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IgG4-Related Tumefactive Lesions at the Pulmonary Artery Causing Stenosis of Bilateral Primary Branches and Resultant Pulmonary Hypertension

机译:IgG4相关的肺动脉肺动脉造成双侧初级分支狭窄和所得肺动脉高压的鼻腔

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摘要

IgG4-related disease (IgG4-RD) is a newly recognized but rare entity involving multiple organs, with autoimmune pancreatitis, retroperitoneal mass, and the inflammation of glands being typical in most cases. IgG4-related perivascular lesions, although uncommon, have been increasingly reported in recent years. Diagnosis of IgG4-RD relies on comprehensive consideration of characteristic histopathological and immunostaining results, clinical and imaging findings, and serological results according to several widely recognized diagnostic criteria. This benign disorder frequently presenting tumefactive lesions should be distinguished from malignancy and other inflammatory mimics. Here we report a case of tumefactive mass at the bifurcation of the pulmonary trunk causing stenosis of the proximal left and right pulmonary artery (PA) and resultant pulmonary hypertension (PH). Bypass from the PA trunk to the right branch distal to stenosis was performed to resolve the obstructive hemodynamic disturbance and PH. Glucocorticoid monotherapy was performed after a diagnosis of definite IgG4-RD. Longitudinal disease activity assessment via imaging modalities, serological parameters, and IgG4-RD responder index verified no relapse during follow-up and the validity of the treatment strategy.
机译:IgG4相关疾病(IgG4-Rd)是一种新公认但罕见的实体,涉及多个器官,具有自身免疫性胰腺炎,腹膜后肿块,以及大多数情况下典型的腺体炎症。近年来,虽然罕见地报道了IgG4相关的血管外病变。 IGG4-RD的诊断依赖于综合考虑特性组织病理学和免疫染色结果,临床和成像结果,以及根据几种广泛认可的诊断标准的血清学结果。这种良性障碍经常呈现稀释病变应与恶性肿瘤和其他炎症模仿区分开来。在这里,我们在肺躯干分叉引起近端左右肺动脉(PA)的狭窄和肺动脉高压(pH)的牙线分叉的肺躯干分叉的情况下报告了稀释物质的情况。旁路从PA后备箱到右侧分支到狭窄的右侧分支,以解决阻塞性血流动力学干扰和pH。糖皮质激素单疗法在诊断到明确的IgG4-Rd后进行。通过成像方式,血清学参数和IgG4-RD响应者指数的纵向疾病活动评估在后续行动和治疗策略的有效性期间核实复发。

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