Sjogren's Syndrome Complicated with IgA Nephropathy and Leukocytoclastic Vasculitis

We report a case of primary Sjögren's syndrome (SS) with cutaneous leukocytoclastic vasculitis and IgA nephropathy. The accurate diagnosis of SS was established based on objective signs and symptoms of ocular and oral dryness, a characteristic appearance of a biopsy sample from a minor salivary gland, and the presence of anti-SS-A autoantibody. A second autoimmune disorder was not present, so the diagnosis of primary SS was established. A histologic finding of skin biopsy of purpuric lesion was typical for leukocytoclastic vasculitis. Renal biopsy was performed for nephrotic range proteinuria. The pathologic finding of renal biopsy was IgA glomerulonephritis with crescent formation. The patient was treated with small doses of glucocorticoids and maintenance hemodialysis. Leukocytoclastic vasculitis is one of the most characteristic extraglandular manifestations of SS. However, IgA nephropathy associated with SS and leukocytoclastic vasculitis is a rare finding. SS patients with glomerulonephritis present a more diverse outcome, even requiring hemodialysis. Therefore, renal biopsy is warranted in SS with glomerulonephritis and systemic vasculitis.     

Vasculite testicular – uma manifestação rara de artrite reumatoide

Testicular vasculitis is a very rare extra-articular manifestation of rheumatoid arthritis (RA). We describe the case of a 53-year-old man diagnosed with RA for eight years, who was poorly controlled and developed rheumatoid vasculitis, which manifested as leg ulcers and peripheral polyneuropathy. The patient also had acute neutrophilic meningitis and was treated with antibiotics and intravenous pulse therapy with methylprednisolone (500 mg daily) for three days, followed by oral cyclophosphamide (2 mg/kg daily) and prednisone. Overall improvement was observed, and the patient was discharged. But 15 days later, the meningitis recurred, and the patient was readmitted and treated again with antibiotics. Three days later, he developed pain and enlargement of his left testicle with gangrene. Unilateral orchiectomy was performed, revealing lymphocytic vasculitis. The patient died two days later due to aspiration pneumonia. This case illustrates a rare and severe manifestation of rheumatoid vasculitis.     

The avidity of PR3‐ANCA in patients with granulomatosis with polyangiitis during follow‐up

The objective of this study is to investigate whether the avidity of proteinase‐3‐anti‐neutrophil cytoplasmic antibody (PR3‐ANCA) changes during follow‐up in different subgroups of patients with granulomatosis with polyangiitis (GPA). We selected 10 patients with renal relapsing GPA, 10 patients with renal non‐relapsing GPA and 10 patients with non‐renal relapsing GPA. In all patients, an ANCA rise occurred during remission. The avidity was measured using a chaotropic approach at the time of an ANCA rise and at the time of a relapse in relapsing patients or time‐matched during remission in non‐relapsing patients. No difference was observed in the avidity at the ANCA rise between renal relapsing patients [26·2% (15·5–47·5)], renal patients without a relapse [39·6% (21·2–63·4)] and non‐renal relapsing patients [34·2% (21·6–59·5)]. In renal relapsing patients, the avidity increased significantly from the moment of the ANCA rise to the relapse [difference 6·4% (0·0–17·1), P = 0·0273]. The avidity did not increase after an ANCA rise in renal non‐relapsing patients [difference 3·5 (?6·0 to 10·1), P = 0·6250] or in non‐renal relapsing patients [difference ?3·1% (?8·0 to 5·0), P = 0·5703]. The avidity of PR3‐ANCA increases after an ANCA rise during follow‐up in renal relapsing patients, but not after an ANCA rise in renal patients who remain in remission or in non‐renal relapsing patients.     

