Rituximab Induced Vasculitis: Dose the Antigen-Antibody Complex of Rituximab Play a Role in Developing Leukocytoclastic Vasculitis?—A Case Report and Review of the Literature

Rituximab is a monoclonal antibody that targets CD20, which is a specific B-cell surface antigen. It was the first monoclonal antibody that was approved for the treatment of non-Hodgkin lymphoma, rheumatoid arthritis, and other cutaneous lymphoid malignancies. There are many off-label uses of rituximab, such as systemic lupus erythematosus, autoimmune hemolytic anemia, multiple sclerosis, graft-versus-host disease, chronic lymphocytic leukemia, and chronic immune-mediated thrombocytopenia. Among the rare side effects associated with rituximab treatment is vasculitis, more specifically, leukocytoclastic vasculitis. Here, we describe a 21-year-old Saudi female with leukocytoclastic vasculitis occurring three months after treatment with rituximab.

American tegumentary leishmaniasis mimicking myiasis and granulomatous vasculitis:A case report

Rationale:American tegumentary leishmaniasis comprises cutaneous and mucocutaneous manifestations caused by parasitic infections by various Leishmania species.This report details the clinical interventions for a patient with American tegumentary leishmaniasis in Mendoza,Argentina,a non-endemic region.Patient concerns:A 43-year-old male was admitted to a tertiary care hospital in Mendoza,Argentina Republic with a history of progressive nasal discharge,septal perforation,facial pain,and pruritus.Despite treatment for presumed nasal myiasis and vasculitis with granulomatosis,symptoms persisted.Diagnosis:American tegumentary leishmaniasis.Interventions:Intravenous liposomal amphotericin B.Outcomes:Follow-up at 30 days showed no recurrence of symptoms with a remarkable clinical improvement of the nasal lesion.Lessons:This case sheds light on the necessity of accurate identification for timely intervention and the need to recognize the diverse manifestations of American tegumentary leishmaniasis to avoid misdiagnosis.

高dV/dt电压下航空线缆局部放电仿真及特性分析

多电飞机发展中,电压等级与功率密度不断提升,新型电力电子器件的应用将有效提升飞机能量密度,但同时增加高dV/dt脉冲电压下局部放电风险。该文基于电场模型开展联合仿真,通过数值计算、场强分析,及试验对比,对航空线缆典型模型进行局部放电测试,深入探究高dV/dt电压下局部放电随上升时间与频率的变化机制。结果表明:上升时间的缩短对线缆局部放电有着明显的加强作用,上升时间50ns较250ns时模型的累计放电幅值与放电次数增加超80%,放电时延、气隙电场恢复时间也明显缩短。电压频率的增加对线缆局部放电的影响呈现“拐点”机制,1到10kHz附近,介质损耗效应对线缆放电有着明显的促进作用,模型放电幅值、放电次数均呈现增加趋势;更高频段内,电平时间缩短主导的电荷累积效应对放电起到抑制作用。结果可为多电飞机电气系统中高频、高压、高dV/dt波形下的航空线缆绝缘设计、检测与评估提供一定的借鉴和参考。

Endoscopic and radiographic features of gastrointestinal involvement in vasculitis

Vasculitis is an inflammation of vessel walls,followed by alteration of the blood flow and damage to the dependent organ.Vasculitis can cause local or diffuse pathologic changes in the gastrointestinal (GI) tract.The variety of GI lesions includes ulcer,submucosal edema,hemorrhage,paralytic ileus,mesenteric ischemia,bowel obstruction,and life-threatening perforation.The endoscopic and radiographic features of GI involvement in vasculitisare reviewed with the emphasis on small-vessel vasculitis by presenting our typicalcases,including Churg-Strauss syndrome,HenochSch nlein purpura,systemic lupus erythematosus,and Beh et's disease.Important endoscopic features are ischemic enterocolitis and ulcer.Characteristic computed tomographic findings include bowel wall thickening with the target sign and engorgement of mesenteric vessels with comb sign.Knowledge of endoscopic and radiographic GI manifestations can help make an early diagnosis and establish treatment strategy.

Ischemic colitis associated with intestinal vasculitis: Histological proof in systemic lupus erythematosus

I schemic colitis is an uncommon complication in patients with systemic lupus erythematosus (SLE). In previously reported cases of colitis caused by SLE, intestinal vasculitis is implicated as the causative process, but is rarely confirmed histologically. We described a case of a 32-year-old man with increased activity of SLE, who presented with hematochezia and abdominal pain due to ischemic colitis with small vessel vasculitis which was proven by sigmoidoscopic biopsy. The clinical course of the patient was improved after steroid and conservative management.

