Summary of recommendation statements and practice points

Document Overview

Design and created by Guideline Central in participation with the Kidney Disease Improving Global Outcomes.

Management of Immunoglobulin A Nephropathy (IgAN) and Immunoglobulin A Vasculitis (IgAV)

Kidney Disease Improving Global Outcomes

Publication Date: Sep 18, 2025

Page Last Updated: May 6, 2026

1.4 Treatment

1.4.1 Defining patients with IgAN at risk of progressive loss of kidney function requiring treatment
Practice Point 1.4.1.1: Because patients with IgAN are at risk of progressive loss of kidney function if they have proteinuria ≥0.5 g/d (or equivalent) while on or off treatment of IgAN, treatment or additional treatment should be considered in all such cases.

1.4.2 Defining a treatment goal in patients with IgAN at risk of progressive loss of kidney function

Practice Point 1.4.2.1: The treatment goal in patients with IgAN at risk of progressive loss of kidney function is to reduce the rate of loss of kidney function to the physiological state (i.e., <1 ml/min/yr for most adults) for the rest of the patient’s life. The only validated early biomarker to help guide clinical decision-making is urine protein excretion, which should be maintained at a minimum of <0.5 g/d (or equivalent), and ideally at <0.3 g/d (or equivalent), accepting that in some patients with extensive kidney scarring, this may not be possible and that multiple treatment strategies, including nonpharmacologic interventions, may be needed to achieve this.

Practice Point 1.4.2.2: Treatment of patients with IgAN who are at risk of progressive loss of kidney function and do not have a variant form (Section 1.5) of primary IgAN (Figure 3):

The focus of management in most patients should be to simultaneously:
- Prevent or reduce immunoglobulin A–containing immune complex (IgA-IC) formation and IgA-IC–mediated glomerular injury (whether this requires lifelong or intermittent therapy is currently unknown)
Manage the consequences of existing IgAN-induced nephron loss (likely lifelong)
Reduction or prevention of IgA-IC formation should incorporate treatments that have been proven to reduce pathogenic forms of IgA (commonly measured as galactose-deficient IgA1 [gd-IgA1]).
Prevention of IgA-IC–mediated injury should incorporate treatments with proven anti-inflammatory and/or antifibrotic effects and ideally should be used in combination with, and not as a replacement for, treatments that prevent or reduce IgA-IC formation.
Management of the consequences of IgAN-induced nephron loss should include:
- Lifestyle advice, including information on dietary sodium restriction (<2 g/d), smoking and vaping cessation, weight control, and endurance exercise, as appropriate
- Control of blood pressure with a target of ≤120/70 mm Hg
- Measures to reduce glomerular hyperfiltration and the impact of proteinuria on the tubulointerstitium, using renin-angiotensin system (RAS) blockade or dual endothelin angiotensin receptor antagonism, singly or in combination with a sodium-glucose cotransporter-2 inhibitor (SGLT2i)
- A thorough cardiovascular risk assessment and commencement of appropriate interventions, as per local guidelines and as necessary
Enrollment in a clinical trial should always be considered for all patients with IgAN. With the increase in the number of newly approved treatments, clinical trial design will need to evolve from the current 2-year, placebo-controlled approach to remain relevant to the changing standard of care for treating IgAN.
Issues related to accessibility and affordability of newly approved treatments for IgAN, alongside the requirement for continual or cyclical dosing, mean that it is unlikely that newly approved treatments will be used in resource-limited settings, where cheaper and more easily resourced drugs will be used.
The International IgAN Prediction Tools have not been evaluated as a means of determining the likely impact of any particular treatment regimen and at present should not be used to decide on a specific treatment therapy.
Kidney biopsy features are often used in clinical practice to help inform treatment decisions in IgAN. However, biopsies offer only a snapshot in time of a relatively small sample of tissue, and there are no data from clinical studies that show patients prospectively randomized to particular treatment regimens based on their Oxford Classification MEST-C scores have better clinical outcomes. In particular:
- There is insufficient evidence to base treatment decisions solely on the presence and number of cellular/fibrocellular crescents in the kidney biopsy. Histopathologic features must be interpreted in the context of clinical features, in particular the rate of change in eGFR.
- While it would seem logical that very proliferative or inflammatory lesions may be more amenable to treatment with agents targeting inflammation than lesions with sclerotic or fibrotic changes, this has not been proven in a prospective clinical trial.
Due to the lack of proximate kidney biopsies in all phase 3 clinical trials in IgAN, with many patients undergoing random assignment many years after their biopsy, it is not possible to determine whether any of the new treatments for IgAN should be preferentially selected based on the Oxford Classification MEST-C score or histology in general.
Dynamic assessment of patient risk over time should be performed, as decisions regarding the relative merits of different treatments may change.

