Yamazaki-Nakashimada Marco A, Espinosa-Lopez Margarita, Hernandez-Bautista Victor, Espinosa-Padilla Sara, Espinosa-Rosales Francisco
Clinical Immunology Department, Instituto Nacional de Pediatria, Mexico City, Mexico.
Semin Arthritis Rheum. 2006 Jun;35(6):349-54. doi: 10.1016/j.semarthrit.2006.02.002.
Juvenile Polyarteritis nodosa (PAN) and Kawasaki Disease (KD) are disseminated vasculitides of unknown cause affecting small- and medium-sized vessels in children. We present an unusually severe case that fulfilled criteria for both KD and PAN. The diagnosis, overlapping clinical features, and treatment options for the 2 diseases are discussed.
A 3-year-old girl with systemic vasculitis is presented. We compare our case to 4 other cases reported in the literature which presented with a similar diagnostic dilemma. A review of the medical literature and a qualitative analysis of the diseases were performed, with emphasis on overlapping features, atypical cases, and treatment options.
Many features of KD and PAN are shared; however, there are some clinical features that could help differentiate one from the other. Fever, weight loss, rash, abdominal pain, arthritis, coronary arteritis, peripheral gangrene, anemia, leukocytosis, thrombocytosis, and elevated C-reactive protein are among many of the features that are shared by both diseases. However, KD also has unique clinical features that include conjunctivitis, changes in the lips and mouth, desquamation of the fingertips, and gallbladder hydrops, whereas renal involvement in KD is rare.
Occasionally juvenile PAN and KD share clinical manifestations, and when they do, it may be impossible to differentiate between them. Treatment should be directed according to the severity and persistence of these clinical manifestations.
儿童结节性多动脉炎(PAN)和川崎病(KD)是病因不明的弥漫性血管炎,累及儿童的中小血管。我们报告了1例同时符合KD和PAN诊断标准的异常严重病例。并对这两种疾病的诊断、重叠的临床特征及治疗选择进行讨论。
介绍1例患有系统性血管炎的3岁女孩。我们将该病例与文献中报道的其他4例存在类似诊断困境的病例进行比较。对医学文献进行综述并对这两种疾病进行定性分析,重点关注重叠特征、非典型病例及治疗选择。
KD和PAN有许多共同特征;然而,也有一些临床特征有助于区分两者。发热、体重减轻、皮疹、腹痛、关节炎、冠状动脉炎、外周坏疽、贫血、白细胞增多、血小板增多及C反应蛋白升高是这两种疾病共有的许多特征。然而,KD也有独特的临床特征,包括结膜炎、唇口改变、指尖脱皮及胆囊积液,而KD累及肾脏罕见。
儿童PAN和KD偶尔会有共同的临床表现,出现这种情况时可能无法区分两者。应根据这些临床表现的严重程度和持续时间进行治疗。