Dynamically changing antineutrophil cytoplasmic antibodies in granulomatosis with polyangiitis:A case report

BACKGROUND Granulomatosis with polyangiitis(GPA)is one of the most prevalent forms of the antineutrophil cytoplasmic antibody(ANCA)-associated vasculitis.GPA is characterized histologically by necrotizing granulomatous inflammation in addition to vasculitis.The diagnosis of GPA depends on clinical presentation,serological evidence of a positive ANCA,and/or histological evidence of necrotizing vasculitis or granulomatous destructive parenchymal inflammation.Cytoplasmic ANCA(c-ANCA)is positive in 65%-75% of GPA patients,accompanied by proteinase 3(PR3),the main target antigen of c-ANCA,another 5% of GPA patients had negative ANCA.CASE SUMMARY The patient,a 52-year-old male,presented with unexplained nasal congestion,tinnitus,and hearing loss.After a duration of 4 months experiencing these symptoms,the patient subsequently developed fever and headache.The imaging examination revealed the presence of bilateral auricular mastoiditis and partial paranasal sinusitis,and the ANCA results were negative.The anti-infective therapy proved to be ineffective,but the patient's symptoms and fever were quickly relieved after 1 wk of treatment with methylprednisolone 40 mg once a day.However,after continuous use of methylprednisolone tablets for 3 months,the patient experienced a recurrence of fever accompanied by right-sided migraine,positive c-ANCA and PR3,and increased total protein in cerebrospinal fluid.The and cyclophosphamide 0.8 g monthly,the patient experienced alleviation of fever and headache.Additionally,the ANCA levels became negative and there has been no recurrence.CONCLUSION For GPA patients with negative ANCA,there is a potential for early missed diagnosis.The integration of histopathological results and multidisciplinary communication plays a crucial role in facilitating ANCA-negative GPA.

Significance of antineutrophil cytoplasmic antibody in adult patients with Henoch-Schnlein purpura presenting mainly with gastrointestinal symptoms

AIM： To test the clinical significance of antineutrophil cytoplasmic antibody （ANCA） in evaluation of adult Henoch-Schonlein purpura （HSP） patients presenting mainly with abdominal symptoms. METHODS： Twenty-eight consecutive HSP patients who presented predominantly with abdominal symptoms were enrolled in this study. Control subjects included 27 ageand sex-matched patients with peptic ulcer disease, colon cancer, acute gastroenteritis, irritable bowel syndrome and colonic polyps. ANCA was measured by indirect immunofluorescence （IIF） in all patients, and follow-up ELISA was performed in patients with positive IIF tests. RESULTS： ANCA was detected in 9 HSP patients by IIF （2 were positive for c-ANCA and 7 were positive for p-ANCA）. No ANCA was found in the control group. The sensitivity and specificity of a positive ANCA test （either c- or p-ANCA） were 32.1% and 100% respectively. Only one out of the 9 patients with positive ANCA by IIF had positive ANCA by ELISA and the antigen was myeloperoxidase （MPO）. The patients positive for ANCA had higher HSP clinical scores, and were more likely to have renal function impairment. Patients with late purpura development were also associated with more severe clinical manifestations. CONCLUSION： A positive ANCA test is associated with more severe symptoms in HSP. After inflammatory bowel disease is excluded, a positive ANCA test provides a clue to the diagnosis of HSP presenting predominantly with abdominal symptoms.

A subset of ulcerative colitis with positive proteinase-3 antineutrophil cytoplasmic antibody

A small subset of patients with active ulcerative colitis is non-responsive to major known non-biological therapies. We reported 5 patients with positive serum proteinase-3 antineutrophil cytoplasmic antibody (PR3-ANCA) and tried to (1) identify the common clinical features of these patients; (2) investigate the efficacy of a novel therapy using a Chinese medicine compound; and (3) attract more gastroenterologists to be engaged in further study of this subset of patients. The common manifestations of disease in these 5 patients included recurrent bloody diarrhea and inflammatory lesions involving the entire colorectal mucosa. Initial treatment with intravenous methylprednisolone successfully induced remission. Four of these 5 patients were steroid-dependence, and immunosuppressants, such as azathioprine and cyclophosphamide, were in effective. In 3 patients, only the particular Chinese medicine compound could induce and maintain remission. One patient underwent colectomy. No vascular inflammatory lesions were found by histopathological examination. Although more cases are needed for confirmation, our study indicates thatulcerative colitis with positive PR3-ANCA may belong to a subtype of refractory ulcerative colitis. The particular Chinese medicine compound used in our study is by far the most effective in the management of these patients, with additional advantages of having no noticeable side-effects and less financial burden.

