This mini review explores the links between anti-neutrophil cytoplasmic antibody-associated vasculitis(AAV)and kidney cancer.Several studies suggest an increased incidence of cancer for patients with AAV.Different can...This mini review explores the links between anti-neutrophil cytoplasmic antibody-associated vasculitis(AAV)and kidney cancer.Several studies suggest an increased incidence of cancer for patients with AAV.Different cancer types have shown different standardized incidence ratios(SIRs)in association with AAV.The SIRs of kidney cancer were found to be between 1.7 and 3.3 as per three retrospective data analyses.This association is likely multifactorial,with increased de novo cancer risks associated with inflammatory diseases;carcinogenic therapies such as cyclophosphamide;and reduced immune surveillance of neoplastic cells in immunocompromised individuals.Some studies have proposed that cancers,including kidney cancer,could be a potential trigger for AAV.Due to variability in SIRs and a lack of multicenter studies looking specifically into the incidence of kidney cancer at AAV diagnosis and on follow-up post initiation of AAV treatment,there remains a lack of evidence to support formal screening for kidney cancer in the AAV patient cohort.Greater awareness on the increased risk of cancer in AAV patients,prompt urological assessment of“red flag”symptoms of kidney cancer,and smoking cessation advice to reduce cancer risk should be standard of care for patients with AAV.展开更多
BACKGROUND Hepatitis B virus(HBV)infection can lead to renal involvement,commonly manifested as HBV-associated glomerulonephritis(HBV-GN),which typically presents as nephrotic or nephritic syndrome.Antineutrophil cyto...BACKGROUND Hepatitis B virus(HBV)infection can lead to renal involvement,commonly manifested as HBV-associated glomerulonephritis(HBV-GN),which typically presents as nephrotic or nephritic syndrome.Antineutrophil cytoplasmic antibody-associated vasculitis(AAV)is a systemic disease characterized by immune necrotizing inflammation of small blood vessels involving multiple organs with complex and severe clinical implications.The coexistence of HBV-GN and AAV is sporadic,with limited data existing regarding its diagnosis,management,clinical outcomes,and prognosis,especially in patients with AAV.CASE SUMMARY This manuscript presents the case of an older male patient who presented with persistent foamy urine lasting over two weeks.Initial clinical findings included nephrotic syndrome and renal insufficiency,which subsequently progressed to involve the lungs,immune system,hematologic system,and other organ systems.The patient was diagnosed with HBV-GN complicated by AAV,a rare and complex condition.Despite receiving comprehensive treatment,including corticosteroids,cyclophosphamide for immune regulation,plasma exchange,and immunoadsorption targeting antineutrophil cytoplasmic antibody-associated antibodies,the patient required long-term dialysis and demonstrated a poor prognosis.CONCLUSION HBV infection may trigger nephropathy with AAV.Early recognition and intervention are crucial for improving patient prognosis.展开更多
BACKGROUND This case report describes myeloperoxidase-anti-neutrophil cytoplasmic antibody associated vasculitis with kidney involvement in a patient with relapsing polychondritis,which was successfully treated with A...BACKGROUND This case report describes myeloperoxidase-anti-neutrophil cytoplasmic antibody associated vasculitis with kidney involvement in a patient with relapsing polychondritis,which was successfully treated with Avacopan.Although relapsing polychondritis has been associated with anti-neutrophil cytoplasmic antibody-associated vasculitis,overlap can result in severe organ involvement,particularly renal damage progressing to end-stage kidney disease.This case presents a unique opportunity to evaluate the potential role of Avacopan as an alternative therapeutic option in managing myeloperoxidase-anti-neutrophil cytoplasmic antibody-associated vasculitis in the context of relapsing polychon-dritis highlighting a positive renal response despite treatment challenges.This is a case of a 69-year-old Caucasian woman who presented to our hospital’s emergency department with a 4 week history of inflammatory polychondritis affecting the auricular cartilage,accompanied by acute kidney injury.On admission,serum creatinine was elevated at 4.0 mg/dL,which progressively increased to 6.07 mg/dL on day 6.The renal biopsy revealed necrotizing and crescentic glomerulonephritis affecting more than 50%of the glomeruli.She was treated with a total of 2500 mg intravenous methylprednisolone over 3 days followed by oral prednisone.Induction treatment included intravenous cyclophosphamide induction,with plans for a total of 2 doses followed by transition