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免疫球蛋白 A 血管炎合并艰难梭菌感染:一例罕见病例报告并文献复习。

Immunoglobulin A Vasculitis Complicated with Clostridium difficile Infection: a Rare Case Report and Brief Review of the Literature.

机构信息

Gr. T. Popa University of Medicine and Pharmacy;St. Spiridon Emergency Hospital, Institute of Gastroenterology and Hepatology, Iasi, Romania.

St. Spiridon Emergency Hospital, Institute of Gastroenterology and Hepatology, Iasi, Romania.

出版信息

J Gastrointestin Liver Dis. 2016 Jun;25(2):235-8. doi: 10.15403/jgld.2014.1121.252.csd.

DOI:10.15403/jgld.2014.1121.252.csd
PMID:27308656
Abstract

Immunoglobulin A (IgA) vasculitis, formerly called Henoch-Schönlein purpura, is a leukocytoclastic type of vasculitis affecting small vessels with a deposition of immune IgA complexes, clinically characterized by the classic tetrad of nonthrombocytopenic palpable purpura, arthralgia (or arthritis), and gastrointestinal and renal involvement. Although the cause of the disease remains unknown, immune complexes of IgA and unidentified antigens seem to play a central pathogenic role. The diagnosis is easily established in the presence of purpura, but may be challenging in its absence, especially when colicky abdominal pain precedes the cutaneous lesions. IgA vasculitis is usually a self-limited disease with a benign course and symptomatic treatment is sufficient for most; in severe cases, however, corticosteroids are necessary. We describe the case of a young adult male presenting with severe abdominal pain, vomiting and fever (38.4ºC). Clinical examination, abdominal ultrasound and plain abdominal radiography excluded an acute abdomen. The occurrence of arthralgia involving both knees and erosive duodenitis at endoscopy, 48 hours upon admission, suggested the diagnosis of IgA vasculitis, confirmed on the following day by the presence of typical purpuric rash on the lower extremities. Corticosteroid therapy led to the resolution of all gastrointestinal and joint manifestations as well as to a significant improvement of cutaneous purpura. However, during the 3rd week of corticosteroid treatment, the patient developed watery diarrhea and the clinical suspicion of Clostridium difficile infection (CDI) was confirmed. The treatment with metronidazole led to the resolution of diarrhea.The peculiarity of this case resides in several aspects: the gastrointestinal and joint manifestations preceded purpura, making diagnosis more difficult; CDI is an extremely rare complication of IgA vasculitis, being, in fact, the second case reported in adults in the literature.

摘要

免疫球蛋白 A(IgA)血管炎,以前称为亨诺克-舒恩莱因紫癜,是一种影响小血管的白细胞碎裂性血管炎,免疫 IgA 复合物沉积,临床上以非血小板减少性可触及紫癜、关节炎(或关节炎)、胃肠道和肾脏受累的典型四联征为特征。尽管疾病的原因仍然未知,但 IgA 免疫复合物和未识别的抗原似乎发挥了中心致病作用。在存在紫癜的情况下,诊断很容易确立,但在没有紫癜的情况下可能具有挑战性,尤其是在皮肤病变之前出现绞痛性腹痛时。IgA 血管炎通常是一种自限性疾病,具有良性病程,大多数情况下对症治疗就足够了;然而,在严重的情况下,需要使用皮质类固醇。我们描述了一名年轻男性的病例,他表现为严重腹痛、呕吐和发热(38.4°C)。临床检查、腹部超声和腹部平片排除了急性腹痛。入院后 48 小时,出现累及双膝的关节炎和内镜下侵蚀性十二指肠炎,提示 IgA 血管炎的诊断,次日下肢出现典型紫癜皮疹进一步证实了这一诊断。皮质类固醇治疗导致所有胃肠道和关节表现以及皮肤紫癜的显著改善。然而,在皮质类固醇治疗的第 3 周,患者出现水样腹泻,临床怀疑为艰难梭菌感染(CDI),并得到证实。甲硝唑治疗导致腹泻缓解。

这个病例的特点在于几个方面

胃肠道和关节表现先于紫癜,使诊断更加困难;CDI 是 IgA 血管炎的一种极其罕见的并发症,实际上是文献中报告的成人中的第二例。

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