方法:一名63岁男子出现发烧,胸痛,减肥,弥漫性淋巴结病,还有大量的胸腔积液.广泛的实验室和放射学调查可能的自身免疫,传染性,血液学,和肿瘤条件都是阴性的。淋巴结活检显示肉芽肿性坏死性淋巴结炎,怀疑肺结核。尽管从未分离出结核分枝杆菌(MT),结核菌素皮肤试验结果为阴性,诊断为肺外结核并开始抗结核治疗.尽管严格遵守5个月的治疗,他回到急诊室,抱怨发烧,胸痛和胸腔积液;全身CT和PET扫描显示新的播散性结节巩固进展。
方法:对MT和其他微生物的显微镜和文化搜索再次导致尿液阴性,凳子,血,胸腔积液,和脊柱病变活检。因此,我们开始考虑坏死性肉芽肿病的替代诊断,包括耐多药结核病,韦格纳肉芽肿病,ChurgStrauss综合征,类风湿性关节炎的坏死结节,淋巴瘤样肉芽肿病和坏死性结节病肉芽肿病(NSG)。已经拒绝了其他自身免疫,血液学,和肿瘤性疾病,NSG得出了最一致的假设。因此,我们与专家一起重新检查了暗示结节病非典型表现的组织学标本。开始类固醇治疗,实现症状改善。
结论:结节病是一种罕见的疾病,对诊断具有挑战性,由于其临床表现的可变性,经常模仿其他疾病,如播散性结核病。高度怀疑和有经验的解剖病理学实验室对于最终诊断至关重要。
METHODS: A 63-year-old man presented with fever, thoracalgia, weight loss, diffuse lymphadenopathy, and a massive pleural effusion. Extensive laboratory and radiologic investigations for possible autoimmune, infectious, hematologic, and neoplastic conditions all resulted negative. A lymph node biopsy showed a granulomatous necrotizing lymphadenitis, suspicious for tuberculosis. Although mycobacterium tuberculosis (MT) was never isolated and tuberculin skin test resulted negative, diagnosis of extrapulmonary tuberculosis was made and anti-tubercular therapy was started. Despite the strict adherence to 5 months of treatment, he returned to the emergency ward complaining of fever, chest pain and pleural effusion; total-body CT and PET scans demonstrated a progression of new disseminated nodular consolidations.
METHODS: Microscopic and cultural search for MT and other micro-organisms resulted again negative on urine, stool, blood, pleural fluid, and spinal lesion biopsy. We therefore started considering alternative diagnosis for necrotizing granulomatosis, including multidrug-resistant tuberculosis, Wegener granulomatosis, Churg Strauss syndrome, necrobiotic nodules of rheumatoid arthritis, lymphomatoid granulomatosis and Necrotizing Sarcoid Granulomatosis (NSG). Having already rejected other autoimmune, hematological, and neoplastic disorders, NSG resulted the most consistent hypothesis. With an expert we thus re-examined histological specimens that were suggestive for an atypical presentation of sarcoidosis. Steroid therapy was initiated, achieving symptoms improvement.
CONCLUSIONS: Sarcoidosis is a rare condition that can be challenging to diagnose, due to its variability in clinical presentation, often mimicking alternative conditions like disseminated tuberculosis. A high degree of suspicion and an experienced lab in anatomical pathology are essential for final diagnosis.