to rituximab.However the patient was unable to tolerate rituximab due to allergic reaction so intravenous cyclophosphamide was continued for a total of 6 doses(cumulative dose 3000 mg).In the setting of persistent acute kidney injury,Avacopan was added to the regimen 3 months after diagnosis.Maintenance therapy included azathioprine in addition to Avacopan.Prednisone gradually tapered off at 6 months.CONCLUSION Avacopan may be beneficial in treating anti-neutrophil cytoplasmic antibody-associated vasculitis with coexisting relapsing polychondritis,especially in cases where preservation of kidney function is critical.Further research will be essential to validate these findings and refine treatment protocols for such complex cases.展开更多
Introduction: Among the chronic and feared complications of this disease, rheumatoid vasculitis stands out as one of the most severe, albeit rare. The most frequently affected sites by rheumatoid vasculitis are the sk...Introduction: Among the chronic and feared complications of this disease, rheumatoid vasculitis stands out as one of the most severe, albeit rare. The most frequently affected sites by rheumatoid vasculitis are the skin and the peripheral nervous system. We report a case of rheumatoid vasculitis complicating a 30-year history of untreated rheumatoid arthritis. Case Report: The patient is a 75-year-old male with a 30-year history of deforming and erosive seropositive rheumatoid arthritis. He presented with polyarthritis and digital necrosis. Physical examination revealed peripheral joint syndrome with characteristic deformities of rheumatoid arthritis. Additionally, there was well-demarcated dry gangrene affecting the first and second digits of the right hand. Laboratory findings indicated an inflammatory syndrome. Tests for antinuclear antibodies (ANA) and anti-neutrophil cytoplasmic antibodies (ANCA) to investigate other causes of vasculitis were negative. The patient received a corticosteroid bolus. After two weeks, there was a marked reduction in pain and an improvement in the general condition. The dry gangrene remained stable. Conclusion: Rheumatoid vasculitis remains a serious and chronic complication of rheumatoid arthritis, associated with significant mortality. This case highlights the crucial importance of early diagnosis and effective management of rheumatoid arthritis.展开更多
Rationale:IgA vasculitis or Henoch-Schonlein purpura is an immune complex mediated small vessel vasculitis characterized by clinical triad of arthritis,palpable purpura and gastrointestinal symptoms.It is commonly see...Rationale:IgA vasculitis or Henoch-Schonlein purpura is an immune complex mediated small vessel vasculitis characterized by clinical triad of arthritis,palpable purpura and gastrointestinal symptoms.It is commonly seen in children below 10-year-old,though adults may be affected.Patient’s Concern:A 40-year-old man with type-2 diabetes mellitus presented with fever,melena,palpable purpuric rash and hematuria with acute kidney injury,associated with nephrotic range proteinuria.Skin biopsy revealed leukocytoclastic vasculitis and renal biopsy showed IgA deposits in the mesangium and capillary loops with fibro cellular crescents confirming the diagnosis of IgA vasculitis with crescentic rapidly progressive glomerulonephritis.Diagnosis:IgA vasculitis with rapidly progressive glomerulonephritis.Interventions:The patient was treated with pulse intravenous methylprednisolone 500 mg/day for 3 days with tapering doses of oral prednisolone and intravenous cyclophosphamide(0.5 gm/m^(2) body surface area)under the cover of intravenous antibiotics for diabetic foot ulcer.Outcomes:Patient improved gradually with return to baseline creatinine after 3 months of follow up.Lessons:Rapidly progressive glomerulonephritis is a rare manifestation of IgA vasculitis and warrants special care and early treatment.The incidence of rapidly progressive glomerulonephritis in IgA vasculitis is unknown.It carries a high risk of progression to chronic kidney disease and thus should be treated as soon as feasible after diagnosis.展开更多
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,...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.展开更多
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 patie...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.展开更多
The clinical spectrum of immunoglobulin A vasculitis nephritis(IgAVN)ranges from the relatively common transitory microscopic hematuria and/or low-grade proteinuria to nephritic or nephrotic syndrome,rapidly progressi...The clinical spectrum of immunoglobulin A vasculitis nephritis(IgAVN)ranges from the relatively common transitory microscopic hematuria and/or low-grade proteinuria to nephritic or nephrotic syndrome,rapidly progressive glomerulonephritis,or even renal failure.Clinical and experimental studies have shown a multifactor pathogenesis:Infection triggers,impaired glycosylation of IgA1,complement activation,Toll-like-receptor activation and B cell proliferation.This knowledge can identify IgAVN patients at a greater risk for adverse outcome and increase the evidence for treatment recommendations.展开更多