1.4.3 Managing the IgAN-specific drivers of nephron loss
Practice Point 1.4.3.1: Factors to consider before using Nefecon in patients with IgAN:

A 9-month treatment course of Nefecon, a targeted-release formulation of budesonide, may not result in a sustained clinical response in terms of proteinuria reduction or eGFR stabilization.
Data on the safety and efficacy of additional courses of Nefecon are awaited.
Nefecon’s approval status, labeled indication, and availability vary globally.
Practice Point 1.4.3.2: Reduced-dose systemic glucocorticoid regimen:
Methylprednisolone (or equivalent) 0.4 mg/kg/d (maximum 32 mg/d) for 2 months followed by dose tapering by 4 mg/d each month for a total of 6–9 months.
The conversion of methylprednisolone to commonly used forms of systemic glucocorticoids is as follows: 1 mg of methylprednisolone equals 1.25 mg of prednisone or prednisolone.
Treatment with systemic glucocorticoids should incorporate antimicrobial prophylaxis against Pneumocystis jirovecii and antiviral prophylaxis in hepatitis B carriers, along with gastroprotection and bone protection according to national guidelines.

Practice Point 1.4.3.3: Factors that increase the risk of toxicity of systemic glucocorticoids:

eGFR <30 ml/min per 1.73 m2
Diabetes and prediabetes
Obesity
Latent infections (e.g., viral hepatitis and tuberculosis)
Active peptic ulceration
Uncontrolled psychiatric illness
Osteoporosis
Cataracts

Practice Point 1.4.3.4: Other pharmacologic therapies evaluated in IgAN:

Multiple agents have been evaluated, often in small studies in restricted populations, and they failed to show a consistent benefit in IgAN (Figure 4).

Practice Point 1.4.3.5: Tonsillectomy in IgAN:

Tonsillectomy alone or with pulsed glucocorticoids may extend kidney survival and increase the likelihoods of partial or complete remission of hematuria and proteinuria based on multiple, mostly retrospective studies from Japan (Supplementary Table S550–54).40,50–52,54,55
Tonsillectomy alone or with pulsed glucocorticoids is recommended in the Japanese Society of Nephrology guidelines for the treatment of patients with IgAN.
Tonsillectomy should not be performed as a treatment of IgAN in non-Japanese patients.

1.4.4 Managing the responses to IgAN-induced nephron loss
Practice Point 1.4.4.1: For lifestyle and blood pressure targets for all patients with IgAN, please refer to Practice Point 1.4.2.2.

Practice Point 1.4.4.2: Factors to consider before using an ACEi or ARB:

All patients with IgAN should receive an ACEi or ARB at the maximally tolerated dose, except patients with contraindications such as low blood pressure, bilateral renal artery stenosis, or hyperkalemia, especially due to advanced CKD.
As ACEi or ARB do not mitigate the IgAN-specific drivers of nephron loss, their use should not preclude the concomitant introduction of therapies that target the drivers of IgAN or glomerular inflammation as stated in Section 1.4.3 for patients who will likely benefit from them.

