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首页> 外文期刊>Nagoya journal of medical science >SELECTIVE IGA DEFICIENCY MIMICKING CHURG-STRAUSS SYNDROME AND HYPEREOSINOPHILIC SYNDROME: A CASE REPORT
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SELECTIVE IGA DEFICIENCY MIMICKING CHURG-STRAUSS SYNDROME AND HYPEREOSINOPHILIC SYNDROME: A CASE REPORT

机译：选择性IGA缺陷模拟楚格氏综合征和超嗜酸性综合征：病例报告

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摘要

Selective IgA deficiency (SIgAD) is the most common type of primary immunoglobulin deficiency. Most individuals with SIgAD are asymptomatic. However, some patients are associated with allergic and autoimmune disease. SIgAD is included in the list of differential diagnoses of eosinophilia. We experienced a patient who initially presented with abdominal pain and eosinophilia. A >1-year follow-up revealed SIgAD, and we had difficulty differentiating it from Churg-Strauss syndrome (CSS) or hypereosinophilic syndrome (HES). A 66-year-old Japanese male presented with a history of recurrent abdominal pain. A diagnostic work-up revealed eosinophilia, eosinophilic gastritis, eosinophilic pneumonia, and SIgAD over 1 year of clinical observation. He also suffered from asthma and sinusitis. Anti-neutrophil cytoplasmic antibody was negative and vasculitis was not detected in the obtained tissue specimens of stomach, lung, nose and skin. The patient showed no evidence of drug ingestion, parasitic infections, or malignant neoplasms. Although we cannot rule out prevasculitic CSS and idiopathic HES, the whole clinical picture in this patient can be explained most consistently by SIgAD.
机译：选择性IgA缺乏症（SIgAD）是最常见的原发性免疫球蛋白缺乏症。患有SIgAD的大多数个体无症状。但是，有些患者与变态反应和自身免疫性疾病有关。 SIgAD包括在嗜酸性粒细胞的鉴别诊断中。我们遇到了一位最初出现腹痛和嗜酸性粒细胞增多的患者。一项> 1年的随访发现SIgAD，我们很难将其与Churg-Strauss综合征（CSS）或嗜酸性粒细胞增多症（HES）区分开。一位66岁的日本男性，有腹部反复发作的病史。在一年的临床观察中，诊断性检查显示嗜酸性粒细胞增多，嗜酸性胃炎，嗜酸性肺炎和SIgAD。他还患有哮喘和鼻窦炎。获得的胃，肺，鼻子和皮肤组织标本中抗中性粒细胞胞浆抗体阴性，未检出血管炎。该患者未显示任何药物摄入，寄生虫感染或恶性肿瘤的迹象。尽管我们不能排除脉管炎前期CSS和特发性HES，但SIgAD可以最一致地解释该患者的整个临床情况。

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