Long-term MR follow-up of cerebral lesions in neuro-Behçet's disease

To study the long-term evolution of cerebral lesions in neuro-Behçet's disease, MRI was carried out on 12 patients, with follow-up from 1.5 to 6 years (mean 3.5 years). On the first MRI, 66 lesions in all were found; each patient had 1–10 lesions (mean 5.5). There were 30 (46 %) lesions in the brain stem, 18 (27 %) in the basal ganglia region and 18 (27 %) in the periventricular white matter. Of these 22 (33 %) were small, 31 (47 %) medium-size and 13 (20 %) large lesions. On the last MRI, 60 lesions were found; each patient had 1–10 lesions (mean 5). At this time 31 lesions (52 %) were in the brain stem, 13 (22 %) in the basal ganglia region and 16 (26 %) in the periventricular white matter. There were 41 (68 %) small, 13 (22 %) medium-size and 6 (10 %) large lesions. About 40 % of the lesions disappeared, 35 % reduced in size and 25 % remained unchanged. No lesion had enlarged. Of the 60 final lesions 20 (34 %) were not observed on the first study. Small new lesions were found in 5 of 12 patients (42 %), and were asymptomatic. Medium-size or large new lesions were found in 2 patients (17 %) who had stopped steroid treatment and had a neurological relapse. Enlargement of the ventricular system or worsening of initial cerebral atrophy was observed in 9 of 12 patients. Appearance of small lesions and worsening of cerebral atrophy on long-term follow-up suggest the possibility of subclinical progression of cerebral vasculitis and should be considered in the prognosis of neuro-Behçet's disease.     

茵陈蒿汤加减治疗湿热阻络型变应性皮肤血管炎溃疡的临床观察

目的:观察茵陈蒿汤加减治疗湿热阻络型变应性皮肤血管炎溃疡的临床疗效。方法:纳入湿热阻络型变应性皮肤血管炎溃疡患者60例,随机分为治疗组和对照组,每组各30例。所有患者均给予基础治疗,包括抗凝、抗感染、创面换药等,对照组患者在基础治疗的同时给予甲泼尼龙片口服,治疗组患者在基础治疗的同时给予茵陈蒿汤加减口服。两组均以10 d为1个疗程,共观察6个疗程。治疗结束后,评价两组患者的临床疗效;于治疗前及治疗10 d、20 d、30 d、40 d、50 d、60 d后,采用视觉模拟评分法(VAS)评估患者的创面疼痛程度;于治疗10 d、20 d、30 d、40 d、50 d、60 d后,比较两组的痊愈率;治疗全程记录两组患者的不良反应发生情况,比较两组患者的不良反应发生率。结果:治疗后,治疗组的临床总有效率为96.67%,对照组为86.67%,两组临床疗效比较差异有统计学意义(P0.05),治疗组的疗效优于对照组。治疗10 d、20 d、30 d、40 d、50 d、60 d后,两组患者的疼痛VAS评分较治疗前均降低(P0.05);且治疗30 d、40 d、50 d、60 d后,治疗组患者的VAS评分均低于对照组(P0.05)。治疗30 d、40 d、50 d、60 d后,治疗组患者的创面痊愈率均明显高于对照组(P0.05)。在治疗过程中,治疗组的不良反应发生率为6.67%,对照组为26.67%,治疗组的不良反应发生率明显低于对照组(P0.05)。结论:在基础治疗的同时联合应用茵陈蒿汤加减治疗湿热阻络型变应性皮肤血管炎溃疡,具有较好的临床疗效和安全性,可明显减轻患者的疼痛症状,促进创面愈合。     

Clinicopathological analysis of Sjogren's syndrome complicated with ANCA associated vasculitis with renal involvement

