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ACR Special Report: Chicago, IL 2011

First Lupus Nephritis Diagnosis and Management Guidelines Released

About 60% of people with lupus will eventually develop lupus-related kidney disease, or lupus nephritis. Yet until now, there was no consensus on the best way to diagnose and manage this common complication. That changed at ACR, when ALR grantee and ALR Scientific Advisory Board (SAB) member Bevra H. Hahn, MD, of the University of California-Los Angeles School of Medicine, presented new guidelines from the American College of Rheumatology. The guidelines, which will be published in an upcoming issue of Arthritis & Rheumatism, were developed by a 24-member committee that Dr. Hahn headed, and vetted by separate task force.

The guidelines recommend that physicians perform kidney biopsies on anyone with clinical signs of the disease; classify the disease stage based on the International Society of Nephrology /Renal Pathology Society’s classification; and treat the disease based on that classification.

Other key recommendations include:

  • All patients should receive hydroxychloroquine, which can reduce the risk of long-term kidney damage
  • Patients may receive azathioprine during the induction phase of therapy, but it should no longer be used as maintenance therapy
  • Patients with a proteinuria of 0.5 g/24 hours or greater, or an equivalent protein/creatinine ratio, should receive an angiotensin converting enzyme inhibitor (ACEi) or angiotensin receptor blocker
  • Patients should maintain a blood pressure of 130/80 mmHg or less
  • Patients with low-density lipoprotein (LDL) cholesterol levels greater than 100 mg/dL should receive a statin
  • Women of reproductive age should be counseled about pregnancy

For induction therapy, the panel recommended either mycophenolate mofetil (MMF) or cyclophosphamide (CYC) in patients with Class III/IV lupus nephritis, but MMF (2-3 g/day for 6 months) for African Americans and Hispanics. All patients should also receive a glucocorticoid IV pulse for 3 days, then prednisone 0.5-1mg/kg per day with eventual tapering to the lowest effective dose.

The guidelines also offer a choice of induction method for CYC: either the low-dose, Euro-Lupus regimen (500 mg IV every 2 weeks for 6 weeks) for those of Caucasian or European background; or the high-dose, National Institutes of Health regimen (500-1000 mg/m2 body surface area monthly for 6 months). Both provide similar improvement according to several studies.

If patients fail the first induction drug, they should be switched to the other. If patients fail both MMF and CYC, the guidelines suggest starting rituximab (Rituxan®) or calcineurin inhibitors. Several open-label trials suggest that rituximab may benefit patients with lupus nephritis.

Once the acute phase of the disease improves, doctors can maintain patients on MMF (1-2 g/day) or azathioprine (2 mg/kg/d ± low dose).

Patients with Class V membranous lupus nephritis should start on MMF (2-3 g daily for 6 months) plus prednisone (0.5 mg/kg/day for 6 months) and, if they improve, receive maintenance therapy with MMF or azathioprine. If they do not improve, they should be started on CYC (500-1000 mg/m2 monthly for 6 months), plus a glucocorticoid pulse followed by daily prednisone (0.5-1.0 mg/kg/day).

The guidelines also address management in pregnant women. Women with a history of Class III or higher disease do not require treatment if there is no evidence of disease activity. Those with mild disease activity should receive hydroxychloroquine (200mg-400mg daily), while those with clinically active disease should receive prednisone at doses required to suppress activity and, if necessary, azathioprine not to exceed 2/mg/kg/day).

“These are only guidelines designed to remind clinicians of what is good clinical practice,” said Dr. Hahn. Clinicians can still treat individual patients outside of the recommendations, she said.

Key point: These guidelines represent the best evidence to date about available treatments for lupus nephritis. If your doctor is not treating your disease according to these recommendations, share them and discuss possible treatment changes.

More information about lupus and treatment advances can be found by visiting www.lupusresearch.org.

The Alliance for Lupus Research Special Report on the 2011 American College of Rheumatology Meeting was made possible in part by generous support from Genentech.

©2011 Alliance for Lupus Research. All Rights Reserved.

Contents herein may not be reproduced, republished or distributed without the prior written permission of the Alliance for Lupus Research. To request permission to reproduce, republish or distribute any part of this report, contact us at 212-218-2840 or email info@lupusresearch.org.

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