PDF(Pubmed)
Abstract：
Chronic beryllium disease (CBD), or berylliosis, is an interstitial lung disease caused by the chronic inhalation of finely particulate beryllium, frequently mistaken for sarcoidosis. It is rarely associated with skin nodular lesions, asymptomatic granulomatous hepatitis or calcium nephrolithiasis. To date, it has never been reported as a diffused multi-organ granulomatous disease. A 60-year-old Pakistani man, a former excavation worker with ancient history of suspected sarcoidosis, underwent a left nephroureterectomy for suspected papillary kidney carcinoma. The histopathological analysis showed a benign non-necrotic granulomatous infiltration of the renal pelvis and ureter. Six months later, he suffered from two consecutive episodes of acute kidney failure. Bladder biopsies found similar noncaseous granulomatosis and kidney biopsies showed interstitial nephritis. Known for suspected asthma, sleep apnea, and usual interstitial pneumonia, the patient would regularly consult for episodes of pyrexia, chills, nocturnal coughing, and wheezing. As kidney function gradually worsened, he ultimately started hemodialysis and was transferred to our facility. A positive blood beryllium lymphocyte proliferation test confirmed the diagnosis of CBD. This original report is the first description of multi-organ berylliosis with diffused urothelial granulomatosis and pseudo-tumor. The patient\'s pulmonary disease is minimal compared with renal and urinary tract involvement, eventually responsible for end-stage kidney disease. Berylliosis usually responds to glucocorticoids. This case report highlights the importance of evoking the diagnosis of CBD in the presence of any granulomatosis, even extra-thoracic, especially if associated with pulmonary symptoms, however atypical.
摘要：
慢性铍病（CBD），或者铍病,是一种由慢性吸入细颗粒铍引起的间质性肺病，经常被误认为结节病。它很少与皮肤结节性病变有关，无症状肉芽肿性肝炎或钙肾结石。迄今为止,它从未被报道为弥漫性多器官肉芽肿病。一名60岁的巴基斯坦男子,一名有怀疑结节病历史的前挖掘工人，怀疑乳头状肾癌接受了左肾输尿管切除术。组织病理学分析显示肾盂和输尿管良性非坏死性肉芽肿浸润。六个月后,他连续两次急性肾衰竭发作。膀胱活检发现类似的非干酪样肉芽肿病，肾脏活检显示间质性肾炎。因疑似哮喘而闻名，睡眠呼吸暂停,和常见的间质性肺炎,患者会定期咨询发热发作，发冷,夜间咳嗽,还有喘息.随着肾功能逐渐恶化，他最终开始血液透析并被转移到我们的机构.血铍淋巴细胞增殖试验阳性证实了CBD的诊断。这份原始报告是对多器官铍病伴有弥漫性尿路上皮肉芽肿和假性肿瘤的首次描述。与肾脏和泌尿道受累相比，患者的肺部疾病轻微，最终导致终末期肾病。铍病通常对糖皮质激素有反应。此病例报告强调了在存在任何肉芽肿的情况下唤起CBD诊断的重要性，甚至是胸外,特别是如果与肺部症状有关，然而非典型。
