Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ONGOING

low risk of progression

1st line – observation

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ONGOING
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low risk of progression
1st line – 

observation

Patients with isolated hematuria or proteinuria <0.5 g/day and a normal glomerular filtration rate (GFR) do not require treatment.

medium risk of progression

1st line – supportive therapy

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ONGOING
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medium risk of progression
1st line – 

supportive therapy

Once hypertension (>140/90 mmHg) and/or proteinuria (>0.5 g/day) develops, all patients should receive either an ACE inhibitor or an angiotensin-II receptor antagonist as first-line treatment, unless there is a contraindication (e.g., pregnancy planning).[52][53][54][55] Angiotensin-II receptor antagonists are generally reserved for patients intolerant of ACE inhibitors.[56] Combination therapy with an angiotensin-II receptor antagonist and an ACE inhibitor should avoided.

As part of pregnancy planning, women with IgAN should receive preconception counseling on cessation of renin-angiotensin system inhibitors and the optimization of blood pressure control with alternative agents.[40]

Provide all patients with advice on a low-salt diet, smoking cessation, weight control, and exercise.[40]

The goal of treatment is to reduce urine protein excretion to <1 g/day.[40][57]

Primary options

benazepril: 20-40 mg orally once daily

OR

captopril: 12.5 to 50 mg orally three times daily

OR

enalapril: 5-20 mg orally once daily

OR

lisinopril: 10-40 mg orally once daily

OR

ramipril: 2.5 to 20 mg orally once daily

OR

quinapril: 20-80 mg orally once daily

OR

trandolapril: 2-4 mg orally once daily

Secondary options

losartan: 25-100 mg orally once daily

OR

valsartan: 80-320 mg orally once daily

OR

candesartan cilexetil: 8-32 mg orally once daily

OR

irbesartan: 75-300 mg orally once daily

OR

olmesartan medoxomil: 20-40 mg orally once daily

high risk of progression

1st line – supportive therapy

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ONGOING
|
high risk of progression
1st line – 

supportive therapy

Once hypertension (>140/90 mmHg) and/or proteinuria (>0.5 g/day) develops, all patients should receive either an ACE inhibitor or an angiotensin-II receptor antagonist as first-line treatment, unless there is a contraindication (e.g., pregnancy planning).[52][53][54][55] Angiotensin-II receptor antagonists are generally reserved for patients intolerant of ACE inhibitors.[56] Combination therapy with an angiotensin-II receptor antagonist and an ACE inhibitor should be avoided.

As part of pregnancy planning, women with IgAN should receive preconception counseling on cessation of renin-angiotensin system inhibitors and the optimization of blood pressure control with alternative agents.[40]

Provide all patients with advice on a low-salt diet, smoking cessation, weight control, and exercise.[40]

The goal of treatment is to reduce urine protein excretion to <1 g/day.[40][57]

Primary options

benazepril: 20-40 mg orally once daily

OR

captopril: 12.5 to 50 mg orally three times daily

OR

enalapril: 5-20 mg orally once daily

OR

lisinopril: 10-40 mg orally once daily

OR

ramipril: 2.5 to 20 mg orally once daily

OR

quinapril: 20-80 mg orally once daily

OR

trandolapril: 2-4 mg orally once daily

Secondary options

losartan: 25-100 mg orally once daily

OR

valsartan: 80-320 mg orally once daily

OR

candesartan cilexetil: 8-32 mg orally once daily

OR

irbesartan: 75-300 mg orally once daily

OR

olmesartan medoxomil: 20-40 mg orally once daily

Consider – corticosteroid

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ONGOING
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high risk of progression
Consider – 

corticosteroid

Treatment recommended for SOME patients in selected patient group

In patients with persistent proteinuria >1 g/day despite maximal renin-angiotensin system blockade and good blood pressure control, immunosuppression may be considered.

KDIGO guidelines suggest a 6-month course of corticosteroids in patients with persistent proteinuria >1 g/day after a 3- to 6-month period of optimization of blood pressure control and renin-angiotensin system inhibitor therapy.[40] This is based on several small studies investigating the use of corticosteroids in IgAN.[58][59] Similarly, a systematic review found that the use of corticosteroids was associated with a reduced risk of renal progression, but a higher risk of adverse events.[60][61] [ Cochrane Clinical Answers logo ] It is difficult to draw any firm conclusions from these studies because ACE inhibitors/angiotensin-II receptor antagonists were used in only a minority of patients, or were stopped prior to recruitment into the studies. In addition, blood pressure control was suboptimal. 

Although primary composite outcomes in the TESTING trial (end-stage kidney disease, death due to kidney failure, or a 40% decrease in eGFR) were lower in the oral methylprednisolone group, no conclusions can be made about the potential kidney benefits as they must be weighed against the serious adverse events observed and the early termination of the study for patient safety reasons.[62]

Avoid or use corticosteroids with great caution in patients with: eGFR <30 ml/min/1.73 m², diabetes, obesity (BMI >30 kg/m²), latent infections (e.g., viral hepatitis, tuberculosis), secondary disease (e.g., cirrhosis), active peptic ulceration, uncontrolled psychiatric illness, and severe osteoporosis.[40]

Primary options

prednisone: 1 mg/kg/day orally for 6-8 weeks, then reduce dose gradually according to response to complete 6-month treatment course

OR

methylprednisolone: 500-1000 mg/day intravenously for 3 consecutive days at the beginning of the first, third, and fifth month

and

prednisone: 0.5 mg/kg/day orally given on alternate days, reduce slowly according to response over 6 months

acute kidney injury

1st line – avoidance of nephrotoxins and close fluid management

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ONGOING
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acute kidney injury
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with severe visible hematuria
1st line – 

avoidance of nephrotoxins and close fluid management

Acute kidney injury may occur during episodes of visible hematuria due to tubular obstruction with erythrocytes and acute tubular necrosis.

Recovery is usual with simple supportive measures including avoidance of nephrotoxins and close fluid management and no specific therapies are required. If there are no signs of recovery within 48 hours of presentation, a kidney biopsy is required.

1st line – combined immunosuppressive therapy

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ONGOING
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acute kidney injury
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with rapidly progressive glomerulonephritis
1st line – 

combined immunosuppressive therapy

Rarely, IgAN can either present as a rapidly progressive glomerulonephritis (RPGN), or RPGN can develop on a background of established IgAN. Kidney biopsy in this setting usually shows focal necrotizing glomerulonephritis and crescent formation should be treated in the same way as an anti-neutrophil cytoplasmic antibody (ANCA)-associated RPGN. Patients are typically treated with cyclophosphamide and corticosteroids for an initial six months, followed by azathioprine.

Consider combined immunosuppressive therapy in patients presenting with an RPGN characterized by rapid progressive deterioration in kidney function (e.g., rising creatinine, hematoproteinuria); although there are few data, some studies show this improves kidney survival.[57][53][73][74]

Primary options

prednisone: 40 mg orally once daily initially, then reduce dose gradually to 10 mg/day according to response over 2 years

and

cyclophosphamide: 2 mg/kg/day orally for 6 months

and

azathioprine: 2 mg/kg/day orally starting after 6-month course of cyclophosphamide, continue for a minimum of 2 years

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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