细胞因子变化是否与IgA血管炎患儿肾损害相关？

背景IgA血管炎（IgAV）的长期预后取决于肾脏损伤的程度,有关IgAV肾损害发病机制的研究发现细胞因子在介导、驱动肾脏的损伤过程中发挥了重要作用。目的通过研究IgAV及肾损害患儿血清细胞因子水平变化,探讨细胞因子在IgAV肾损害过程中的意义和价值。方法选取2018年1月至2020年6月于中国医科大学附属盛京医院小儿肾脏内科住院的IgAV患儿194例作为研究对象,根据有无肾脏损害分为IgAV肾损害组（n=97）,IgAV组（n=97）,选取同时期本院儿童保健科进行体检的健康儿童60例为对照组。收集受试儿童及患儿细胞因子〔白介素（IL）-2、IL-4、IL-6、IL-10、IL-17、干扰素γ（IFN-γ）及肿瘤坏死因子α（TNF-α）〕以及患儿淋巴细胞绝对计数、免疫球蛋白A、免疫球蛋白E。采用多因素Logistic回归分析探讨IgAV肾损害的影响因素,绘制细胞因子对IgAV肾损害诊断价值的受试者工作特征（ROC）曲线。结果lgAV组IL-2水平高于lgAV肾损害组、对照组,且lgAV肾损害组IL-2水平高于对照组（P<0.05）;lgAV肾损害组IL-17水平高于lgAV组、对照组,lgAV组IL-17水平高于对照组（P<0.05）;lgAV组IL-6、IL-10和TNF-α水平高于lgAV肾损害组和对照组（P<0.05）;lgAV肾损害组和对照组IFN-γ水平高于lgAV组（P<0.05）。多因素Logistic回归分析结果显示,IL-2、IL-17、IFN-γ、TNF-α是IgAV肾损害发生的影响因素（P<0.05）。IL-2预测IgAV肾损害的ROC曲线下面积（AUC）为0.589,灵敏度为38.0%,特异度为47.0%;IL-17预测IgAV肾损害的AUC为0.621,灵敏度为47.4%,特异度为77.3%;IFN-γ预测IgAV肾损害的AUC为0.688,灵敏度为75.0%,特异度为55.7%;TNF-α预测IgAV肾损害的AUC为0.614,灵敏度为42.0%,特异度为37.0%;IL-17和IFN-γ联合预测IgAV肾损害的AUC为0.710,灵敏度为71.1%,特异度为66.0%。结论细胞因子IL-17、IFN-γ与IgAV肾损害的发生密切相关,早期检测两者水平并动态监测其变化,可对肾脏受累的早期发现及调整治疗方案起到预警作用。     

中枢神经系统血管炎与头痛

累及中枢神经系统的血管炎被称为中枢神经系统血管炎。头痛是中枢神经系统血管炎 的非特异表现之一,也是其中最常见的神经系统症状。目前按照2012年Chapel Hill会议（Chapel Hill consensus conference,CHCC）血管炎分类标准将血管炎按照受累血管大小不同进行分类。血管炎所致头 痛的发病机制不明,血脑屏障及神经血管单元的破坏在血管炎头痛中可能起到重要作用。不同类型 血管炎累及中枢神经系统的发生率以及头痛的发生时机和特点有差异。识别血管炎相关的头痛,并快 速进行诊断至关重要,以免进行不必要的治疗或导致严重神经系统后遗症。     

Cerebral arteriostenosis associated with elevated serum-immunoglobulin E level in young adults without risk factors for ischemic stroke: A possible manifestation of cerebral vasculitis?

We report a series of young adults with symptomatic cerebral arteriostenosis characterized by elevated serum immunoglobulin (Ig) E levels. All patients had no definite risk factors for cerebral vascular diseases. The clinical data of 26 young adults (age 18–50 years) with ischemic stroke, characterized only by increased serum IgE levels and without risk factors for cerebral vascular disease, were retrospectively reviewed. Arteriostenosis was surveyed and followed-up by digital subtraction angiography (DSA), and the stenosis rate was estimated using the warfarin–aspirin symptomatic intracranial disease technique. All patients were treated with corticosteroids according to the common strategy for vasculitis. There was no recurrent stroke during follow-up. The mean degree of stenosis before and after treatment was 69.3 ± 29.8% and 47.9 ± 45.1%, respectively. The difference of stenosis rates between initial and follow-up DSA evaluation was significant using a paired samples test (21.31 ± 26.88, 95% confidence interval [CI] 13.58–29.03, t = 5.55, p < 0.001). Kaplan–Meier survival analysis revealed that the 13-month cumulative improved lesion rate was 40.3 ± 8.7%. This remained the same at 18 months. The mean time to lesion improvement was 12.58 ± 0.96 months (95% CI 10.70–14.46) and median time was 13 ± 3.88 months (95% CI 5.39–20.61). To our knowledge, cerebral arteriostenosis with only elevated IgE serum levels has not been reported. Our data showed that corticosteroid treatment can achieve clinical and artery improvement. This suggests that the cerebral arteriostenosis seen in our study might be caused by some specific type of vessel inflammation.