IgG4-related disease: a clinical perspective
IgG4-related disease: a clinical perspective
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DOI：
10.1093/rheumatology/kez667
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发表时间：
2020-05-01
期刊：
影响因子：
5.5
通讯作者：
Vaglio, Augusto
中科院分区：
文献类型：
--
作者：
Maritati, Federica;Peyronel, Francesco;Vaglio, Augusto
IgG 4相关疾病（IgG 4-RD）是最近发现的一种纤维炎性疾病，几乎可以影响任何器官。常见的表现包括唾液腺和泪腺肿大、眼眶疾病、自身免疫性胰腺炎、腹膜后纤维化和肾小管间质性肾炎。主要的组织病理学特征是密集的、多克隆的、富含IgG 4+浆细胞的淋巴浆细胞浸润、Storiform纤维化和闭塞性静脉炎。IgG 4-RD的确切致病机制尚不清楚。CD 4 + T和B细胞，包括表达IgG 4的浆母细胞，构成主要的炎性细胞群，并被认为引起器官损伤和组织纤维化。该疾病的诊断可能具有挑战性，应基于特定的组织病理学结果，典型的实验室和放射学方面以及适当的临床背景。IgG 4-RD的一线治疗基于糖皮质激素，通常有效。然而，也发现利妥昔单抗诱导的B细胞耗竭可诱导类固醇耐药疾病的缓解，或已被用作复发性疾病的类固醇保留剂。本文综述了IgG 4-RD的临床和治疗方面的最新进展。
IgG4-related disease (IgG4-RD) is a recently recognized fibro-inflammatory disorder that can affect almost any organ. Common presentations include major salivary and lacrimal gland enlargement, orbital disease, autoimmune pancreatitis, retroperitoneal fibrosis and tubulointerstitial nephritis. The main histopathological features are a dense, polyclonal, lymphoplasmacytic infiltrate rich in IgG4+ plasma cells, storiform fibrosis and obliterative phlebitis. The precise pathogenic mechanisms of IgG4-RD are still unclear. CD4+ T and B cells, including IgG4-expressing plasmablasts, constitute the major inflammatory cell populations and are believed to cause organ damage and tissue fibrosis. The diagnosis of the disease may be challenging and should be based on specific histopathological findings, typical laboratory and radiological aspects and an appropriate clinical context. The first-line treatment of IgG4-RD is based on glucocorticoids, which are usually efficacious. However, B cell depletion induced by rituximab has also been found to induce remission in steroid-resistant disease or has been used as steroid-sparing agent for relapsing disease. This review provides an update on clinical and therapeutic aspects of IgG4-RD.