A review of the AURORA and BLISS trials: will it revolutionize the treatment of lupus nephritis?

PURPOSE OF REVIEW: Renal involvement in patients with systemic lupus erythematosus can lead to significant complications including end-stage renal disease. Treatment of lupus nephritis has evolved over the last several decades, but despite this evolution, many patients fail to achieve remission and often progress to end-stage kidney disease or carry a burden of adverse side effects related to treatment. RECENT FINDINGS: The recent findings from AURORA 1 and BLISS LN trials led the FDA to approve voclosporin and belimumab for the treatment of lupus nephritis. The AURORA 1 trial demonstrated that voclosporin, a second-generation calcineurin inhibitor, effectively lowers proteinuria in patients with lupus nephritis, when added to mycophenolate mofetil with a better safety profile, compared with other calcineurin inhibitors. The BLISS LN trial revealed better control of disease and lower risk of progression to end stage kidney disease (ESKD) and relapses in patients treated with belimumab in addition to standard therapy. SUMMARY: Both voclosporin and belimumab are costly and have not shown any early evidence to revolutionize practice in the management of lupus nephritis. Until more data are made available with future studies or other cost-effective treatment options become available, the widespread adoption and utility of these novel agents remains limited.

Cyclophosphamide for the Treatment of Proliferative Glomerulonephritis with Monoclonal Immunoglobulin Deposition.

About 3% of the population aged more than 50 years, is affected by monoclonal gammopathy of undetermined significance (MGUS), a premalignant condition that may progress to lymphoproliferative disorders. Since MGUS does not represent the diseases associated with end organ damage, a new term, monoclonal gammopathy of renal significance (MGRS) is coined for the monoclonal gammopathies that are associated with renal disorders. MGRS is classified into various types, including monoclonal immunoglobulin deposition disease (MIDD) and proliferative glomerulonephritis with monoclonal immunoglobulin deposition (PGNMID). PGNMID presents with membranoproliferative glomerulonephritis-type lesions associated with immunoglobulin deposition. This disease entity has a poor prognosis and its optimum treatment is yet to be established. We present the case of an elderly male, a known patient of light chain deposition disease, a form of MIDD, who initially lost to follow-up but later presented with PGNMID, wherein he was treated with steroid and cyclophosphamide, to which he responded.