病例报告:1例罕见的复杂behet病合并急性肾小管坏死和IgA肾病,并发骨髓增生异常综合征、8三体和累及肠道。

IF 5.9 2区 医学 Q1 IMMUNOLOGY
Frontiers in Immunology Pub Date : 2025-09-15 eCollection Date: 2025-01-01 DOI:10.3389/fimmu.2025.1618495
Peipei Zhang, Wen Zhang, Hong Xia, Zhenliang Fan, Junfen Fan, Hongzhen Ma, Chunli Zhang, Shuyan Liu
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引用次数: 0

摘要

behet病(BD)是一种罕见的全身疾病,其小血管炎累及多个身体器官。它的典型特征是复发性口腔和生殖器溃疡、葡萄膜炎和皮肤病变。然而,外周血管、心血管结构、中枢神经系统、胃肠道、关节、肺或肾脏也可能受到影响。肾脏受累,虽然不常见,但可表现为蛋白尿、血尿和不同程度的肾功能不全。在本文中,我们描述了一位35岁的患有长期双相障碍、骨髓增生异常综合征(MDS)和8号三体的男性。他表现为皮肤红斑和胃肠道出血(需要结肠切除术),后来发展为急性肾功能衰竭。随后的活检显示急性肾小管坏死(ATN)和IgA肾病的特征。经持续肾脏替代治疗及静脉注射甲基强的松龙治疗后,肾功能逐渐恢复。这种情况代表了一种罕见且严重的双相障碍多系统表现,并伴有复杂的合并症,将观察到的肾脏损伤归因于上述联合损伤。鉴于BD的持续性和多面性,早期识别和有针对性的处理肾脏并发症对于保持功能和改善患者预后至关重要。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Case Report: A rare case of complex Behçet's disease complicated with acute tubular necrosis and IgA nephropathy, coexists with myelodysplastic syndrome, trisomy 8 and intestinal involvement.

Behçet's disease (BD) is a rare systemic disease in which small-vessel vasculitis impacts multiple bodily organs. It is typically marked by recurrent oral and genital ulcers, uveitis, and cutaneous lesions. However, peripheral vessels, cardiovascular structures, central nervous system, gastrointestinal tract, joints, lungs, or kidneys may be affected as well. Renal involvement, although uncommon, may manifest as proteinuria, hematuria, and varying degrees of renal insufficiency. Herein, we describe a 35-year-old man with longstanding BD, myelodysplastic syndrome (MDS), and trisomy 8. He presented with cutaneous erythema and gastrointestinal bleeding (requiring colonic resection), later developing acute renal failure. Features of both acute tubular necrosis (ATN) and IgA nephropathy appeared on subsequent biopsy. Following continuous renal replacement therapy and intravenous methylprednisolone treatment, there was gradual recovery of renal function. This scenario represents a rare and severe multisystem presentation of BD with complex comorbidities, attributing the observed kidney injury to combined insults as above. Given the persistent and multifaceted nature of BD, early recognition and targeted management of renal complications are essential to preserve functional capacity and improve patient outcomes.

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来源期刊
CiteScore
9.80
自引率
11.00%
发文量
7153
审稿时长
14 weeks
期刊介绍: Frontiers in Immunology is a leading journal in its field, publishing rigorously peer-reviewed research across basic, translational and clinical immunology. This multidisciplinary open-access journal is at the forefront of disseminating and communicating scientific knowledge and impactful discoveries to researchers, academics, clinicians and the public worldwide. Frontiers in Immunology is the official Journal of the International Union of Immunological Societies (IUIS). Encompassing the entire field of Immunology, this journal welcomes papers that investigate basic mechanisms of immune system development and function, with a particular emphasis given to the description of the clinical and immunological phenotype of human immune disorders, and on the definition of their molecular basis.
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