Lupus Nephritis Guidelines

Updated: Mar 04, 2019
  • Author: Lawrence H Brent, MD; Chief Editor: Vecihi Batuman, MD, FASN  more...
  • Print

Guidelines Summary

Guidelines for managing lupus nephritis have been issued by the American College of Rheumatology. [75]  Key points of the guidelines are as follows:

  • Patients with clinical evidence of active, previously untreated lupus nephritis should have a renal biopsy to classify the disease according to International Society of Nephrology/Renal Pathology Society criteria

  • All patients with lupus nephritis should receive background therapy with hydroxychloroquine, unless contraindicated; this recommendation was based on a prospective controlled trial showing lower flare rates in those who continued hydroxychloroquine, compared with those who switched to placebo [76]

  • Glucocorticoids plus either cyclophosphamide intravenously (IV) or mycophenolate mofetil orally for induction in patients with ISN class III/IV disease; patients with ISN/RPS class I and II nephritis do not require immunosuppressive therapy

  • Administer ACEIs or ARBs if proteinuria is 0.5 g/24 h or more

  • Maintain blood pressure at or below 130/80 mm Hg

Joint guidelines for the management of adult and pediatric lupus nephritis have also been issued by European League Against Rheumatism and European Renal Association-European Dialysis and Transplant Association (EULAR/ERA-EDTA). The EULAR/ERA-EDTA recommendations include the following [77] :

  • Any sign of renal involvement can be an indication for biopsy, which should be performed within the first month after disease onset, preferably before the institution of immunosuppressive treatment
  • Use mycophenolate mofetil or or low-dose IV cyclophosphamide plus glucocorticoids as the initial treatment for patients with ISN class III–IV A disease
  • In patients with adverse clinical or histological features, cyclophosphamide can be prescribed at higher doses, while azathioprine is an alternative for milder cases
  • For pure class V lupus nephritis LN with nephrotic-range proteinuria, use mycophenolate mofetil in combination with oral glucocorticoids for initial immunosuppressive therapy
  • Patients who improve after initial treatment should receive mycophenolate mofetil or azathioprine for at least 3 years; patients who start on mycophenolate mofetil should continue on that agent
  • Patients in whom mycophenolate mofetil or cyclophosphamide therapy fails should be switched to the other agent or to rituximab

A European initiative, the Single Hub and Access point for pediatric Rheumatology in Europe (SHARE) project, has published guidelines on the diagnosis and treatment of childhood-onset lupus nephritis. [78, 79]

Diagnostic recommendations from SHARE include the following [79] :

  • Although pediatric patients with lupus nephritis may present with renal dysfunction, hypertension, and macro- or microscopic hematuria and proteinuria, percutaneous renal biopsy is required because symptoms alone do not reflect disease severity
  • For patients with proteinuria and/or a low glomerular filtration rate, consult with a pediatric nephrologist to discuss the need for biopsy
  • Consult with an experienced renal pathologist to evaluate renal biopsies
  • Exclude orthostatic proteinuria, which is the leading cause of proteinuria in teenagers, by collecting a first-morning urine sample; in girls, avoid collecting samples during menstruation

SHARE recommends complete renal response as the treatment goal in lupus nephritis, with an early-morning urinary protein/creatinine ratio of less than 50 mg/mmol and normal renal function. Patients should achieve a partial response within 6 to 12 months after starting treatment. [79]  Treatment recommendations include the following:

  • Immunosuppressive treatment should be guided by renal biopsy results; when biopsy is not possible, patients with nephrotic syndrome, hypertension, and impaired renal function should be treated as for lupus nephritis class IV.
  • In patients with proteinuria, consider adding angiotensin-converting enzyme inhibitors or angiotensin receptor blockers.

Recommendations for management based on the International Society of Nephrology/Renal Pathology Society 2003 classification system include the following [79] :

  • Class I: Treatment should be guided by symptoms
  • Class II: Low-dose prednisone, followed by a disease-modifying antirheumatic drug in patients who have 3 months of persistent proteinuria or develop prednisone dependency.
  • Class III/IV: Induction with mycophenolate mofetil (MMF) or intravenous cyclophosphamide plus steroids; maintenance treatment for at least 3 years with MMF, azathioprine, or both
  • Class V: MMF with low-dose prednisone for induction; maintenance treatment with MMF