BACKGROUND Anti-glomerular basement membrane(GBM)disease is a rare rapidly progressive glomerulonephritis,frequently associated with alveolar hemorrhage in the lungs and involving the kidney by crescentic glomerulonep...BACKGROUND Anti-glomerular basement membrane(GBM)disease is a rare rapidly progressive glomerulonephritis,frequently associated with alveolar hemorrhage in the lungs and involving the kidney by crescentic glomerulonephritis.It has been described in association with other glomerulonephritides[such as anti-neutrophilic antibody(ANCA)-glomerulonephritis,membranous nephropathy,and immunoglobulin(Ig)A nephropathy].CASE SUMMARY Herein we present an unusual case of concurrent anti-GBM disease,ANCAassociated crescentic glomerulonephritis and diffuse proliferative immune complex mediated glomerulonephritis with predominant staining for IgA and C3 by immunofluorescence.The patient is a 46-year-old Caucasian male who presented to the emergency department with acute onset of flank pain and was found to have high serum creatinine levels of 15 mg/dL,proteinuria,and hematuria.He rapidly deteriorated and became anuric.He was found to have high anti-GBM antibodies titers(151 units)and high anti-neutrophil cytoplasmic-ANCA.Despite prompt and early treatment,the patient’s condition worsened,and he succumbed to his illness.CONCLUSION Our case emphasizes the importance of a renal biopsy in anti-GBM disease,even in the presence of positive serum anti-GBM antibodies,to identify other potential causes of rapidly progressive glomerulonephritis.The challenge in treating such cases lies in the different therapy modalities.展开更多
Rationale:Coexistence of anti-glomerular basement membrane(anti-GBM)disease with anti-neutrophil cytoplasmic antibody(ANCA)in a case of glomerulonephritis is often identified as a"double-positive"disease.Int...Rationale:Coexistence of anti-glomerular basement membrane(anti-GBM)disease with anti-neutrophil cytoplasmic antibody(ANCA)in a case of glomerulonephritis is often identified as a"double-positive"disease.Interestingly,the majority of"double positive"ANCA is myeloperoxidase(MPO)-ANCA and some of the MPO-ANCA positive cases showed intrarenal arteritis,suggesting an ANCA-associated kidney lesion.Proteinase 3-ANCA positive diseases are also rarely reported.Patients positive for all three antibodies,i.e.,triple-positive patients,are extremely rare.Patient's Concern:A 53 year-old female presented with anasarca and oliguria of 2 months'duration.Diagnosis:Pauci-immune type renal limited crescentic glomerulonephritis positive for MPO-ANCA,proteinase 3-ANCA,and anti-GBM antibody(triple-positive).Interventions:Intravenous high dose cyclophosphamide,oral azathioprine,intravenous methylprednisolone,and plasma exchange as per British Health Professionals in Rheumatology Guidelines.Outcomes:After one-month follow-up,anasarca and proteinuria were lessened,serum creatinine was normalized,titers of MPO-ANCA levels were decreased,and anti-GBM antibody levels were normalized.Lessons:Triple-positive renal limited vasculitis is rare and response to combined immunosuppressive therapy and plasma exchange can contribute to successful remission.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
<strong>Aim:</strong> The objectives of this study are to determine the epidemiology of biopsy-proven glomerular disease (GD) in Central Queensland and the effect of a severe cyclone on its incidence and c...<strong>Aim:</strong> The objectives of this study are to determine the epidemiology of biopsy-proven glomerular disease (GD) in Central Queensland and the effect of a severe cyclone on its incidence and clinical phenotype. <strong>Background:</strong> Central Queensland (CQ) has a relatively high incidence of kidney disease. Since its biopsy service commenced in 2005, there have been no data on biopsy-proven GD. It has been suggested that GD incidence changes around times of natural disasters. In February 2015, the CQ region was affected by a category 5 Cyclone Marcia. This provides an opportunity to explore possible environmental triggers of GD. <strong>Methods:</strong> This was a single-centre retrospective observational study on biopsy-proven kidney disease in CQ. All kidney biopsies performed between January 2005 and December 2019 were included. Patients with biopsy-proven GD during 3 years before and after Cyclone Marcia (from 2012 to 2018) were analysed. <strong>Results:</strong> 170 native kidney biopsies occurred during the 15 years. The number of annual biopsies steadily increased from 7 to 16. The most common biopsy-proven kidney disease was IgA Nephropathy (27%) followed by diabetic nephropathy (20%). GD comprised 64% of biopsies. Unlike other GD, the incidence of ANCA-associated vasculitis (AAV) significantly increased after cyclone (one pre- and eight post-cyclone, P value = 0.039). The majority of AAV cases occurred in the first year after the cyclone. <strong>Conclusion:</strong> Kidney biopsies in CQ provide important epidemiological data on biopsy-proven kidney disease. Cyclones have a possible effect on the incidence and clinical phenotype of ANCA associated vasculitis.展开更多