The role of mycophenolate in the treatment of antineutrophil cytoplasmic antibody-associated vasculitis

Mycophenolic acid, the active metabolite for mycophenolate mofetil and mycophenolic sodium, is a strong, noncompetitive, reversible inhibitor of inosine monophosphate dehydrogenase, the key enzyme in de novo synthesis of guanosine nucleotides leading to selective inhibition of lymphocyte proliferation. Mycophenolic acid has been evaluated as induction and remission maintenance agent in the treatment of antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Since the course of disease of AAV usually requires long term immunosuppression, mycophenolate has been explored as a less toxic agent compared to cyclophosphamide and azathioprine. Mycophenolate is a potent immunosuppressive agent in the therapy of AAV, non-inferior to other available drugs with comparable side effect profile. Therefore, it could be a valuable alternative in cases of toxicity with life threatening side effects or intolerance to cyclophosphamide or azathioprine, in cases with high cumulative dose of cyclophosphamide, but also in cases with insufficient response. Several studies have shown a higher relapse rate following discontinuation of mycophenolate or in mycophenolate treated subjects that raises concerns about its usefulness in the treatment of AAV. This review describes the efficacy of mycophenolate in AAV as remission induction agent, as remission maintenance agent, and as therapeutic option in relapsing AAV disease, the relapse rate following discontinuation of mycophenolate, and the adverse events related to mycophenolate treatment.

CLINICAL AND PATHOLOGICAL MANIFESTATI-ONS OF PATIENTS WITH ANTINEUTROPHIL CYTO-PLASMIC AUTOANTIBODIES DIRECTED AGA INST PROTEINASE 3 OR MYELOPEROXIDASE

To compare the clinical and pathological manifestations of patients with antineutrophil cytoplasmic autoantibodies (ANCA) directed against proteinas e 3 (anti PR3) or myeloperoxidase (anti MPO). Methods. One hundred and forty patients with ANCA were detected for anti PR3 a nd anti MPO by ELISA. The clinical features at presentation, histopathological characteristics and outcome of all patients who were tested positive for anti P R3 or anti MPO were analysed.Results. In anti PR3 group (n=21), 16 cases (76.2%) had systemic vasculitis , in which Wegener’s granulomatosis prevailed (13 cases, 61.9%). In anti MPO g roup (n=31), 19 cases (61.3%) were diagnosed as systemic vasculitis and 12 case s (38.7%) as microscopic angiitis. For vasculitic patients with anti PR3 and a nti MPO, the disease duration at diagnosis was 9.6±2.0m and 4.4±0.9m respecti vely, P< 0.05;vasculitis activity index (BVAS) and mean number of affected organ were 22.5±2.1, 5.0±0.4 and 25.1±1.7, 4.8±0.4 respectively, P >0.05;upper r espiratory tract, eye and joint involvements were 11(68.8%), 7(43.8%), 11(68.8 %) and 7(36.8%), 2(10.5%), 5(26.3%) respectively, P< 0.05.Although there was no statistical difference in renal involvement between these two groups, patien ts with serum creatine >500 μmol/L were more commonly seen in anti MPO group t han in anti PR3 group, which were 8(42.1%) and 2(12.5%) respectively, P< 0.05 . Ten relapses were seen in anti PR3 group and only 2 in anti MPO group, but t he acute mortality rate in anti MPO group (5/19, 27.4%) was much higher than t hat in anti PR3 group (1/16, 6.3%). Conclusions. Anti PR3 and anti MPO occurred mainly in systemic vasculitis. A large divergence was seen in the disease spectrum between patients with anti PR 3 and those with anti MPO. In particular, upper respiratory tract, eye and join t involvements, granuloma formation and relapse were more prominent in anti PR3 patients. By contrast, the anti MPO patients had a more acute disease onset, m ore rapid progressive renal involvement and a higher acute mortality rate.

