BACKGROUND Anti-glomerular basement membrane(GBM)disease is a rare autoimmune disease manifesting as acute progressive nephritis syndrome with or without varying degrees of pulmonary hemorrhage.Anti-GBM disease coexis...BACKGROUND Anti-glomerular basement membrane(GBM)disease is a rare autoimmune disease manifesting as acute progressive nephritis syndrome with or without varying degrees of pulmonary hemorrhage.Anti-GBM disease coexisting with Immunoglobulin A(IgA)nephropathy is rarer and has different clinical manifestations and prognoses than simple anti-GBM disease.We describe a case of coexistence of these two diseases.CASE SUMMARY A 49-year-old man with hematuria and proteinuria accompanied by a slight elevation of serum creatinine was admitted to our hospital.The pathological results of renal biopsy and the elevated serum anti-GBM antibody titer supported a diagnosis of anti-GBM disease combined with IgA nephropathy.After treatment with corticosteroids and cyclophosphamide,the patient's serum creatinine was relatively stable,and the hematuria and proteinuria moderately improved in the subsequent six months.CONCLUSION Anti-GBM disease coexisting with IgA nephropathy is rare.The clinical manifestations and prognosis are better than those of simple anti-GBM disease.In this case,the patient's condition was improved and his renal function remained relatively stable with corticosteroid and cyclophosphamide treatment.New detection methods to identify whether the crescents in this case were derived from anti-GBM disease or IgA nephropathy are worthy of further exploration.展开更多
A 22-year-old man suffered from acute pulmonary hemorrhage and deteriorated renal function occurred within 3 days after traumatic brain injury.Mechanical ventilation cannot correct his severe hypoxemia,therefore,venoa...A 22-year-old man suffered from acute pulmonary hemorrhage and deteriorated renal function occurred within 3 days after traumatic brain injury.Mechanical ventilation cannot correct his severe hypoxemia,therefore,venoarterial extracorporeal membrane oxygenation(VA-ECMO)support was initiated and finally resolved his hypoxemia.Concomitantly,continuous renal replacement therapy was performed to improve his kidney function.Although no anti-glomerular basement membrane(anti-GBM)antibody was detected in serum,Goodpasture’s syndrome was considered.After treated with methylprednisolone pulse therapy and plasmapheresis,his renal function was significantly improved.ECMO was eventually discontinued after 60 hours of treatment and extubated on day 10.He was discharged home with normal pulmonary and renal functions.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
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
Objective To investigate the characteristics and outcome of glomerulonephritis in patients with both antineutrophil cytoplasmic antibody and anti-glomerular basement membrane antibody.Methods The sera of 23 antiGBM gl...Objective To investigate the characteristics and outcome of glomerulonephritis in patients with both antineutrophil cytoplasmic antibody and anti-glomerular basement membrane antibody.Methods The sera of 23 antiGBM glomerulonephritis patients were collected and were tested for ANCA respectively.Characteristics and outcome of patients with coexisting anti-GBM antibody展开更多
文摘BACKGROUND Anti-glomerular basement membrane(GBM)disease is a rare autoimmune disease manifesting as acute progressive nephritis syndrome with or without varying degrees of pulmonary hemorrhage.Anti-GBM disease coexisting with Immunoglobulin A(IgA)nephropathy is rarer and has different clinical manifestations and prognoses than simple anti-GBM disease.We describe a case of coexistence of these two diseases.CASE SUMMARY A 49-year-old man with hematuria and proteinuria accompanied by a slight elevation of serum creatinine was admitted to our hospital.The pathological results of renal biopsy and the elevated serum anti-GBM antibody titer supported a diagnosis of anti-GBM disease combined with IgA nephropathy.After treatment with corticosteroids and cyclophosphamide,the patient's serum creatinine was relatively stable,and the hematuria and proteinuria moderately improved in the subsequent six months.CONCLUSION Anti-GBM disease coexisting with IgA nephropathy is rare.The clinical manifestations and prognosis are better than those of simple anti-GBM disease.In this case,the patient's condition was improved and his renal function remained relatively stable with corticosteroid and cyclophosphamide treatment.New detection methods to identify whether the crescents in this case were derived from anti-GBM disease or IgA nephropathy are worthy of further exploration.
基金Supported by the Natural Science Foundation of Guangdong Province of China(No.2015A030313735)the Science and Technology Planning Project of Guangdong Province of China(No.2014A020212651)
文摘A 22-year-old man suffered from acute pulmonary hemorrhage and deteriorated renal function occurred within 3 days after traumatic brain injury.Mechanical ventilation cannot correct his severe hypoxemia,therefore,venoarterial extracorporeal membrane oxygenation(VA-ECMO)support was initiated and finally resolved his hypoxemia.Concomitantly,continuous renal replacement therapy was performed to improve his kidney function.Although no anti-glomerular basement membrane(anti-GBM)antibody was detected in serum,Goodpasture’s syndrome was considered.After treated with methylprednisolone pulse therapy and plasmapheresis,his renal function was significantly improved.ECMO was eventually discontinued after 60 hours of treatment and extubated on day 10.He was discharged home with normal pulmonary and renal functions.
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.
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Objective To investigate the characteristics and outcome of glomerulonephritis in patients with both antineutrophil cytoplasmic antibody and anti-glomerular basement membrane antibody.Methods The sera of 23 antiGBM glomerulonephritis patients were collected and were tested for ANCA respectively.Characteristics and outcome of patients with coexisting anti-GBM antibody
