Retroperitoneal fibrosis requiring prompt nephrostomy in a case with immunoglobulin A vasculitis
Retroperitoneal fibrosis requiring prompt nephrostomy in a case with immunoglobulin A vasculitis
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免疫球蛋白 A 血管炎病例中腹膜后纤维化需要立即进行肾造口术
DOI：
10.1080/03009742.2022.2047312
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发表时间：
2022
影响因子：
2.1
通讯作者：
Hashimoto M
中科院分区：
文献类型：
--
作者：
Ishihama Y;Fukumoto K;Watanabe R;Nakatani S;Tsuda A;Otoshi T;Yamada K;Yamada S;Negoro N;Emoto M;Hashimoto M
免疫球蛋白A （IgA）血管炎（以前称为Henoch-Schönlein紫癜）是一种小血管血管炎，IgA为主的免疫沉积通常累及皮肤、肠道和肾小球，并与关节痛和/或关节炎相关(1)。已有几例IgA血管炎合并输尿管狭窄的报道（2,3）。然而，已发表的文献仅限于一例伴有腹膜后纤维化（RPF）的病例(4)。我们报告一例IgA血管炎，他表现出腹膜后受累，需要及时肾造口术和类固醇治疗。一名73岁女性因腰痛和腿下区可触及的紫癜入住我院，已存在一个月（图1A）。除使用钙通道阻滞剂治疗高血压外，无明显病史或合并症。体格检查：体温36.6℃，血压157/80 mmHg，脉搏96次/分。腿部下部有凹陷性水肿及可触及的紫癜。白细胞20600个/μL，嗜酸性粒细胞1854个/μL， c反应蛋白31.3 mg/dL，血清肌酐4.98 mg/dL，尿素氮53 mg/dL。抗核抗体、抗ro （SSA）抗体、抗中性粒细胞胞浆抗体、冷球蛋白检测均为阴性。血清补体（C3和C4）浓度正常。血清IgG、IgA和IgG4水平分别升高至1977 mg/dL、500 mg/dL和138 mg/dL。尿沉渣红细胞计数正常，尿蛋白0.16 g/gCr。皮肤活检显示白细胞破裂性血管炎（图1B）。未检出IgA沉积。全身计算机断层扫描（CT）显示腹膜后肿块引起的双侧肾积水（图1C）， 18f -氟脱氧葡萄糖正电子发射断层扫描显示轻度摄取（图1D）。双侧输尿管置管未能释放尿潴留，随后需要对右肾进行经皮肾造口术（图1E）。口服强的松龙（每天1mg /kg）和伴随的抗生素导致实验室结果和皮肤病症状的迅速改善。随访CT显示输尿管梗阻消退。肾活检显示轻度肾小球系膜扩张伴IgA沉积（图1F），证实IgA血管炎的诊断。未见igg4阳性浆细胞浸润。2个月后复查CT显示腹膜后肿块明显消退。输尿管受累可表现为各种形式的系统性小血管炎。已有几例IgA血管炎合并输尿管狭窄的报道（2,3）。然而，在IgA血管炎中RPF的发生率是罕见的，因为文献中只发表了一例记录病例(4)。RPF是一种罕见的疾病，其特征是存在腹膜后肿块，由慢性炎症和明显的纤维化组成，经常包裹输尿管或其他腹部器官(5)。继发性RPF与恶性疾病、药物、暴露于辐射和手术有关。相反，大多数RPF被归类为特发性腹膜后纤维化，其免疫学病因已被提出(5)。据报道，特发性RPF是igg4相关疾病谱系的一部分(6)。RPF与自身免疫性疾病之间的关系，如中小型血管炎，如肉芽肿病合并多血管炎，
Immunoglobulin A (IgA) vasculitis (formerly known as Henoch–Schönlein purpura) is a small-vessel vasculitis with IgA-dominant immune deposits that typically involves the skin, gut, and glomeruli, and is associated with arthralgia and/or arthritis (1). Several cases of IgA vasculitis with ureteral stenosis have been reported (2, 3). However, the published literature is limited to only one documented case concurrent with retroperitoneal fibrosis (RPF)(4). We report a case of IgA vasculitis who demonstrated retroperitoneal involvement requiring prompt nephrostomy and steroid administration. A 73-year-old woman was admitted to our hospital presenting with lower back pain and palpable purpura on the lower region of her legs, which had been present for a month (Figure 1A). No remarkable medical history or comorbidities were noted, other than hypertension treated with a calcium channel blocker. On physical examination, her temperature was 36.6 C, blood pressure 157/80 mmHg, and pulse rate 96 beats per minute. Pitting oedema and palpable purpura were found on the lower region of her legs. Laboratory tests showed a leucocyte count of 20 600 cells/μL, eosinophils 1854 cells/μL, C-reactive protein 31.3 mg/dL, serum creatinine (Cr) 4.98 mg/dL, and blood urea nitrogen 53 mg/dL. Tests for anti-nuclear