Antineutrophil cytoplasmic antibodies crescentic allograft glomerulonephritis after sofosbuvir therapy

Antineutrophil cytoplasmic antibodies(ANCA) are well known to be associated with several types of vasculitis, including pauci-immune crescentic glomerulonephritis, a form of rapid progressive glomerular nephritis(RPGN). ANCA vasculitis has also been reported after administration of propylthiouracil, hydralazine, cocaine(adulterated with levimasole), allopurinol, penicillamine and few other drugs. All previously reported cases of drug-associated ANCA glomerulonephritis were in native kidneys. Sofosbuvir is a new and effective drug for hepatitis C virus infection. Here, we report a case of ANCA vasculitis and RPGN following sofosbuvir administration in a kidney transplant recipient. It also represents the first case of drug-associated ANCA vasculitis in a transplanted kidney. Further drug monitoring is necessary to elucidate the degree of association and possible causal effect of sofosbuvir and perinuclear ANCA vasculitis.

Antineutrophil cytoplasmic antibody associated vasculitides with renal involvement: Open challenges in the remission induction therapy

Renal involvement with rapidly progressive glomeru-lonephritis is a common manifestation of antineutrophil cytoplasmic antibody(ANCA) associated vasculitides, which is characterized by end-stage renal disease and high mortality rates in untreated and/or late referral patients. The long-term renal survival has improved dramatically since the addition of cyclophosphamide(CYC) and recently of rituximab(RTX) in association with corticosteroids in the remission induction thera-peutic regimens. However, renal prognosis remains unfavorable for many patients and the mortality rate is still significantly high. In this review, we analyze the open challenges to be addressed to optimize the induction remission therapy, principally in patients with advanced kidney failure. This concern the first-line therapy(CYC or RTX) based on different parameters(estimated glomerular filtration rate at baseline, new or relapsed disease, ANCA specificity, tissue injury, safety), the role of plasma exchange and the role of new therapies. Indeed, we discuss future perspectives in induction remission therapy by reporting recent advances in new targeted therapies with particular reference to avacopan, an orally administered selective C5a receptor inhibitor.

Systemic lupus erythematosus and antineutrophil cytoplasmic antibody-associated vasculitis overlap syndrome in a 77-year-old man: A case report

BACKGROUND Systemic lupus erythematosus(SLE)and antineutrophil cytoplasmic antibodyassociated vasculitis(AAV)are classically thought to cause renal impairment and small vessel vasculitis with different pathophysiologies.Their overlap constitutes a rare rheumatologic disease.To date,only dozens of such cases with biopsyproven glomerulonephritis have been reported worldwide typically in women of childbearing age.Here,we present a unique clinical case due to its rarity and individualized treatment of a Chinese man in his eighth decade of life.CASE SUMMARY A 77-year-old man was admitted to several hospitals for shortness of breath and received nonspecific treatments over the past 3 years.As his symptoms were not completely relieved,he visited our hospital for further treatment.Laboratory examinations revealed kidney dysfunction,severe anaemia,hypocomplementemia,glomerular proteinuria,and microscopic haematuria.Antinuclear antibodies,as well as anti-dsDNA antibodies,were positive.Computed tomography of the chest showed right pleural effusion.Renal biopsy was performed,and histology suggested crescentic glomerulonephritis,pauci-immune type.After treatment with plasmapheresis,glucocorticoid,and cyclophosphamide,the disease was in remission,and the patient remained in a stable condition for over 3 years post-hospital discharge.CONCLUSION Due to its complexity and rarity,SLE and AAV overlap syndrome is easily misdiagnosed.An accurate diagnosis and treatment at the earliest stage may significantly improve the condition and reduce irreversible organ injury.

Myeloperoxidase-antineutrophil cytoplasmic antibody-associated vasculitis with headache and kidney involvement at presentation and with arthralgia at relapse:A case report

BACKGROUND Patients with proteinase 3-antineutrophil cytoplasmic antibody associated vasculitis(AAV)experience different manifestations at the initial onset and relapse.However,such cases of different initial and relapse manifestations have not been reported in myeloperoxidase(MPO)-AAV patients.CASE SUMMARY A 52-year-old woman was admitted to our hospital because of headache.Laboratory findings indicated nephrotic range proteinuria and microscopic hematuria,serum creatinine of 243μmol/L,anti-MPO antibody titer of>400 RU/mL,and positive perinuclearantineutrophil cytoplasmic antibody.Renal biopsy showed pauci-immune crescentic glomerulonephritis.The cerebrospinal fluid examination and brain magnetic resonance imaging did not show any abnormality.Therefore,MPO-AAV was diagnosed.Corticosteroids,plasmapheresis,and cyclophosphamide as induction therapy and mycophenolate mofetil(MMF)as maintenance therapy were administered.The patient’s headache disappeared;serum creatinine returned to normal;complete remission of microscopic hematuria and proteinuria was observed.Anti-MPO antibody titer reached normal limits after immunosuppressive treatment.Twenty-five months after stopping the immunosuppressive treatment,the patient relapsed with arthralgia,without neurological or renal involvement.The patient’s arthralgia improved after treatment with prednisone and MMF.CONCLUSION We have reported a rare case of MPO-AAV who initially presented with headache and kidney involvement.However,relapse presented with only arthralgia,which was completely different from the initial manifestations.This case suggests that AAV relapse should be highly suspected in MPO-AAV patients after remission,when clinical manifestations at relapse are different from those at onset.Prednisone and MMF may provide a good choice for refractory arthralgia during relapse in MPO-AAV patients.