BACKGROUND Single-organ vasculitis(SOV)is characterized by inflammation of a blood vessel,affecting one organ,such as the skin,genitourinary system,or the aorta without systemic features.Gastrointestinal SOV is rare,w...BACKGROUND Single-organ vasculitis(SOV)is characterized by inflammation of a blood vessel,affecting one organ,such as the skin,genitourinary system,or the aorta without systemic features.Gastrointestinal SOV is rare,with hepatic artery involvement reported only in two prior published cases.Herein,we presented a case of isolated hepatic artery vasculitis presenting after Pfizer-BioNTech mRNA corona virus disease 2019(COVID-19)vaccination.CASE SUMMARY A 50-year-old woman with hypertension presented to our Emergency Department with recurrent diffuse abdominal pain that localized to the epigastrium and emesis without diarrhea that began eight days after the second dose of the PfizerBioNTech COVID-19 vaccine.Blood work revealed an elevated C-reactive protein(CRP)of 19 mg/L(normal<4.8 mg/L),alkaline phosphatase 150 U/L(normal 25-105 U/L),gamma-glutamyl transferase(GGT)45 U/L(normal<43 U/L)and elevated immunoglobulins(Ig)G 18.4 g/L(normal 7-16 g/L)and IgA 4.4 g/L(normal 0.7-4 g/L).An abdominal computed tomography revealed findings in keeping with hepatic artery vasculitis.A detailed review of her history and examination did not reveal infectious or systemic autoimmune causes of her presentation.An extensive autoimmune panel was unremarkable.COVID-19polymerase chain reaction nasopharyngeal swab,human immunodeficiency virus,viral hepatitis and Heliobacter pylori serology were negative.At six months,the patient’s symptoms,and blood work spontaneously normalized.CONCLUSION High clinical suspicion of SOV is required for diagnosis in patients with acute abdominal pain and dyspepsia.展开更多
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 ...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.展开更多
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 eventuall...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.展开更多
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, b...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.展开更多
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 ...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.展开更多
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 identifica...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.展开更多
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 MH...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.展开更多
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 d...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.展开更多
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 t...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.展开更多
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...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.展开更多
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 L...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.展开更多
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 ...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.展开更多
文摘This mini review explores the links between anti-neutrophil cytoplasmic antibody-associated vasculitis(AAV)and kidney cancer.Several studies suggest an increased incidence of cancer for patients with AAV.Different cancer types have shown different standardized incidence ratios(SIRs)in association with AAV.The SIRs of kidney cancer were found to be between 1.7 and 3.3 as per three retrospective data analyses.This association is likely multifactorial,with increased de novo cancer risks associated with inflammatory diseases;carcinogenic therapies such as cyclophosphamide;and reduced immune surveillance of neoplastic cells in immunocompromised individuals.Some studies have proposed that cancers,including kidney cancer,could be a potential trigger for AAV.Due to variability in SIRs and a lack of multicenter studies looking specifically into the incidence of kidney cancer at AAV diagnosis and on follow-up post initiation of AAV treatment,there remains a lack of evidence to support formal screening for kidney cancer in the AAV patient cohort.Greater awareness on the increased risk of cancer in AAV patients,prompt urological assessment of“red flag”symptoms of kidney cancer,and smoking cessation advice to reduce cancer risk should be standard of care for patients with AAV.
基金Supported by the Natural Science Foundation of Hunan Province,China,No.2023JJ30842.