TC4-DT钛合金切削加工参数研究

针对YG8和TiAlN涂层硬质合金两种刀具,通过单因素车削、低速铣削及正交高速铣削加工试验,探究刀具切削工艺参数对TC4-DT钛合金加工件表面粗糙度、表层硬度的影响规律。实验结果表明:钛合金的表面粗糙度随着切削三要素发生变化,切削速度越高,粗糙度越低;进给量越大,粗糙度越大;但随切削深度波动变化。使用TiAlN涂层硬质合金立铣刀进行加工得到的平均表面粗糙度小于YG8硬质合金立铣刀,且加工表面硬度变化更小,更适合用于TC4-DT的铣削加工。

Henoch-Schonlein purpura from vasculitis to intestinal perforation: A case report and literature review

Henoch-Sch?nlein purpura(HSP) is generally a selflimited vasculitis disease and has a good prognosis. We report a 4-year-old Thai boy who presented with palpable purpura, abdominal colicky pain, seizure, and eventually developed intestinal ischemia and perforation despite adequate treatment, including corticosteroid and intravenous immunoglobulin therapy. Imaging modalities, including ultrasonography and contrastenhanced computed tomography, could not detect intestinal ischemia prior to perforation. In this patient, we also postulated that vasculitis-induced mucosal ischemia was a cause of the ulcer, leading to intestinal perforation, and high-dose corticosteroid could have been a contributing factor since the histopathology revealed depletion of lymphoid follicles. Intestinal perforation in HSP is rare, but life-threatening. Close monitoring and thorough clinical evaluation are essential to detect bowel ischemia before perforation, particularly in HSP patients who have hematochezia, persistent localized abdominal tenderness and guarding. In highly suspicious cases, exploratory laparotomy may be needed for the definite diagnosis and prevention of further complications.

Two uncommon manifestations of leptospirosis:Sweet's syndrome and central nervous system vasculitis

To leptospirosis is the commonest spirocheatal infection in the tropical and temperate countries of Indian sub-continent and Africa and the most common zoonosis worldwide.The protean manifestation of this infectious disease is a challenge for practising clinicians across the world. In poor developing countries,at most clinical suspicion it is essential in the diagnosis of this disease.In this report,we are able to document two uncommon manifestations of leptospirosis, namely Sweet’s syndrome and central nervous system vasculitis.

Prolonged small vessel vasculitis with colon mucosal inflammation as first manifestations of Behet's disease

Behet's disease is a chronic, relapsing, systemic vasculitis of unknown aetiology. Patients present manifestations of gastrointestinal complications, including mouth lesions, small and large intestinal lesions, and vascular lesions in the abdomen. In some cases, the intestinal ulcers of patients with Behet's disease are indistinguishable from those of Crohn's disease, tuberculosis, vasculitis and other diseases. In this article, we present a case of atypical Behet's disease with a complicated medical history and multisystem damage, for the purpose of better management of this disease.

Idiopathic hypereosinophilic syndrome presenting with severe vasculitis successfully treated with imatinib

Idiopathic hypereosinophilic syndrome(HES) is a rare disorder characterized by peripheral eosinophilia exceeding 1500/mm3, a chronic course, absence of secondary causes, and signs and symptoms of eosinophil-mediated tissue injury. One of the best-characterized forms of HES is the one associated with FIP1L1-PDGFRA gene rearrangement, which was recently demonstrated as responsive to treatment with the small molecule kinase inhibitor drug, imatinib mesylate. Here, we describe the case of a 51-year-old male, whose symptoms satisfied the clinical criteria for HES with cutaneous and cardiac involvement and who also presented with vasculitic brain lesions and retroperitoneal bleeding. Molecular testing, including fluorescence in situ hybridization, of bone marrow and peripheral blood showed no evidence of PDGFR rearrangements. The patient was initially treated with high-dose steroid therapy and then with hydroxyurea, but proved unresponsive to both. Upon subsequent initiation of imatinib mesilate, the patient showed a dramatic improvement in eosinophil count and progressed rapidly through clinical recovery. Long-term follow-up confirmed the efficacy of treatment with low-dose imatinib and with no need of supplemental steroid treatment, notwithstanding the absence of PDGFR rearrangement.