Objective To investigate the clinical and pathological features of patients with a combination of Sjogren's syndrome (SS) and antineutrophil cytoplasmic antibody (ANCA) associated vasculitis with renal involvement. Methods By searching the Peking Union Medical College Hospital medical database and literature between January 1990 and June 2017, patients had a combination of SS and ANCA associated vasculitis with renal involvement were included. Data of clinical information, autoimmune antibodies, renal manifestations and renal pathology were retrieved and analyzed. Results Eighteen patients were enrolled: 4 from our hospital and 14 from literature. SS was diagnosed no later than ANCA associated vasculitis in all the patients, among which 83.3%(15/18) of patients had extra-glandular and extra-renal organs involved. All the patients were tested positive for myeloperoxidase (MPO)-ANCA, and only two were protein 3 (PR3)-ANCA positive concurrently. The positivity rates of antinuclear antibody (ANA), rheumatoid factor (RF), anti-SSA antibody, and anti-SSB antibody were 83.3%(15/18), 55.6%(10/18), 77.8%(14/18), and 38.9%(7/18), respectively. The renal manifestations were characterized by renal insufficiency with a median serum creatinine of 174 μmol/L, hematuria, moderate proteinuria with a median 24 hour urine protein of 1.70 g, and necrotizing vasculitis with oligo-immune complex and varying degrees of interstitial damage in pathology. Conclusions A combination of Sjogren's syndrome and ANCA associated vasculitis with renal involvement is rare in clinical setting, and almost all of the patients are MPO-ANCA positive, with high probability of ANA positivity and extra-glandular involvement. Physicians should beware of ANCA associated glomerulonephritis in SS patients with inexplicable renal dysfunction and renal biopsy should be carried out in time.     

TLR5 rs2072493 A/G基因多态性与广西人群抗中性粒细胞胞浆抗体相关性小血管炎的关系

目的 探讨TLR5 rs2072493 A/G基因多态性与广西人群抗中性粒细胞胞浆抗体(ANCA)相关性小血管炎(AAV)的关联性.方法 采用PCR-RFLP法检测117例AAV患者(AAV组)及207例健康成人(对照组)的TLR5 rs207249基因型,采用病例-对照研究并行临床和病理资料分析.结果 (1)AAV组患者TLR5rs2072493基因多态性AA、AG、GG基因型频率分别为47.0％、41.0％、12.0％,对照组为50.2％、43.5％、6.3％;A和G基因分布:AAV组为67.5％、32.5％,对照组为72.0％、28.0％.两组基因型及等位基因频率比较差异无统计学意义(P＞0.05).(2)3种基因型间关节疼痛差异有统计学意义(P＜0.05),以GG基因型最多发;尿酸、白蛋白、IgA比较差异有统计学意义(P＜0.05),尿酸GG基因型明显低于AA和AG型,白蛋白、IgA GG基因型明显高于AA和AG型.(3)AAV组中有88例行肾穿活检,3种基因型患者间病理学评分差异无统计学意义(P＞0.05).结论 TLR5 rs2072493基因多态性在广西人群普遍存在,可能与AAV遗传易感性无关联,可能与关节痛、UA、Alb、IgA水平有关联.     

广西汉族人群Toll样受体91486T/C基因多态性与ANCA相关性小血管炎的相关性

目的 探讨广西汉族人群TLR9 1486T/C基因多态性与原发抗中性粒细胞胞浆抗体(ANCA)相关性小血管炎(AAV)的相关性.方法 采用聚合酶链反应-限制性片段长度多态性(PCR-RFLP)检测116例AAV患者(AAV组)和209例性别、年龄相匹配的健康成人(对照组)TLR9 1486T/C位点多态性,并检测其血清生化指标.结果 AAV组与对照组TLR9 1486T/C位点基因型和等位基因分布比较差异无统计学意义(P＞0.05).AAV患者三组基因型间尿素氮、肌酐、IgM水平比较差异有统计学意义(P＜0.05).病理资料分析显示AAV患者三组基因型小血管炎活动指数及慢性指数差异无统计学意义(P＞0.05).结论 广西汉族人群中,TLR9 1486T/C基因多态性可能与尿素氮、肌酐、IgM升高具有相关性,可能与AAV的遗传易感性不相关.     

