川崎病合并巨噬细胞激活综合征:及时诊断和治疗的重要性(附3例报告

Rheumato Pub Date : 2023-09-30 DOI:10.3390/rheumato3040015
Elena Corinaldesi, Marianna Fabi, Ilaria Scalabrini, Elena Rita Praticò, Laura Andreozzi, Francesco Torcetta, Marcello Lanari
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引用次数: 0

摘要

川崎病(Kawasaki disease, KD)是一种急性血管炎,主要影响5岁以下儿童,25%未经治疗的患者会导致冠状动脉改变(CAAs)。巨噬细胞激活综合征(MAS)是一种继发性噬血细胞性淋巴组织细胞增多症(HLH),可使KD的急性、亚急性和慢性期复杂化。我们回顾性分析了意大利北部艾米利亚罗马涅两个儿科住院的3例KD合并MAS患儿。病例1:一名健康的23个月大的女性,完全符合KD的临床标准,在疾病的急性期出现MAS引起的出血性皮疹。该患者对高剂量静脉注射免疫球蛋白(IVIG)和三次高剂量甲基强的松龙(MPD)反应迅速,临床症状和实验室检查均有改善,在疾病的任何阶段均未发生CAA。病例2:先前健康的10个月大的女性,不完全性KD伴持续发热和斑疹。该患者对IVIG无反应,并在亚急性期发展为MAS,在两次高剂量IVIG和大剂量MPD后表现为持续发热、高转氨酶血症、高铁蛋白血症和低纤维蛋白原血症。患者对添加IL-1阻滞剂和阿那那有反应,并且在疾病的任何阶段均未出现CAA改变。病例3:先前健康的26个月男性,不完全性KD伴发热、黄斑丘疹、唇炎和充血性结膜炎。该患者在急性期出现胆囊积液和CAA,对两种高剂量IVIG和高剂量MPD没有反应。在亚急性期,该患者并发MAS,并对静脉注射阿那白有反应。在亚急性期,患者出现短暂性动脉瘤,并在慢性期消退。这些病例重申,及时诊断和积极的免疫调节治疗可以限制MAS合并KD的最严重并发症。高剂量IVIG和MPD可能会产生良好的结果,或者可能需要更积极的辅助治疗。阿那白拉、环孢素、单克隆抗体和血浆置换可作为KD患者无反应性MAS的辅助治疗。值得注意的是,在病例2和3的亚急性期出现的MAS,在不使用环孢素的情况下,对IL-1阻滞剂anakinra迅速有反应。我们的经验证实,在对IVIG和MPD无反应的KD合并MAS患者中,IL-1阻滞剂可以被认为是最佳选择,避免过度使用细胞毒性药物。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Kawasaki Disease Complicated with Macrophage Activation Syndrome: The Importance of Prompt Diagnosis and Treatment–Three Case Reports
Kawasaki disease (KD) is an acute vasculitis that mainly affects children under 5 years of age, leading to coronary artery alterations (CAAs) in 25% of untreated patients. Macrophage activation syndrome (MAS) is a secondary hemophagocytic lymphohistiocytosis (HLH) that can complicate the acute, subacute, and chronic phases of KD. We retrospectively reviewed three cases of children affected by KD complicated with MAS hospitalized in two pediatric units in Emilia Romagna, a northern region of Italy. Case 1: a previously healthy 23-month-old female with full clinical criteria of KD and a hemorrhagic rash due to MAS during the acute phase of the illness. This patient responded promptly to a high dose of intravenous immune globulin (IVIG) and three pulses of high doses of methylprednisolone (MPD) with improvement in clinical signs and laboratory tests without the development of CAA at any phase of illness. Case 2: a previously healthy 10-month-old female with incomplete KD with persistent fever and maculopapular rash. This patient did not respond to IVIG and developed MAS during the subacute phase, characterized by persistent fever, hypertransaminasemia, hyperferritinemia, and hypofibrinogenemia after two high doses of IVIG and boluses of MPD. The patient responded to the addition of IL-1 blocker and anakinra and did not present CAA alterations during any phase of the illness. Case 3: a previously healthy 26-month-old male with incomplete KD with fever, maculopapular rash, cheilitis, and hyperemic conjunctivitis. This patient developed gallbladder hydrops and CAA in the acute phase and did not respond to two high doses of IVIG and a high dose of MPD. In the subacute phase, this patient was complicated with MAS and responded to intravenous anakinra. During the subacute phase, the patient developed transient aneurysms that regressed during the chronic phase. These cases reiterate that prompt diagnosis and aggressive immunomodulatory treatment can limit the most severe complications of MAS complicating KD. High doses of IVIG and MPD may result in a favorable outcome or more aggressive adjunctive treatment may be needed. Anakinra, cyclosporine, monoclonal antibodies, and plasmapheresis can be used as adjunctive treatment in the case of unresponsive MAS in KD. Notably, MAS, present during the subacute phase in cases 2 and 3, promptly responded to anakinra, an IL-1 blocker, without the use of cyclosporine. Our experience confirms that the IL-1 blocker can be considered an optimal choice after non-response to IVIG and MPD in KD complicating with MAS, avoiding over-treatment with cytotoxic drugs.
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