IgG4-related disease in Italy: clinical features and outcomes of a large cohort of patients
IgG4-related disease in Italy: clinical features and outcomes of a large cohort of patients
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DOI：
10.3109/03009742.2015.1055796
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发表时间：
2016-03-03
影响因子：
2.1
通讯作者：
Della-Torre, E.
中科院分区：
文献类型：
--
作者：
Campochiaro, C.;Ramirez, G. A.;Della-Torre, E.
目的：描述的临床特征，治疗反应，并随访了一个大的意大利患者的免疫球蛋白（IG）G4相关疾病（IgG 4-RD）的队列提到一个单一的三级care centre.Method：临床，实验室，组织学和影像学特征进行了回顾性分析。根据国际共识指南和IgG 4-RD的综合诊断标准，IgG 4-RD被分类为明确的“或可能的”。结果：41例患者（15例女性，26例男性）纳入本研究，其中26例为明确的“IgG 4-RD”，15例为可能的“IgG 4-RD”。诊断时的中位年龄为62岁。中位随访时间为36个月（IQR 24-51）。30%的患者有特应性病史。胰腺、腹膜后和大唾液腺是最常见的受累器官。68%的病例血清IgG 4水平升高。36例患者最初接受糖皮质激素（GC）治疗以诱导缓解。IgG 4-RD RI从基线时的中位数7.8降至治疗1个月后的2.9。41例患者中有19例（46%）复发，需要额外的免疫抑制药物来维持长期缓解。少数患者发生多次发作。一个单一的情况下，眼眶假瘤并没有回应药物治疗，并进行了手术debulking.Conclusions：IgG 4-RD是一种难以捉摸的炎症性疾病被认为是孤立或多个tumefective病变的鉴别诊断。在大多数情况下，皮质类固醇和免疫抑制药物可以实现长期疾病控制。
Objectives: To describe the clinical features, treatment response, and follow-up of a large cohort of Italian patients with immunoglobulin (Ig)G4-related disease (IgG4-RD) referred to a single tertiary care centre.Method: Clinical, laboratory, histological, and imaging features were retrospectively reviewed. IgG4-RD was classified as definite' or possible' according to international consensus guidelines and comprehensive diagnostic criteria for IgG4-RD. Disease activity was assessed by means of the IgG4-RD Responder Index (IgG4-RD RI).Results: Forty-one patients (15 females, 26 males) were included in this study: 26 with definite' IgG4-RD and 15 with possible' IgG4-RD. The median age at diagnosis was 62years. The median follow-up was 36 months (IQR 24-51). A history of atopy was present in 30% of patients. The pancreas, retroperitoneum, and major salivary glands were the most frequently involved organs. Serum IgG4 levels were elevated in 68% of cases. Thirty-six patients were initially treated with glucocorticoids (GCs) to induce remission. IgG4-RD RI decreased from a median of 7.8 at baseline to 2.9 after 1month of therapy. Relapse occurred in 19/41 patients (46%) and required additional immunosuppressive drugs to maintain long-term remission. Multiple flares occurred in a minority of patients. A single case of orbital pseudotumour did not respond to medical therapy and underwent surgical debulking.Conclusions: IgG4-RD is an elusive inflammatory disease to be considered in the differential diagnosis of isolated or multiple tumefactive lesions. Long-term disease control can be achieved with corticosteroids and immunosuppressive drugs in the majority of cases.