Vanishing cerebral vasculitis in a patient with Lewy pathology

Immune-mediated mechanisms are involved in the pathogenesis of both cerebral vasculitis and Parkinson’s disease（PD, brainstem-predominant Lewy pathology）, but the presentation of cerebral vasculitis with comorbid Lewy pathology has not yet been reported. Here we present a case of pathologically confirmed vasculitis in a 73-year-old male patient whose postmortem examination revealed Lewy pathology diagnostic of PD. This case study suggests a comorbidity of cerebral vasculitis and Lewy pathology, as well as potential pathogenic interactions between these two disorders with immune-mediated mechanisms.

Human hepatitis viruses-associated cutaneous and systemic vasculitis

Human hepatitis viruses(HHVs)include hepatitis A virus,hepatitis B virus(HBV),hepatitis C virus(HCV),hepatitis delta virus,and hepatitis E virus and can cause liver inflammation in their common human host.Usually,HHV is rapidly cleared by the immune system,following acute HHV invasion.The morbidities associated with hepatitis A virus and hepatitis E virus infection occur shortly after their intrusion,in the acute stage.Nevertheless,the viral infectious process can persist for a long period of time,especially in HBV and HCV infection,leading to chronic hepatitis and further progressing to hepatic cirrhosis and liver cancer.HHV infection brings about complications in other organs,and both acute and chronic hepatitis have been associated with clinical presentations outside the liver.Vascular involvement with cutaneous and systemic vasculitis is a well-known extrahepatic presentation;moreover,there is growing evidence for a possible causal relationship between viral pathogens and vasculitis.Except for hepatitis delta virus,other HHVs have participated in the etiopathogenesis of cutaneous and systemic vasculitis via different mechanisms,including direct viral invasion of vascular endothelial cells,immune complex-mediated vessel wall damage,and autoimmune responses with stimulation of autoreactive B-cells and impaired regulatory T-cells.Cryoglobulinemic vasculitis and polyarteritis nodosa are recognized for their association with chronic HHV infection.Although therapeutic guidelines for HHV-associated vasculitis have not yet been established,antiviral therapy should be initiated in HBV and HCV-related systemic vasculitis in addition to the use of corticosteroids.Plasma exchange and/or combined cyclophosphamide and corticosteroid therapy can be considered in patients with severe life-threatening vasculitis manifestations.

Systemic Vasculitis:An Important and Underestimated Cause of Malignant Hypertension

Objectives:Malignant hypertension(MHT)is defi ned as severe hypertension accompanied by ischemic failure of one or more organs.The aims of this study were to evaluate the current clinical and etiologic profi les of MHT.Methods:As a retrospective study,we selected all patients admitted to our center from January 2013 to December 2016.Seventy patients with MHT were included.Results:The average age of the patients was 40 years,and more than half of the patients were male(78.57%).There were 24 patients with essential hypertension,accounting for 34.29%of the patients,and 46 with secondary hypertension,accounting for 65.71%of the patients.For secondary MHT,systemic vasculitis(25.57%)was the most common cause,followed by severe obstructive sleep apnea syndrome(15.71%),primary renal parenchymal hypertension(11.43%),primary aldosteronism(7.14%),and Cushing syndrome(1.43%)and nutcracker phenomenon(1.43%).Twenty patients with systemic vasculitis were characterized by severe hypertension accompanied by damage to two or more target organs of differing severity.The levels of white blood cells,hypersensitive C-reactive protein,serum creatinine,and 24-hour urinary protein were above their normal range.Conclusion:Systemic vasculitis may be one of the main causes of MHT,and has been underestimated in the past.In future clinical work,clinicians need to pay more attention to patients with systemic vasculitis.

Relapsing polychondritis with p-ANCA associated vasculitis: Which triggers the other?

Relapsing polychondritis(RP) is a rare autoimmune disease with chronic inflammatory/destructive lesions of the cartilaginous tissues. In one third of the cases it is associated with other autoimmune disorders, mostly with anti-neutrophil cytoplasmic antibody(ANCA) associated vasculitis(AAV). We report three cases of RP with p-ANCA positive AAV. In the first patient RP developed 1.5 years after the onset of AAV. In the others the signs of RP were present before the onset of severe crescent glomerulonephritis. Patients responded well on steroid and cyclophosphamide. In dialysis dependent cases plasmapheresis was also used successfully. During the 2 and 1.5 years of follow up, they were symptom-free, and had stable glomerular filtration rate. The first patient died after four years of follow-up due to the complications of sudden unset pancytopenia,which raises the possibility of associated hemophagocytic syndrome. In the setting of RP or AAV physicians should always be aware of the possibility of sudden or insidious appearance of the other disease.

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.