文摘BACKGROUND Hepatitis B virus(HBV)infection can lead to renal involvement,commonly manifested as HBV-associated glomerulonephritis(HBV-GN),which typically presents as nephrotic or nephritic syndrome.Antineutrophil cytoplasmic antibody-associated vasculitis(AAV)is a systemic disease characterized by immune necrotizing inflammation of small blood vessels involving multiple organs with complex and severe clinical implications.The coexistence of HBV-GN and AAV is sporadic,with limited data existing regarding its diagnosis,management,clinical outcomes,and prognosis,especially in patients with AAV.CASE SUMMARY This manuscript presents the case of an older male patient who presented with persistent foamy urine lasting over two weeks.Initial clinical findings included nephrotic syndrome and renal insufficiency,which subsequently progressed to involve the lungs,immune system,hematologic system,and other organ systems.The patient was diagnosed with HBV-GN complicated by AAV,a rare and complex condition.Despite receiving comprehensive treatment,including corticosteroids,cyclophosphamide for immune regulation,plasma exchange,and immunoadsorption targeting antineutrophil cytoplasmic antibody-associated antibodies,the patient required long-term dialysis and demonstrated a poor prognosis.CONCLUSION HBV infection may trigger nephropathy with AAV.Early recognition and intervention are crucial for improving patient prognosis.
文摘BACKGROUND This case report describes myeloperoxidase-anti-neutrophil cytoplasmic antibody associated vasculitis with kidney involvement in a patient with relapsing polychondritis,which was successfully treated with Avacopan.Although relapsing polychondritis has been associated with anti-neutrophil cytoplasmic antibody-associated vasculitis,overlap can result in severe organ involvement,particularly renal damage progressing to end-stage kidney disease.This case presents a unique opportunity to evaluate the potential role of Avacopan as an alternative therapeutic option in managing myeloperoxidase-anti-neutrophil cytoplasmic antibody-associated vasculitis in the context of relapsing polychon-dritis highlighting a positive renal response despite treatment challenges.This is a case of a 69-year-old Caucasian woman who presented to our hospital’s emergency department with a 4 week history of inflammatory polychondritis affecting the auricular cartilage,accompanied by acute kidney injury.On admission,serum creatinine was elevated at 4.0 mg/dL,which progressively increased to 6.07 mg/dL on day 6.The renal biopsy revealed necrotizing and crescentic glomerulonephritis affecting more than 50%of the glomeruli.She was treated with a total of 2500 mg intravenous methylprednisolone over 3 days followed by oral prednisone.Induction treatment included intravenous cyclophosphamide induction,with plans for a total of 2 doses followed by transition to rituximab.However the patient was unable to tolerate rituximab due to allergic reaction so intravenous cyclophosphamide was continued for a total of 6 doses(cumulative dose 3000 mg).In the setting of persistent acute kidney injury,Avacopan was added to the regimen 3 months after diagnosis.Maintenance therapy included azathioprine in addition to Avacopan.Prednisone gradually tapered off at 6 months.CONCLUSION Avacopan may be beneficial in treating anti-neutrophil cytoplasmic antibody-associated vasculitis with coexisting relapsing polychondritis,especially in cases where preservation of kidney function is critical.Further research will be essential to validate these findings and refine treatment protocols for such complex cases.
文摘Introduction: Among the chronic and feared complications of this disease, rheumatoid vasculitis stands out as one of the most severe, albeit rare. The most frequently affected sites by rheumatoid vasculitis are the skin and the peripheral nervous system. We report a case of rheumatoid vasculitis complicating a 30-year history of untreated rheumatoid arthritis. Case Report: The patient is a 75-year-old male with a 30-year history of deforming and erosive seropositive rheumatoid arthritis. He presented with polyarthritis and digital necrosis. Physical examination revealed peripheral joint syndrome with characteristic deformities of rheumatoid arthritis. Additionally, there was well-demarcated dry gangrene affecting the first and second digits of the right hand. Laboratory findings indicated an inflammatory syndrome. Tests for antinuclear antibodies (ANA) and anti-neutrophil cytoplasmic antibodies (ANCA) to investigate other causes of vasculitis were negative. The patient received a corticosteroid bolus. After two weeks, there was a marked reduction in pain and an improvement in the general condition. The dry gangrene remained stable. Conclusion: Rheumatoid vasculitis remains a serious and chronic complication of rheumatoid arthritis, associated with significant mortality. This case highlights the crucial importance of early diagnosis and effective management of rheumatoid arthritis.
