1 HRCT表现。(A, B) 实质内多个边界清楚的囊肿(箭头)和伴有结构扭曲的实质网状结构(箭头);(C、D) 散在毛玻璃样混浊,在下叶更广泛(圆圈)。这些发现对应于具有实质囊肿的无法分类模式的间质性肺病。HRCT,高分辨率CT。图2 肺活检显示。(A) 肺实质与淋巴聚集体;(B) 细支气管周围(箭头)淋巴聚集体,具有突出的萌发中心(箭头);(C) 扩散……
更新日期:2024-03-15
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