What is new in the management of rapidly progressive glomerulonephritis? - PubMed (original) (raw)

What is new in the management of rapidly progressive glomerulonephritis?

George H B Greenhall et al. Clin Kidney J. 2015 Apr.

Abstract

Rapidly progressive glomerulonephritis (RPGN) results from severe crescentic damage to glomeruli and leads to irreversible kidney failure if not diagnosed and managed in a timely fashion. Traditional treatment has relied on glucocorticoids and cyclophosphamide, with additional plasmapheresis for certain conditions. Here we describe updates in the management of RPGN, according to the underlying renal pathology. However, there remains a paucity of trials that have enrolled patients with more advanced renal disease, dialysis dependence or with RPGN, and we are therefore still reliant on extrapolation of data from studies of patients with a less severe form of disease. In addition, reporting bias results in publication of cases or cohorts showing benefit for newer agents in advanced disease or RPGN, but it remains unclear how many unsuccessful outcomes in these circumstances take place. Since clinical trials specifically in RPGN are unlikely, use of biologic registries or combination of sufficient sized cohort series may provide indications of benefit outside of a clinical trial setting and should be encouraged, in order to provide some evidence for the efficacy of therapeutic regimens in RPGN and advanced renal disease.

Keywords: AKI; IgA nephropathy; glomerulonephritis; systemic lupus erythematosus; vasculitis.

PubMed Disclaimer

Figures

Fig. 1.

Fig. 1.

Glomerulus showing crescentic glomerulonephritis due to pauci-immune ANCA-associated vasculitis, with a segmental area of thrombosis, tuft disruption and cells in Bowman's space (Haematoxylin and eosin ×400). Courtesy of Professor Alexander Howie.

Similar articles

Cited by

References

    1. Stone JH, Merkel PA, Spiera R, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med 2010; 363: 221–232 - PMC - PubMed
    1. Jones RB, Tervaert JWC, Hauser T, et al. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. N Engl J Med 2010; 363: 211–220 - PubMed
    1. Geetha D, Specks U, Stone JH, et al. (November 7, 2014) Rituximab versus cyclophosphamide for ANCA-associated vasculitis with renal involvement. J Am Soc Nephrol, doi:10.1681/ASN.2014010046 - DOI - PMC - PubMed
    1. Hu W, Liu C, Xie H, et al. Mycophenolate mofetil versus cyclophosphamide for inducing remission of ANCA vasculitis with moderate renal involvement. Nephrol Dial Transplant 2008; 23: 1307–1312 - PubMed
    1. Silva F, Specks U, Kalra S, et al. Mycophenolate mofetil for induction and maintenance of remission in microscopic polyangiitis with mild to moderate renal involvement--a prospective, open-label pilot trial. Clin J Am Soc Nephrol 2010; 5: 445–453 - PMC - PubMed

LinkOut - more resources