Mohankumar Shivaprasad Pannasamudra, Das Samannay, Likitha P, Naranje Priyanka, Jana Manisha, Gupta Saurabh Kumar, Bagri Narendra Kumar
Department of Paediatrics, AIIMS, New Delhi, India.
Department of Radiodiagnosis and Interventional Radiology, AIIMS, New Delhi, India.
Rheumatol Int. 2023 Dec;43(12):2327-2331. doi: 10.1007/s00296-023-05388-1. Epub 2023 Jul 11.
Polyarteritis nodosa (PAN) is a medium-vessel vasculitis presenting with cutaneous and multisystem involvement with considerable morbidity. The necrotizing vasculitis in PAN typically involves renal, celiac, and mesenteric vascular beds. Coronary artery involvement is a characteristic feature of Kawasaki disease, another medium-vessel vasculitis; however, it has been rarely reported with PAN. Here, we present 2 cases with PAN involving coronaries mimicking Kawasaki disease. A 3.5-year-old boy with classical features of Kawasaki disease with giant coronary aneurysm refractory to IVIg, methylprednisolone, infliximab presented with persistent rise in inflammatory markers and gastrointestinal bleeding. Digital subtraction angiography (DSA) revealed celiac artery branches stenosis and beading suggestive of PAN. Another 2-year-old girl presented with persistent fever, abdominal pain, and distension. She had hypertension, hepatomegaly, and splenomegaly on examination. Echocardiography revealed multiple coronary aneurysms and DSA revealed numerous renal artery aneurysms. Coronary aneurysm although is a rare presentation of childhood PAN, and can mimic Kawasaki disease. Although both are medium-vessel vasculitis differentiation between these two entities is pivotal, as there are differences in treatment modalities, duration of immunomodulatory therapy, and the outcome. This manuscript describes the salient differences which can help differentiate PAN masquerading as Kawasaki disease at initial presentation.
结节性多动脉炎(PAN)是一种累及皮肤和多系统的中血管血管炎,发病率较高。PAN中的坏死性血管炎通常累及肾、腹腔和肠系膜血管床。冠状动脉受累是另一种中血管血管炎——川崎病的特征性表现;然而,PAN累及冠状动脉的情况鲜有报道。在此,我们报告2例PAN累及冠状动脉且酷似川崎病的病例。一名3.5岁男孩具有川崎病的典型特征,伴有巨大冠状动脉瘤,对静脉注射免疫球蛋白(IVIg)、甲泼尼龙、英夫利昔单抗治疗无效,出现炎症标志物持续升高和胃肠道出血。数字减影血管造影(DSA)显示腹腔动脉分支狭窄和串珠样改变,提示PAN。另一名2岁女孩表现为持续发热、腹痛和腹胀。检查发现她有高血压、肝肿大和脾肿大。超声心动图显示多个冠状动脉瘤,DSA显示多个肾动脉瘤。冠状动脉瘤虽然是儿童PAN的罕见表现,且可酷似川崎病。尽管两者都是中血管血管炎,但区分这两种疾病至关重要,因为在治疗方式、免疫调节治疗持续时间和预后方面存在差异。本文描述了一些显著差异,有助于在初次就诊时鉴别伪装成川崎病的PAN。