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
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...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.展开更多
Introduction: Vasculitis associated with anti-neutrophil cytoplasm antibodies (ANCA) can be grouped with granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MAP), and eosinophilic granulomatosis with pol...Introduction: Vasculitis associated with anti-neutrophil cytoplasm antibodies (ANCA) can be grouped with granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MAP), and eosinophilic granulomatosis with polyangiitis (EGPA). Diagnosis of these rare pathologies is based on clinical presentation, the positivity of ANCA, and, if possible, histological proof of vasculitis. Our study describes a series of six cases of ANCA-associated vasculitis where due to the severity of symptoms apheresis sessions were started from the beginning of the therapy. Patients and methods: We conducted a retrospective, single-center observational, monocentric study on all patients treated by apheresis for ANCA vasculitis in the period January 01, 2016 to December 01, 2019. Results: We identified six cases of ANCA vasculitis treated by apheresis over a 3-year period. The mean age was 61 ± 19 years;M/F gender ratio was 1:1. Initial renal damage in all patients was rapidly progressive glomerulonephritis. Inflammatory syndrome occurred in all patients with average CRP of 82 mg/L. All patients had positive ANCA at diagnosis. Four patients required renal replacement therapy at the time of diagnosis. The induction regimen consisted of rituximab associated with IV boluses of methylprednisolone. The apheresis techniques used were the same for all patients, i.e. plasmapheresis. Outcomes were favorable for five patients;only one patient became dependent on hemodialysis. No mortality occurred. Conclusion: This study analyzed practices for the management of patients with ANCA vasculitis. No patient was treated with cyclophosphamide as a first approach but rituximab instead. Plasmapheresis was given because of symptoms severity at initial diagnosis.展开更多
Objectivs For better understanding the incidence, clinical and pathological features of rapidlyprogressive glomerulonephritis and improving the level of diagnosis and treatent. Methods Records Of 19 biopsyproved RPGN ...Objectivs For better understanding the incidence, clinical and pathological features of rapidlyprogressive glomerulonephritis and improving the level of diagnosis and treatent. Methods Records Of 19 biopsyproved RPGN were reviewed. Results Nephrotic syndrome and acute renal failure were common. The incidenceof this disease is about 2% of all renal biopsies of the same period. Conclusion 1. Renal biopsy should beperformed as soon as possible if RPGN is suspected. 2. The detection of antineutrophil cytoplasmic autoantibody isvery important. 3. The indication of pulse therapy should be strictly justified according to the pathologicalfindings. 4. Dialysis and plasma exchange should be given to ARF patients in the early stage.展开更多
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.
文摘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.
文摘Introduction: Vasculitis associated with anti-neutrophil cytoplasm antibodies (ANCA) can be grouped with granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MAP), and eosinophilic granulomatosis with polyangiitis (EGPA). Diagnosis of these rare pathologies is based on clinical presentation, the positivity of ANCA, and, if possible, histological proof of vasculitis. Our study describes a series of six cases of ANCA-associated vasculitis where due to the severity of symptoms apheresis sessions were started from the beginning of the therapy. Patients and methods: We conducted a retrospective, single-center observational, monocentric study on all patients treated by apheresis for ANCA vasculitis in the period January 01, 2016 to December 01, 2019. Results: We identified six cases of ANCA vasculitis treated by apheresis over a 3-year period. The mean age was 61 ± 19 years;M/F gender ratio was 1:1. Initial renal damage in all patients was rapidly progressive glomerulonephritis. Inflammatory syndrome occurred in all patients with average CRP of 82 mg/L. All patients had positive ANCA at diagnosis. Four patients required renal replacement therapy at the time of diagnosis. The induction regimen consisted of rituximab associated with IV boluses of methylprednisolone. The apheresis techniques used were the same for all patients, i.e. plasmapheresis. Outcomes were favorable for five patients;only one patient became dependent on hemodialysis. No mortality occurred. Conclusion: This study analyzed practices for the management of patients with ANCA vasculitis. No patient was treated with cyclophosphamide as a first approach but rituximab instead. Plasmapheresis was given because of symptoms severity at initial diagnosis.
文摘Objectivs For better understanding the incidence, clinical and pathological features of rapidlyprogressive glomerulonephritis and improving the level of diagnosis and treatent. Methods Records Of 19 biopsyproved RPGN were reviewed. Results Nephrotic syndrome and acute renal failure were common. The incidenceof this disease is about 2% of all renal biopsies of the same period. Conclusion 1. Renal biopsy should beperformed as soon as possible if RPGN is suspected. 2. The detection of antineutrophil cytoplasmic autoantibody isvery important. 3. The indication of pulse therapy should be strictly justified according to the pathologicalfindings. 4. Dialysis and plasma exchange should be given to ARF patients in the early stage.
