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Fluorine-18-fluorodeoxyglucose PET/CT rare finding of a unique multiorgan involvement of Wegeners granulomatosis

机译:氟18-氟脱氧葡萄糖PET / CT罕见发现韦格纳肉芽肿独特的多器官参与

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摘要

Wegener's granulomatosis (WG) is an uncommon autoimmune disorder, which mainly involves the blood vessels, kidneys and respiratory tract. We report an interesting case of WG with unusual multiorgan involvement in a young male who presented with a short history of right-sided otalgia, nasal obstruction and a right parotid mass. His initial CT and MRI scans showed a large parotid mass with features suggestive of malignancy with bilateral cavitating pulmonary nodules suggesting metastatic disease. The imaging-based differential diagnosis was squamous cell carcinoma or adenoid cystic carcinoma. The microscopic findings on ultrasound-guided biopsy of the parotid mass were, surprisingly, those of acute necrotising granulomatous inflammation with some features suggestive of a vasculitic process. A multidisciplinary team discussion and further investigation resulted in the additional findings of haematuria, raised erythrocyte sedimentation rate and positive serum cytoplasmic anti-neutrophil cytoplasmic antibody test, which led to the diagnosis of WG. Subsequently, the patient developed acute urinary retention owing to gross prostatic enlargement related to further disease involvement, which was confirmed with a positive biopsy. Fluorine-18-fluorodeoxyglucose (18F-FDG) positron emission tomography (PET)/CT scan showed disease distribution at the right maxillary sinusasal cavity, right parotid, mediastinum, lungs and prostate. To our knowledge, this is the first reported 18F-FDG PET/CT case with multiorgan involvement in a single WG patient. The patient has improved both clinically and on imaging after appropriate treatment with immunosuppressive therapy and steroids. Although 18F-FDG PET/CT imaging did not actually alter the management of this patient, it can help to establish the disease distribution and guide the biopsy.
机译:韦格纳肉芽肿病(WG)是一种罕见的自身免疫性疾病,主要涉及血管,肾脏和呼吸道。我们报告了一个有趣的WG病例,该异常病例是一名年轻男性的异常多器官受累,该男性表现出较短的右侧耳痛,鼻塞和右侧腮腺肿块病史。他的最初CT和MRI扫描显示腮腺肿块很大,具有恶性特征,双侧空洞性肺结节提示转移性疾病。基于影像学的鉴别诊断为鳞状细胞癌或腺样囊性癌。超声引导下腮腺肿块的活组织检查的显微镜结果令人惊讶地是急性坏死性肉芽肿性炎症,具有某些提示血管形成过程的特征。多学科小组的讨论和进一步调查导致了血尿的其他发现,血沉增加和血清胞浆抗中性粒细胞胞浆抗体检测阳性,从而导致了WG的诊断。随后,由于与进一步疾病侵袭相关的总体前列腺肿大,患者出现了急性尿retention留,活检证实了这一点。氟18-氟脱氧葡萄糖(18F-FDG)正电子发射断层显像(PET)/ CT扫描显示疾病分布在右上颌窦/鼻腔,右腮腺,纵隔,肺和前列腺。据我们所知,这是首例报道的单个WG患者中多器官受累的18F-FDG PET / CT病例。经过免疫抑制疗法和类固醇的适当治疗后,患者的临床和影像学均得到改善。尽管18F-FDG PET / CT成像实际上并未改变该患者的治疗方法,但它可以帮助建立疾病分布并指导活检。

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