IgA Nephropathy Guidelines

Updated: Feb 15, 2018
  • Author: Sohail Abdul Salim, MD, FASN, FACP; Chief Editor: Vecihi Batuman, MD, FASN  more...
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Guidelines

Guidelines Summary

Kidney Disease: Improving Global Outcomes (KDIGO) Guidelines

In its clinical practice guidelines for glomerulonephritis, Kidney Disease: Improving Global Outcomes (KDIGO) recommends the following for patients with IgA nephropathy [26] :

  • All patients should be assessed by biopsy for secondary causes of IgA nephropathy
  • Risk of progressession should be evaluated by measurement of proteinuria, blood pressure, and estimated glomerular filtration rate (eGFR) at the time of diagnosis and during follow-up
  • Pathological findings can be used to assess prognosis

KIDGO recommendations for IgA nephropathy treatment include the following [26] :

  • Long-term therapy with angiotensin-converting enzyme inhibitors (ACEIs) or angiotensin receptor blockers (ARBs) is recommended for patients with proteinuria >1 g/day \ with up-titration of the drug based on blood pressure (grade 1B).
  • ACEI or ARB therapy is suggested for proteinuria of 0.5-1 g/d (grade 2D).
  • Upward titration of the ACEI or ARB as far as tolerated is suggested, to achieve proteinuria of 1 g/d (grade 2C).
  • Blood pressure treatment goals: 1 g/d (not graded)
  • A 6-month course of corticosteroid therapy may be given to patients who have persistent proteinuria ≥1 g/d despite 3–6 months of optimized supportive care (including ACEI or ARB treatment and blood pressure control), and a GFR >50 ml/min/1.73m 2 (grade 2C).
  • Fish oil may be given to patients who have persistent proteinuria ≥1 g/d despite 3–6 months of optimized supportive care (including ACEI or ARB treatment and blood pressure control) (grade 2D).

KIDGO guidelines recommend against using mycophenolate mofetil, antiplatelet agents, or tonsillectomy for the treatment of IgA nephropathy (grade 2C). In addition, the following treatments are recommended against unless the patient has crescentic IgA nephropathy with rapidly deteriorating kidney function [26] :

  • Corticosteroids combined with cyclophosphamide or azathioprine (grade 2D)
  • Immunosuppressive therapy in patients with GFR 2</sup> (grade 2C)

Crescentic IgA nephropathy is defined by KIDGO as IgA nephropathy with crescents in more than 50% of glomeruli in the renal biopsy with rapidly progressive renal deterioration. [26]  

Japan Ministry of Health Labour and Welfare (MHLW) and the Japanese Society of Nephrology (JSN) Guidelines

Clinical guidelines for managment of IgA nephropathy have been developed by the Japan Ministry of Health Labour and Welfare (MHLW) and the Japanese Society of Nephrology (JSN). [9] These guidelines are meant to to address the clinical situation and establish a standard treatment in Japan, and recommend focusing the managent of IgA nephropathy on prevention of renal dysfunction. Treatments to suppress IgA nephropathy progression are based on the following patient factors:

  • Renal function
  • Urinary protein level
  • Age
  • Renal histopathological findings

Treatments to be considered, if necessary. are as follows:

  • Reduce blood pressure
  • Reduce salt intake
  • Normalize lipid and glucose metabolism
  • Reduce body weight
  • Smoking cessation 

In patients with urinary protein level ≥1 g/day and chronic kidney disease (CKD) stage G1-3b, the guidelines give a strong recommendation (grade A) for the use of ACEI or ARB therapy to control progression of renal dysfuntion. In patients with urinary protein level ≥1 g/day and chronic kidney disease (CKD) stage G1-2, the guidelines recommendations include the following:

  • High-dose oral steroid therapy: prednisolone at 0.8–1.0 mg/kg for about 2 months, followed by gradual tapering over about 6 months (grade B)
  • Steroid pulse therapy: methylprednisolone 1 g for 3 days by infusion (or IV) every other month, 3 times plus prednisolone 0.5 mg/kg, every other day, for 6 months (grade B) 

The following treatment options may be considered, but only weak evidence supports these recommendations (grade C1) [9] :

  • ACEI or ARB therapy to reduce proteinuria in patients with urinary protein level of 0.5–1.0 g/day
  • Steroid therapy to reduce proteinuria in patients with urinary protein level of 0.5–1.0 g/day and CKD stage G1-2
  • Tonsillectomy alone or combined with steroid pulse therapy to improve urinary findings and lower the progression of renal dysfunction
  • Off-label use of cyclophosphamide, azathioprine, cyclosporine, mycophenolate mofetil, and mizoribine to improve the renal prognosis
  • Antiplatelet agents: dipyridamole to reduce proteinuria and control the progression of renal dysfunction; dilazep to reduce proteinuria
  • Fish oil to improve the renal prognosis