Etiology and Outcome of Crescentic Glomerulonephritis

  Crescentic GN remains one of the leading histologic diagnoss associated with acute or rapidly progressive kidney failure.There is a paucity of reports from China and few seriws from other parts of the world.We previously analyzed data from 172 Chinese patients with crescentic GN to characterize the clinical spectrum of crescentic GN during 1989 to 2001;however,the sample was small and clinicopathologic information was limited.This report provodes more extensive and detailed information about the occurrence and outcome of biopsy-diagnosed crescentic GN 33747 nontransplant-related biopsies performed during the past 10 years in the biggest renal center in China.

  Crescents can occur with most forms of primary GN and also are asociated with various systemic diseases.Among 33747 kidney biopsies was IGA nephritis and the mostprevalent secondary glomerular discase was lupus nephritis.Of note,in the present study,we also observed a limited number of instances of crescents associated with unusual disease entities,such as light-chain kidney disease,Alport syndrome,membranous nephropathy,focal segmental glomerulosclerosis,diabetic nephritis,and minimal change disease.These biopsy specimens had various percentage of crescents,usually with <50% of glomeruli having crescents,thus highlighting the need for considering these diseases as a cause of GN with crescents with decreased eGFR and the importance of performing an early kidney biopsy for accurate diagnosis and appropriate therapeutic decisions.

  The spectrum of crescentic GN varied substantially among groups stratified by age and sex.Similar to previous reports,patients with anti-GBM disease(type I crescentic GN) were mainly middle-aged men with worse prognoses,and most had oliguria or hemoptysis and extensive glomerular crescent formation pathologically.Patients with immune complex-mediated crescentic GN(type II) were mainly young or middle-aged women and many had nephrotic syndrome,but nevertheless they showed good response to intensive immunosuppressive therapy.Patients with auci-immune crescentic GN(type III) were mainly middle-aged or elderly with multisystem involvement and had a relatively good response to intensive immunosuppressive therapy.

  Almost half the patients in our cohort had AKI as their clinical presrntation,but it was remarkable that clinical presentation also included AKD in 13.1% and CKD in 28.8%,which were most commonly seen in patients with type II crescentic GN.Therefore,timely kidney biopsy should be emphasized for patients with decreased eGFRs,asymptomatic urinary abnormalities,and nephrotic syndrome without kidney atrophy in a kidney ultrasound to facilitate early diagnosis and proper treatment.

   In pathologic findingd,mean proportions of glomeruli showing crescents were 80.6% in anti-GBM disease,63.1% in immune complex-mediated GN,and 73.2% in pauci-immune GN;crescents involving >80% glomeruli were seen in 55.7%,14.5%,and 39.0% of the 3 types,respectively.Proportions of cellular crescents,and fibrocellular crescents were similar among the 3 groups.


  In conclusion, lupus nephritis may be the most common type of crescentic GN in china.and anti-GBM disease and pauci-immune crescentic GN are not rare in china. Clinical manifestations and outcomes vary by cresentic GN type.the distinction between vary by subtypes based on immunofluorescence and serologic findings has important implicationsfor therapy and outcome. We believe that the treatment of traditional chinese medicine combined with western medicine for crescentic GN ,is  a kind of good method.