颞动脉炎(TA)是一种大血管血管炎,主要见于老年患者。继发于慢性炎症的淀粉样蛋白A(AA)淀粉样变性诱导多器官功能障碍,包括胃肠道功能障碍。在这里,我们介绍了一例TA并发AA淀粉样变性,对口服和静脉注射类固醇耐药。一位80岁的男性有新发头痛史,颌骨跛行,扩张的颞动脉被转诊到我们部门。一入场,患者表现为两个太阳穴动脉的压痛和皮下颞叶结节。结节的超声检查显示右颞动脉周围有无回声血管周围晕。根据TA的诊断,开始大剂量泼尼松龙治疗.然而,患者出现复发性腹痛和难治性腹泻。由于难治性腹泻的起源不清楚,广泛的工作,包括十二指肠粘膜活检,已执行。内窥镜检查显示十二指肠慢性炎症。十二指肠粘膜活检样品的免疫组织化学分析显示AA淀粉样蛋白沉积导致AA淀粉样变性的诊断。托珠单抗(TCZ)给药后,难治性腹泻减少;然而,患者在开始TCZ给药后1个月死于肠穿孔.胃肠道受累是本病例AA淀粉样变性的主要临床表现。这个病例突出了肠活检筛查淀粉样沉积的重要性,患者的原因不明的胃肠道症状,即使在最近的大血管血管炎发作中。在目前的情况下,SAA1.3等位基因的携带可能导致AA淀粉样变性与TA的罕见关联。
Temporal arteritis (TA) is a large-vessel vasculitis mostly seen in older patients. Amyloid A (AA) amyloidosis secondary to a chronic inflammation induces multiple organ dysfunctions, including a dysfunction of the gastrointestinal tract. Herein, we present a
case of TA complicated by AA amyloidosis that was resistant to oral and intravenous steroids. An 80-year-old man with a history of new-onset headache, jaw claudication, and distended temporal arteries was referred to our department. On admission, the patient presented with tenderness and a subcutaneous temporal nodule in both temple arteries. Ultrasonography of the nodule revealed an anechoic perivascular halo surrounding the right temporal artery. Following the diagnosis of TA, high-dose prednisolone therapy was initiated. However, the patient presented with recurrent abdominal pain and refractory diarrhea. Due to the unclear origin of refractory diarrhea, an extensive workup, including biopsy of the duodenal mucosa, was performed. Endoscopy revealed chronic inflammation in the duodenum. Immunohistochemical analysis of duodenal mucosal biopsy samples revealed AA amyloid deposition resulting in the diagnosis of AA amyloidosis. After tocilizumab (TCZ) administration, refractory diarrhea reduced; however, the patient died of intestinal perforation 1 month after the start of TCZ administration. Gastrointestinal involvement was the main clinical manifestation of AA amyloidosis in the present
case. This
case highlights the importance of bowel biopsy screening for amyloid deposition in patients with unexplained gastrointestinal tract symptoms, even in a recent onset of large-vessel vasculitis. In the present
case, the carriage of the SAA1.3 allele likely contributed to the rare association of AA amyloidosis with TA.