文摘Rationale:IgA vasculitis or Henoch-Schonlein purpura is an immune complex mediated small vessel vasculitis characterized by clinical triad of arthritis,palpable purpura and gastrointestinal symptoms.It is commonly seen in children below 10-year-old,though adults may be affected.Patient’s Concern:A 40-year-old man with type-2 diabetes mellitus presented with fever,melena,palpable purpuric rash and hematuria with acute kidney injury,associated with nephrotic range proteinuria.Skin biopsy revealed leukocytoclastic vasculitis and renal biopsy showed IgA deposits in the mesangium and capillary loops with fibro cellular crescents confirming the diagnosis of IgA vasculitis with crescentic rapidly progressive glomerulonephritis.Diagnosis:IgA vasculitis with rapidly progressive glomerulonephritis.Interventions:The patient was treated with pulse intravenous methylprednisolone 500 mg/day for 3 days with tapering doses of oral prednisolone and intravenous cyclophosphamide(0.5 gm/m^(2) body surface area)under the cover of intravenous antibiotics for diabetic foot ulcer.Outcomes:Patient improved gradually with return to baseline creatinine after 3 months of follow up.Lessons:Rapidly progressive glomerulonephritis is a rare manifestation of IgA vasculitis and warrants special care and early treatment.The incidence of rapidly progressive glomerulonephritis in IgA vasculitis is unknown.It carries a high risk of progression to chronic kidney disease and thus should be treated as soon as feasible after diagnosis.
文摘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.
基金supported by the Consejo Nacional de Investigaciones Cientıfícas y Técnicas(CONICET)(PIP 2021-2023/11220200102779CO).
文摘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.
文摘The clinical spectrum of immunoglobulin A vasculitis nephritis(IgAVN)ranges from the relatively common transitory microscopic hematuria and/or low-grade proteinuria to nephritic or nephrotic syndrome,rapidly progressive glomerulonephritis,or even renal failure.Clinical and experimental studies have shown a multifactor pathogenesis:Infection triggers,impaired glycosylation of IgA1,complement activation,Toll-like-receptor activation and B cell proliferation.This knowledge can identify IgAVN patients at a greater risk for adverse outcome and increase the evidence for treatment recommendations.
文摘BACKGROUND Single-organ vasculitis(SOV)is characterized by inflammation of a blood vessel,affecting one organ,such as the skin,genitourinary system,or the aorta without systemic features.Gastrointestinal SOV is rare,with hepatic artery involvement reported only in two prior published cases.Herein,we presented a case of isolated hepatic artery vasculitis presenting after Pfizer-BioNTech mRNA corona virus disease 2019(COVID-19)vaccination.CASE SUMMARY A 50-year-old woman with hypertension presented to our Emergency Department with recurrent diffuse abdominal pain that localized to the epigastrium and emesis without diarrhea that began eight days after the second dose of the PfizerBioNTech COVID-19 vaccine.Blood work revealed an elevated C-reactive protein(CRP)of 19 mg/L(normal<4.8 mg/L),alkaline phosphatase 150 U/L(normal 25-105 U/L),gamma-glutamyl transferase(GGT)45 U/L(normal<43 U/L)and elevated immunoglobulins(Ig)G 18.4 g/L(normal 7-16 g/L)and IgA 4.4 g/L(normal 0.7-4 g/L).An abdominal computed tomography revealed findings in keeping with hepatic artery vasculitis.A detailed review of her history and examination did not reveal infectious or systemic autoimmune causes of her presentation.An extensive autoimmune panel was unremarkable.COVID-19polymerase chain reaction nasopharyngeal swab,human immunodeficiency virus,viral hepatitis and Heliobacter pylori serology were negative.At six months,the patient’s symptoms,and blood work spontaneously normalized.CONCLUSION High clinical suspicion of SOV is required for diagnosis in patients with acute abdominal pain and dyspepsia.
文摘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.
文摘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.
文摘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.
基金The Institutional Review Board of National Cheng Kung University Hospital approved this study(No.B-ER-105-108).
文摘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.
基金supported by the National Natural Science Foundation of China(No.81772913).
文摘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.
基金the Special Foundation of Autonomous Region Key Laboratory of China(grant number 2014KL014)and the National Natural Science Foundation of China(grant number 81360051).
文摘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.
文摘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.
文摘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.
文摘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.
文摘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.
基金Supported by Department of Nephrology,University of Debrecen,Clinical Centre,Debrecen,HungaryThe project implemented through the New Hungary Development Planco-financed by the European Union and the European Social Fund,No.TáMOP4.2.2.A-11/1/KONV-2012-0045
文摘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.