antibody, anti-Ro (SSA) antibody, anti-neutrophil cytoplasmic antibodies, and cryoglobulin were negative. Serum concentrations of complements (C3 and C4) were normal. Serum IgG, IgA, and IgG4 levels were increased to 1977 mg/dL, 500 mg/dL, and 138 mg/dL, respectively. Urinary sediment showed a normal red blood cell count, and urinary protein was 0.16 g/gCr. Skin biopsy demonstrated leucocytoclastic vasculitis (Figure 1B). IgA deposition was not detected. Wholebody computed tomography (CT) revealed bilateral hydronephrosis caused by a retroperitoneal mass (Figure 1C), which showed mild uptake on 18F-fluorodeoxyglucose positron emission tomography (Figure 1D). Bilateral ureteral catheterization failed to release urinary retention, which subsequently required percutaneous nephrostomy for the right kidney (Figure 1E). Oral prednisolone (1 mg/kg per day) and concomitant antibiotics led to rapid improvement in the laboratory results and dermatological symptoms. Follow-up CT demonstrated resolution of the ureteral obstruction. Kidney biopsy showed slight mesangial expansion with IgA deposition (Figure 1F), which confirmed the diagnosis of IgA vasculitis. IgG4-positive plasma cell infiltration was not observed. After 2 months, repeated CT showed marked regression of the retroperitoneal mass. Ureteral involvement can be present in various forms of systemic small-vessel vasculitis. Several cases of IgA vasculitis with ureteral stenosis have been reported (2, 3). However, the incidence of RPF in IgA vasculitis is rare, because only one documented case has been published in the literature (4). RPF is a rare disease characterized by the presence of a retroperitoneal mass, consisting of chronic inflammation and marked fibrosis, which often entraps the ureters or other abdominal organs (5). Secondary RPF is associated with malignant disease, drugs, exposure to radiation, and surgery. In contrast, the majority of RPF is classified as idiopathic retroperitoneal fibrosis, for which an immunological aetiology has been suggested (5). Idiopathic RPF has been reported to be part of a spectrum of IgG4-related disease (6). The associations between RPF and autoimmune diseases, eg small and medium-sized vessel vasculitis, such as granulomatosis with polyangiitis,
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双侧输尿管狭窄是过敏性血管炎的并发症
影响因子：
2.2
作者：
C. Ihoriya;Y. Morita;T. Tokura;Kengo Kidokoro;N. Komai;Tamaki Sasaki;N. Kashihara
通讯作者：
N. Kashihara
丙型肝炎相关冷球蛋白血症与腹膜后纤维化相关。
DOI：
--
发表时间：
1996
期刊：
The Journal of rheumatology
影响因子：
--
作者：
L. Hofbauer;R. Magerstädt;Heufelder Ae
通讯作者：
Heufelder Ae
过敏性紫癜和腹膜后纤维化。
影响因子：
2.4
作者：
N. Akman;Y. Avanoğlu;K. Karabay;E. Erek;A. Tokgöz;E. Aras;G. Girisken;N. Tüzüner;H. Avanoğlu
通讯作者：
H. Avanoğlu
影响因子：
11.1
作者：
Vaglio, A;Manenti, L;Buzio, C
通讯作者：
Buzio, C
影响因子：
1.4
作者：
Kasahara, Katsuaki;Uemura, Osamu;Iwata, Naoyuki
通讯作者：
Iwata, Naoyuki