文摘BACKGROUND Anti-glomerular basement membrane(GBM)disease is a rare rapidly progressive glomerulonephritis,frequently associated with alveolar hemorrhage in the lungs and involving the kidney by crescentic glomerulonephritis.It has been described in association with other glomerulonephritides[such as anti-neutrophilic antibody(ANCA)-glomerulonephritis,membranous nephropathy,and immunoglobulin(Ig)A nephropathy].CASE SUMMARY Herein we present an unusual case of concurrent anti-GBM disease,ANCAassociated crescentic glomerulonephritis and diffuse proliferative immune complex mediated glomerulonephritis with predominant staining for IgA and C3 by immunofluorescence.The patient is a 46-year-old Caucasian male who presented to the emergency department with acute onset of flank pain and was found to have high serum creatinine levels of 15 mg/dL,proteinuria,and hematuria.He rapidly deteriorated and became anuric.He was found to have high anti-GBM antibodies titers(151 units)and high anti-neutrophil cytoplasmic-ANCA.Despite prompt and early treatment,the patient’s condition worsened,and he succumbed to his illness.CONCLUSION Our case emphasizes the importance of a renal biopsy in anti-GBM disease,even in the presence of positive serum anti-GBM antibodies,to identify other potential causes of rapidly progressive glomerulonephritis.The challenge in treating such cases lies in the different therapy modalities.
文摘Rationale:Coexistence of anti-glomerular basement membrane(anti-GBM)disease with anti-neutrophil cytoplasmic antibody(ANCA)in a case of glomerulonephritis is often identified as a"double-positive"disease.Interestingly,the majority of"double positive"ANCA is myeloperoxidase(MPO)-ANCA and some of the MPO-ANCA positive cases showed intrarenal arteritis,suggesting an ANCA-associated kidney lesion.Proteinase 3-ANCA positive diseases are also rarely reported.Patients positive for all three antibodies,i.e.,triple-positive patients,are extremely rare.Patient's Concern:A 53 year-old female presented with anasarca and oliguria of 2 months'duration.Diagnosis:Pauci-immune type renal limited crescentic glomerulonephritis positive for MPO-ANCA,proteinase 3-ANCA,and anti-GBM antibody(triple-positive).Interventions:Intravenous high dose cyclophosphamide,oral azathioprine,intravenous methylprednisolone,and plasma exchange as per British Health Professionals in Rheumatology Guidelines.Outcomes:After one-month follow-up,anasarca and proteinuria were lessened,serum creatinine was normalized,titers of MPO-ANCA levels were decreased,and anti-GBM antibody levels were normalized.Lessons:Triple-positive renal limited vasculitis is rare and response to combined immunosuppressive therapy and plasma exchange can contribute to successful remission.
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<strong>Aim:</strong> The objectives of this study are to determine the epidemiology of biopsy-proven glomerular disease (GD) in Central Queensland and the effect of a severe cyclone on its incidence and clinical phenotype. <strong>Background:</strong> Central Queensland (CQ) has a relatively high incidence of kidney disease. Since its biopsy service commenced in 2005, there have been no data on biopsy-proven GD. It has been suggested that GD incidence changes around times of natural disasters. In February 2015, the CQ region was affected by a category 5 Cyclone Marcia. This provides an opportunity to explore possible environmental triggers of GD. <strong>Methods:</strong> This was a single-centre retrospective observational study on biopsy-proven kidney disease in CQ. All kidney biopsies performed between January 2005 and December 2019 were included. Patients with biopsy-proven GD during 3 years before and after Cyclone Marcia (from 2012 to 2018) were analysed. <strong>Results:</strong> 170 native kidney biopsies occurred during the 15 years. The number of annual biopsies steadily increased from 7 to 16. The most common biopsy-proven kidney disease was IgA Nephropathy (27%) followed by diabetic nephropathy (20%). GD comprised 64% of biopsies. Unlike other GD, the incidence of ANCA-associated vasculitis (AAV) significantly increased after cyclone (one pre- and eight post-cyclone, P value = 0.039). The majority of AAV cases occurred in the first year after the cyclone. <strong>Conclusion:</strong> Kidney biopsies in CQ provide important epidemiological data on biopsy-proven kidney disease. Cyclones have a possible effect on the incidence and clinical phenotype of ANCA associated vasculitis.
