Suppurative necrotizing granulomatous lymphadenitis in adult-onset Still's disease: a case report.
Suppurative necrotizing granulomatous lymphadenitis in adult-onset Still's disease: a case report.
复制标题
DOI：
10.1186/1752-1947-6-354
复制
发表时间：
2012-10-18
影响因子：
1
通讯作者：
Gogos C
中科院分区：
文献类型：
--
作者：
Assimakopoulos SF;Karamouzos V;Papakonstantinou C;Zolota V;Labropoulou-Karatza C;Gogos C
淋巴结病见于约65%的成人斯蒂尔病患者，其组织学特征为强烈的皮质旁免疫母细胞增生。成人斯蒂尔病以前没有被描述为化脓性坏死性肉芽肿性淋巴结炎的病因。我们描述了一个27岁的希腊男子谁表现出长期发热，腹痛，炎症标志物增加，偶发性皮疹和肠系膜淋巴结病的组织学特征的坏死性肉芽肿性腺炎与中央化脓。疾病发作的特征是全身炎症反应综合征，对皮质类固醇有立即的临床-实验室反应，但患者需要以高于12 mg/天的剂量延长甲泼尼龙给药以控制疾病。经过广泛的诊断性检查，排除了任何感染性、恶性、风湿性或自身炎症性疾病，患者被诊断为成人型斯蒂尔病。该患者目前接受4 mg甲泼尼龙、每天100 mg阿那白滞素和每周连续两天7.5mg甲氨蝶呤治疗，并在六个月内完全缓解疾病。据我们所知，这是第一次报告化脓性坏死性肉芽肿性淋巴结炎归因于成人发病斯蒂尔病。本病例表明，化脓性坏死性肉芽肿性淋巴结炎的发现不应阻止考虑成人发病斯蒂尔病作为一个潜在的诊断在一个兼容的临床背景下，但是，排除其他诊断是一个先决条件。
Lymphadenopathy is found in about 65% of patients with adult-onset Still’s disease and is histologically characterized by an intense, paracortical immunoblastic hyperplasia. Adult-onset Still’s disease has not been previously described as an etiology of suppurative necrotizing granulomatous lymphadenitis. We describe a 27-year-old Greek man who manifested prolonged fever, abdominal pain, increased inflammatory markers, episodic skin rash and mesenteric lymphadenopathy histologically characterized by necrotizing granulomatous adenitis with central suppuration. Disease flares were characterized by systemic inflammatory response syndrome with immediate clinico-laboratory response to corticosteroids but the patient required prolonged administration of methylprednisolone at a dose of above 12mg/day for disease control. After an extensive diagnostic work-up, which ruled out any infectious, malignant, rheumatic or autoinflammatory disease the patient was diagnosed as having adult-onset Still’s disease. The patient is currently treated with 4mg of methylprednisolone, 100mg of anakinra daily and methotrexate 7.5mg for two consecutive days per week and exerts full disease remission for six months. To the best of our knowledge this is the first report of suppurative necrotizing granulomatous lymphadenitis attributed to adult-onset Still’s disease. This case indicates that the finding of a suppurative necrotizing granulomatous lymphadenitis should not deter the consideration of adult-onset Still’s disease as a potential diagnosis in a compatible clinical context; however, the exclusion of other diagnoses is a prerequisite.