59例肺部受损的MPO阳性ANCA相关性血管炎的临床特征分析

目的 分析肺部受累的MPO阳性ANCA相关性血管炎患者的临床及肺部影像学特征。方法 分析2014年1月至2020年12月在联勤保障部队第九六〇医院肾内科就诊的MPO-ANCA阳性且确诊为ANCA相关性血管炎（AAV）的59例患者的临床特征、辅助检查、肺部影像学改变。根据患者尿常规、血肌酐（CRE）及肾小球滤过率（eGFR）等数值分为两组：单纯肺损害组及肺肾合并损害组,并且对两组患者的上述资料进行分析。结果 1.入选的59例患者中,单纯肺损伤组13例（男/女：6/7）,平均年龄60±17.63岁;肺肾合并损伤组46例（男/女：21/25）,平均年龄62.28±15.12岁。两组的发病年龄及性别差异均无统计学意义（P>0.05）。2.单纯肺损伤组患者血红蛋白（HGB）、白蛋白（ALB）、肾功能（CRE、BUN）、血沉（ESR）、C反应蛋白（CRP）结果分别为：116.38±23.99、34.75±5.31、62.69±14.04、4.02±1.63、57.77±34.56、14.3(5.55,58.70),肺肾合并损伤组上述结果分别为：85.13±23.79、27.55±4.68、402.07±103.90、19.14±11.50、73.60±35.04、28.15(5.08,103.0),两组数据差异有统计学意义（P<0.05）。单纯肺损伤组患者HGB、ALB水平明显高于后者,而ESR、CRP水平低于后者。3.两组比较,肺肾合并损伤组中高血压患者占比（86.96%）明显高于单纯肺损伤组（46.15%）,两组差异有统计学意义（P<0.05）,其余合并症在两组患者中的占比差异无统计学意义。4.两组患者临床表现方面,单纯肺损伤组患者体重下降（30.77%）比后者（4.34%）占比高,肺肾合并损伤组恶心纳差/乏力症状（39.96%）较前者（7.69%）占比高,差异均有统计学意义（P<0.05）。5.对所有患者的影像学改变进行分类,影像学改变以网格样改变、斑片实变影及胸膜增厚多见。肺肾损伤组患者多见于斑片实变影,但两组患者不同的影像学改变差异无统计学意义（P>0.05） 结论 1.MPO阳性的AAV患者肺部影像学改变多样,但两组改变无特异性;2.两组患者临床表现多样,肺肾损伤组中,高血压患者占比明显高于单纯肺损伤组;3.肺肾合并损伤组肾功能损害较重且容易出现严重的贫血及低蛋白血症,提示预后不良,需及时治疗。     

Pathogénie des vascularites associées aux ANCA en 2021 : mise au point

Anticytoplasmic neutrophil antibodies (ANCA)-associated vasculitis (AAV) are rare systemic immune-mediated diseases characterized by small vessel necrotizing vasculitis and/or respiratory tract inflammation. Over the last 2 decades, anti-MPO vasculitis mouse model has enlightened the role of ANCA, neutrophils, complement activation, T helper cells (Th1, Th17) and microbial agents. In humans, CD4T cells have been extensively studied, while the dramatic efficacy of rituximab demonstrated the key role of B cells. Many areas of uncertainty remain, such as the driving force of GPA extra-vascular granulomatous inflammation and the relapse risk of anti-PR3 AAV pathogenesis. Animal models eventually led to identify complement activation as a promising therapeutic target. New investigation tools, which permit in depth immune profiling of human blood and tissues, may open a new era for the studying of AAV pathogenesis.     

Propylthiouracil-induced autoimmune disease

Hyperthyroidism is a condition characterized by excessive production of thyroid hormones. Propylthiouracil (PTU) is commonly used as first line drug in the management of hyperthyroidism. This is a case report of 24-year-old female, a known case of hyperthyroidism since 4 years, who came with a history of fever and myalgia since 3 days and dyspnea with coughing out of blood since 1 day. Patient was taking PTU (100 mg per day) since 4 years for hyperthyroidism. Patient was immediately intubated for type-II respiratory failure. Diagnosed to be having PTU-induced autoimmune disease. PTU was stopped and treated with methylprednisolone and cyclophosphamide. Clinical features improved over a period of 8 days and discharged home successfully. Having a high suspicion for the onset of autoimmune disease in hyperthyroidism patients who are on PTU therapy and timely treatment with immunosuppressants and supportive care along with the withdrawal of the drug can make a difference in morbidity and mortality.     

Possible Cerebral Vasculitis in a Case with Rheumatoid Arthritis

Cerebral rheumatoid vasculitis (CRV) is a rare, fatal, and diagnostically challenging disorder. We herein report an 81-year-old woman with a 4-year history of rheumatoid arthritis who presented with a fever, progressive disturbance of consciousness, high level of rheumatoid factor, and hypocomplementemia. The enhancement of the perforating branches in the left middle cerebral artery led us to suspect CRV. A brain biopsy could not be performed. After we intensified steroid therapy, the size of the cerebral lesions temporarily decreased. However, recurrence in the left frontal lobe occurred one month later, and the patient subsequently died. Early intensive treatments may be needed for CRV.