Olfactory dysfunction in antineutrophil cytoplasmic antibodyassociated vasculitides: A review of the literature

Olfactory dysfunction(OD)has been described in patients with antineutrophil cytoplasmic antibody-associated vasculitides(AAV),but the underlying mechanisms are not completely understood.The causes of altered smell function can generally be divided into conductive,sensorineural or others.To date no specific treatment is available for AAV-related OD and the efficacy of currently available options has not been explored.The aim of this review is to provide an overview of the causes that may lead to OD in patients with AAV.Current available treatments for OD and possible options in patients with AAV presenting with smell impairment are also mentioned.

Risk factors for renal outcomes in children with antineutrophil cytoplasmic antibody-associated vasculitis:a nationwide retrospective study in China

Background Pediatric antineutrophil cytoplasmic antibody-associated vasculitis(AAV)is a life-threatening systemic vasculitis featured by liability to renal involvement.However,there are few studies on the risk factors and predictive models for renal outcomes of AAV in children.Methods Data from 179 AAV children in multiple centers between January 2012 and March 2020 were collected retrospectively.The risk factors and predictive model of end-stage renal disease(ESRD)in AAV were explored.Results Renal involvement was the most typical manifestation(95.5%),and the crescent was the predominant pathological lesion(84.9%).The estimated glomerular filtration rate(eGFR)was evaluated in 114 patients,of whom 59.6%developed ESRD,and the median time to ESRD was 3.20 months.The eGFR[P=0.006,odds ratio(OR)=0.955,95%confidence interval(CI)=0.924–0.987]and the percentages of global glomerulosclerosis(pGGS;P=0.018,OR=1.060,95%CI=1.010–1.112)were independent risk factors for ESRD of renal biopsy.Based on the pGGS and eGFR at renal biopsy,we developed three risk grades of ESRD and one predictive model.The Kaplan‒Meier curve indicated that renal outcomes were significantly different in different risk grades(P<0.001).Compared with serum creatinine at baseline,the predictive model had higher accuracy(0.86 versus 0.58,P<0.001)and a lower coefficient of variation(0.07 versus 0.92)in external validation.Conclusions Renal involvement is the most common manifestation of pediatric AAV in China,of which more than half deteriorates into ESRD.The predictive model based on eGFR at renal biopsy and the pGGS may be stable and accurate in speculating the risk of ESRD in AAV children.

Murtagh安全诊断策略联合思维导图构建临床思维在全科教学门诊中的应用

全科教学门诊是培养住院医师规范化培训(简称住培)全科医生临床思维、提升全科诊疗能力的重要培训方法。本文用1例以乏力为主要表现的患者为教学案例,介绍全科教学门诊中采用澳大利亚著名全科医学专家John Murtagh提出的Murtagh安全诊断策略启发住培全科医生对乏力进行诊断与鉴别诊断:(1)引起乏力的常见疾病有哪些?(2)哪些重要疾病是不能忽视的?(3)乏力有什么容易被遗漏的疾病?(4)是否存在潜在的容易被掩盖的疾病?(5)患者是否有话没有说?结合病史、体格检查和实验室检查结果初步诊断为抗中性粒细胞胞浆抗体相关性血管炎引起急进性肾小球肾炎可能性大,及时转诊,肾病理检查确诊乏力的病因为抗中性粒细胞胞浆抗体相关性肾小球肾炎,取得满意疗效。带教老师基于Murtagh安全诊断策略帮助住培全科医生构建乏力鉴别、分析的系统知识框架,从而提升住培全科医生的临床逻辑思维能力和解决临床实际问题的能力;以思维导图为辅助工具,将Murtagh安全诊断策略诊断与鉴别诊断思路可视化、形象化,优化教学效果。

Antineutrophil cytoplasmic antibodies-associated glomerulonephritis:From bench to bedside

Antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is a group of autoimmune disorders that pre-dominantly affects small vessels. The onset of the disease is closely associated with ANCA. Renal involvement, also known as ANCA-associated glomerulonephritis (AGN), is one of the most common manifestations of AAV. In this mini-review, we described the clinical and pathological features of AGN. We then focused on recent studies on the mechanism of acute kidney lesions, including fibrinoid necrosis and crescent formation. Following the basic aspects of kidney injury in AGN, we demonstrated the clinical importance of kidney injury in determining the outcome of patients with AGN. The prognostic value of the 2010 Histopathological Classification of AGN and validating studies were summarized. Finally, treatment and novel therapeutic strategies were introduced addressing the importance of optimizing management of this patient population.

抗中性粒细胞胞质抗体相关性肥厚性硬脑膜炎患者临床特点及影像学分析

目的分析抗中性粒细胞胞质抗体(anti-neutrophil cytoplasmic antibody,ANCA)相关性肥厚性硬脑膜炎(hypertrophic pachymeningitis,HP)患者的临床特点。方法回顾性收集2015年8月至2022年6月作者单位收治的10例ANCA相关性HP患者,其中来自中南大学湘雅医院9例,陕西省人民医院1例,男、女各5例,发病年龄43~80岁。分析患者的临床特征、影像学特点、诊治方法及疗效。结果10例患者表现为不同程度的慢性头痛或脑神经受累症状,病程1~84个月。5例患者血清髓过氧化物酶(myeloperoxidase,MPO)和核周型ANCA(perinuclear ANCA,p-ANCA)同时阳性,2例患者仅血清MPO阳性,p-ANCA和细胞质型ANCA(cytoplasmic ANCA,c-ANCA)阳性各1例,p-ANCA和c-ANCA同时阳性1例。6例视力受累,2例听力受累。增强MRI显示患者不同程度的硬脑膜增厚并强化,其中弥漫性增厚4例,局灶性增厚6例,均未累及脑实质。1例患者单用糖皮质激素治疗,1例患者行糖皮质激素联合丙球治疗,8例患者糖皮质激素联合免疫抑制剂治疗,其中1例同时使用利妥昔单抗,经治疗后患者症状均明显缓解。2例在激素和免疫抑制剂减量过程出现头痛加重,再次治疗后症状缓解。结论ANCA相关性HP病程长短不一,临床表现不典型,增强MRI显示的硬脑膜增厚部位在不同ANCA抗体阳性患者间有重叠;糖皮质激素和免疫抑制剂治疗ANCA相关性HP有效。

从“五脏痹”理论探析抗中性粒细胞胞质抗体相关性血管炎的病机及治疗

抗中性粒细胞胞质抗体(ANCA)相关性血管炎在临床上较为少见,其症状常随损伤部位不同而表现各异,本病多累及肺、肾、消化、心血管等系统,其表现同中医“五脏痹”证候相似。本文以ANCA相关性血管炎的多脏器受累与中医“五脏痹”症候的相关性为基,根据各脏临床表现不同分别从“肺痹、肾痹、脾痹、肝痹、心痹”论治,体现了中医“以五脏为中心”的理论特点,并为ANCA相关性血管炎的中医诊疗提供新的辨证思路和诊疗依据。

以周围神经病为首发症状的抗中性粒细胞胞浆抗体相关性血管炎的临床特点

目的探讨以周围神经病为首发症状的抗中性粒细胞胞浆抗体(ANCA)相关性血管炎(AAV)的临床特征,为临床诊治提供思路。方法总结2017年至2021年在中南大学湘雅医院就诊的6例以周围神经病为首发症状的AAV患者的临床资料。结果6例均为中老年男性,首发症状主要为肢体麻木、疼痛、无力。除神经系统受累外,部分患者伴有上呼吸道、肺部、肾脏、眼睛、耳、皮肤及全身关节受累,EMG提示多发性周围神经损害;5例患者pANCA、MPO阳性,1例患者cANCA、PR3阳性;经糖皮质激素和环磷酰胺治疗后,临床症状及实验室指标均有不同程度的改善。结论以周围神经病变为首发症状的AAV临床表现缺乏特异性,AAV的早期诊断和治疗可以改善患者生活质量和预后。