Correlations of Serological Markers with Development of Systemic Involvement in Adult Immunoglobulin A Vasculitis:A Retrospective Study of 259 Patients in Central China

Objective:Although relatively rare,adult immunoglobulin A vasculitis(IgAV)can lead to severe complications and longer hospitalization,and result in poor prognosis,when compared to childhood IgAV.Hence,early identification and prevention for patients prone to develop systemic involvement are essential.The purpose of this study was to explore the correlations of common serological markers with the development of systemic involvement in adult IgAV.Methods:A retrospective analysis was performed for adult IgAV patients,who were hospitalized in Wuhan Union Hospital between January 2016 and December 2019.A total of 259 patients were enrolled,and the pre-treatment serological markers were comprehensively assessed.Results:In the present study,49.0% and 33.2% of patients developed renal and gastrointestinal(GI)involvement,respectively.Furthermore,the elevated levels of white blood cells count,D-Dimer(D-D),C-reactive protein(CRP)and neutrophil granulocyte ratio(NE%)>60% were significantly associated with GI involvement in the univariate analysis,while the decrease in high density lipoprotein level,and the elevated D-D and CRP levels were significantly associated with renal involvement(P<0.05).Moreover,a prediction model that combined multiple markers was established by performing a logistic regression analysis,and this presented a more favorable value of prediction than the individual serological markers.Conclusion:The present study suggests that common serological markers have close correlations with systemic involvement in adult IgAV,and that the establishment of a prediction model for systemic involvement may be helpful in facilitating personalized therapeutic strategies and clinical management for IgAV patients.

A rare case of mitral valve dissection and aortic-left ventricular tunnel associated with possible autoimmune vasculitis

We report a very rare case of mitral valve dissection and aorticleft ventricular tunnel caused by possible autoimmune vasculitis.We suspected Behcet’s disease in this patient.There was no obvious clinical evidence of infective endocarditis.Echocardiography is the diagnostic tool of choice to recognize valvular dysfunction,related pathology and possible complications.The patient may require immunosuppressive therapy due to the high likelihood of recurrence perioperation period.

高dV/dt方波电压下高压功率模块封装绝缘局部放电特性研究

高dV/dt方波下封装绝缘发生的局部放电(简称“局放”)是高压功率模块失效的重要原因之一,然而方波上升下降沿产生的电磁干扰和位移电流严重干扰局放的检测,且现行功率模块局放检测标准规定的正弦电压与方波下测试结果存在差异。为解决上述问题,该文提出基于下混频原理的高dV/dt方波电压下局放检测方法,探究不同频率单极性高dV/dt方波和工频正弦电压下封装绝缘的局放特性,并对封装绝缘固—固结构建模,考虑复合绝缘的电荷弛豫,进行动态电场分布仿真计算。结果表明,由于界面电荷弛豫造成的局部电场差异,单极性方波下,封装绝缘的局放起始电压(partial discharge inception voltage,PDIV)高于正弦电压下,且方波下PDIV随频率升高略有增加。外施电压幅值相同时,方波下局放平均幅值高于正弦电压下。随频率升高,单极性方波上升沿处局放幅值分布相近,而下降沿处局放平均幅值降低,相位呈现滞后趋势。该研究分析高dV/dt方波下封装绝缘的局放特点及其与正弦电压下的差异,可为功率模块可靠性检测和评估提供一定参考。

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.

rare type of pancreatitis as the first presentation ofanti-neutrophil cytoplasmic antibody-related vasculitis

A pancreatic tumor was suspected on the abdominal ultrasound of a 72-year-old man. Abdominal computed tomography showed pancreatic enlargement as well as a diffuse, poorly enhanced area in the pancreas; endoscopic ultrasound-guided fine needle aspiration biopsy and endoscopic retrograde cholangiopancreatography failed to provide a definitive diagnosis. Based on the trend of improvement of the pancreatic enlargement, the treatment plan involved follow-up examinations. Later, he was hospitalized with an alveolar hemorrhage and rapidly progressive glomerulonephritis; he tested positive for myeloperoxidase-anti-neutrophil cytoplasmic antibody(ANCA) and was diagnosed with ANCArelated vasculitis, specifically microscopic polyangiitis. It appears that factors such as thrombus formation caused by the vasculitis in the early stages of ANCArelated vasculitis cause abnormal distribution of the pancreatic blood flow, resulting in non-uniform pancreatitis. Pancreatic lesions in ANCA-related vasculitis are very rare. Only a few cases have been reported previously. Therefore, we report our case and a review of the literature.