IgG4-Related Systemic Disease and Lymphoplasmacytic Aortitis
IgG4-Related Systemic Disease and Lymphoplasmacytic Aortitis
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DOI：
10.1002/art.24798
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发表时间：
2009-10-01
影响因子：
--
通讯作者：
Stone, James R.
中科院分区：
文献类型：
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作者：
Stone, John H.;Khosroshahi, Arezou;Stone, James R.
我们在此描述了一个病人谁制定了升主动脉夹层的IgG 4相关的系统性疾病，连接IgG 4相关的系统性疾病与一个新认识的子集的非感染性乳腺炎。在主动脉手术时，在患者主动脉中检测到透壁淋巴浆细胞浸润，主要炎症灶位于中膜内。免疫组织化学研究表明，病变中>50%的浆细胞对IgG 4染色。通过原位杂交，浆细胞显示κ和λ轻链的多型染色，与多克隆浆细胞浸润一致。血清学评价显示，患者的IgG 4水平升高近10倍。主动脉手术前4年，患者接受了纵隔淋巴结活检。淋巴结的复查显示与IgG 4相关的全身性疾病一致的特征，这在最初的活检时未被识别。糖皮质激素治疗IgG 4相关的全身性疾病产生了迅速的反应。认识到IgG4相关的全身性疾病可累及升主动脉和降主动脉，表明需要改变对特发性腹膜炎的认识。该病例为非感染性乳腺炎的短期和长期管理提供了新的潜在考虑，因为IgG 4相关的全身性疾病对糖皮质激素治疗的反应通常良好，无需额外治疗。治疗乳腺炎可防止IgG 4相关全身性疾病进展至累及其他器官。在所有被判定为病因不明的乳腺炎患者中，应考虑IgG 4相关全身性疾病。
We describe herein a patient who developed a dissection of the ascending aorta in the setting of IgG4-related systemic disease, linking IgG4-related systemic disease with a newly-recognized subset of noninfectious aortitis. At the time of aortic surgery, a transmural lymphoplasmacytic infiltrate was detected in the patient's aorta, with a principal focus of inflammation within the media. Immunohistochemical studies demonstrated that >50% of the plasma cells in the lesion stained for IgG4. By in situ hybridization, the plasma cells showed polytypic staining for kappa and lambda light chains, consistent with a polyclonal plasma cell infiltrate. Serologic evaluation revealed that the patient's IgG4 levels were elevated nearly 10-fold. Four years before aortic surgery, the patient had undergone a mediastinal lymph node biopsy. Reexamination of the lymph node revealed features consistent with IgG4-related systemic disease, which had not been recognized at the time of the original biopsy. Glucocorticoid therapy for the IgG4-related systemic disease yielded a prompt response. Recognition that IgG4-related systemic disease can involve the ascending as well as the descending abdominal aorta indicates the need for a change in the way idiopathic aortitis is regarded. This case offers new potential considerations for short- and long-term management of noninfectious aortitis, because of the frequent good response of IgG4-related systemic (disease to glucocorticoid treatment without additional therapy. Treatment of the aortitis may prevent progression of the IgG4-related systemic disease to involvement of other organs. IgG4-related systemic disease should be considered in all patients with aortitis judged to be of unknown etiology.