误诊为肺结核瘤的ANCA相关性血管炎临床分析

目的探讨抗中性粒细胞胞质抗体(ANCA)相关性血管炎的误诊原因及防范措施。方法回顾分析1例曾误诊的ANCA相关性血管炎的临床资料。结果本例因下肢红疹伴溃疡入院。曾行“鼻窦炎”手术,术前筛查发现肺部肿物行“肺部肿物切除术”,术后病理示肺结核瘤,抗结核治疗无效。入院后结合患者多系统受累表现及实验室、肾穿刺病理检查等诊断为ANCA相关性血管炎,给予糖皮质激素及调节免疫等治疗,症状缓解。结论ANCA相关性血管炎临床表现多样且不典型,易误诊。临床中应对疑似病例尽早行相关医技检查。

抗中性粒细胞胞质抗体阳性表达在儿童狼疮肾炎中的临床价值研究

目的探讨抗中性粒细胞胞质抗体(antineutrophilic cytoplasmic antibody,ANCA)阳性在狼疮肾炎(lupus nephritis,LN)患儿中疾病活动性以及对疾病严重程度的影响,为临床医生快速明确诊断,提供合理临床决策,规范诊治提供循证医学证据,为系统性红斑狼疮诊疗研究提供新的思路。方法收集2014年1月到2022年4月期间在昆明市儿童医院肾脏风湿免疫科及重症监护室接受住院治疗的LN患儿170例,其中完成ANCA检查的患儿共93例,ANCA阳性有33例,将其定为实验组,其余60例ANCA阴性患儿为对照组,将两组患儿的临床表现、实验室检查、SLEDAI评分、治疗及预后进行比较。结果LN患儿中最常见表现为蛋白尿(59.13%),其他常见表现依次为发热(48.38%)、水肿(39.78%)、血尿(34.4%);ANCA阳性患儿的肾功能损害程度明显高于ANCA阴性组患儿[ANCA阳性组肌酐(75.17±45.85)μmol/L;ANCA阴性组肌酐(55.63±41.78)μmol/L];ANCA阳性组24 h尿蛋白定量高于ANCA阴性组[(1.61±0.12)g比(1.02±0.38)g];ANCA阳性与ANCA阴性的LN患儿血常规、肝肾功能、血脂、免疫球蛋白、补体、炎症指标等差异无统计学意义;ANCA阳性组的患儿SLEDAI评分明显高于ANCA阴性组(SLEDAI评分平均数分别为15.62和11.78,P=0.033);ANCA阳性组生存率低于阴性组。结论ANCA阳性的LN患儿疾病活动性更强、基础肾功能更差、临床预后不良的可能性更大。

抗中性粒细胞胞质抗体相关性血管炎病人血小板增多的临床意义

目的:分析抗中性粒细胞胞质抗体相关性血管炎(AAV)病人周围血中血小板增多的临床意义。方法:分析110例AAV病人临床与相关实验室资料,根据入院后首次周围血小板计数结果分为血小板正常组(n=67例)和血小板增多组(n=43例),对比2组病人的临床及相关实验检查数据,定期随访了解病人的生存情况。结果:单因素分析发现,2组性别、年龄、周围白细胞计数、中性粒细胞计数、淋巴细胞计数,血清补体C3水平,红细胞沉降率、血清C反应蛋白差异均有统计学意义(P<0.05~P<0.01)。logistic回归分析发现性别、周围血白细胞计数、中性粒细胞计数是影响AAV病人合并血小板增多的独立危险因素(P<0.05)。血小板增多组强化治疗使用率相较于血小板正常组显著升高(P<0.05)。2组病人死亡率差异有统计学意义(P<0.01)。结论:AAV病人周围血小板增多时的病情较重,预后较差,应积极干预,通过早诊断、早治疗来改善预后。

中国移植肾系统性疾病肾损害复发临床诊疗指南

原发病复发是影响移植肾近期和远期存活的重要原因,越来越受到重视。系统性疾病肾损害在肾脏移植术后均有可能复发,并不同程度损伤移植肾。随着对系统性疾病肾损害发病机制的深入认识,移植肾系统性疾病肾损害复发的诊治水平也在逐渐提升。中华医学会器官移植学分会组织器官移植专家,充分阅读、分析和总结目前国际和国内的文献,在《慢性移植肾功能不全诊疗技术规范(2019版)》的基础上,对系统性疾病肾损害复发的危险因素、预防措施、治疗措施及预后等内容,依据推荐评估、发展和评价分级方法对证据质量和建议强度进行客观评估,制定《中国移植肾系统性疾病肾损害复发临床诊疗指南》,在本指南中对相应临床问题提出推荐意见,以更好地保障和促进移植肾脏和受者的长期存活。