Practice Point 1.4.4.3: Factors to consider before using sparsentan in patients with IgAN:

Sparsentan is a dual endothelin angiotensin receptor antagonist (DEARA) and should not be prescribed together with a renin-angiotensin system inhibitor (RASi), because sparsentan already combines RASi with an endothelin antagonist in a single molecule.
Sparsentan’s approval status, labeled indication, and availability vary globally.

Practice Point 1.4.4.4: Factors to consider before using an SGLT2i in patients with IgAN:

There was no requirement for patients with IgAN to be on an optimized maximally tolerated dose of RASi for a minimum of 3 months for inclusion in the Study of Heart and Kidney Protection With Empagliflozin (EMPA-KIDNEY) or the Dapagliflozin and Prevention of Adverse Outcomes in Chronic Kidney Disease (DAPA-CKD) trial.
Patients with IgAN included in EMPA-KIDNEY and DAPA-CKD likely had long-standing disease, based on their age and eGFR at randomization; therefore, there is uncertainty over the value of SGLT2i, especially in younger patients with IgAN and relatively preserved kidney function (eGFR >60 ml/min per 1.73 m2) (see Table 2).

1.5 Special situations

Practice Point 1.5.1: IgAN with nephrotic syndrome:

Rarely, patients with IgAN present with nephrotic syndrome (including edema and both hypoalbuminemia and nephrotic-range proteinuria >3.5 g/d).
In these cases, mesangial IgA deposition can be associated with light and electron microscopy features otherwise consistent with a podocytopathy resembling minimal change disease (MCD).
It is unclear whether this is a specific podocytopathic variant of IgAN or the existence of MCD in a patient with IgAN.
Patients with a kidney biopsy demonstrating mesangial IgA deposition and light and electron microscopy features otherwise consistent with MCD should be treated in accordance with the guidelines for MCD in Chapter 5 of the KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases.15
Patients with nephrotic syndrome whose kidney biopsy has coexistent features of mesangioproliferative glomerulonephritis should be managed in the same way as those who are at risk of progressive loss of kidney function due to IgAN.
Nephrotic-range proteinuria without nephrotic syndrome may also be seen in IgAN, and this commonly reflects coexistent secondary focal segmental glomerulosclerosis (e.g., in obesity and uncontrolled hypertension) or development of extensive glomerulosclerosis and tubulointerstitial fibrosis.

Practice Point 1.5.2: IgAN with acute kidney injury (AKI):

AKI can occur in patients with IgAN in the context of severe visible hematuria, commonly in association with an upper respiratory tract infection. A repeat kidney biopsy should be considered in patients who fail to show improvement in kidney function within 2 weeks of cessation of the hematuria. Immediate management of AKI with visible hematuria should focus on supportive care for AKI.
IgAN may also present with AKI either de novo or during its natural history due to rapidly progressive glomerulonephritis (RPGN), often with extensive crescent formation, commonly in the absence of visible hematuria. When other causes of RPGN (e.g., antineutrophil cytoplasmic antibody [ANCA] –associated vasculitis [AAV] and anti–glomerular basement membrane [GBM] disease) and reversible causes (e.g., drug toxicity and common pre- and post-kidney causes) have been excluded, a kidney biopsy should be performed as soon as possible.

Practice Point 1.5.3: IgAN with RPGN:

Rapidly progressive IgAN is defined as a ≥50% decline in eGFR over ≤3 months, where other causes of RPGN (e.g., AAV and anti-GBM disease) and reversible causes (e.g., drug toxicity and common pre- and post-kidney causes) have been excluded.
A kidney biopsy is essential in these cases and will commonly demonstrate mesangial and endocapillary hypercellularity as well as a high proportion of glomeruli affected by crescents with areas of focal necrosis.
The presence of crescents in a kidney biopsy in the absence of a concomitant change in serum creatinine does not constitute rapidly progressive IgAN; however, these patients require close follow-up to ensure prompt detection of any glomerular filtration rate (GFR) decline. If this occurs, a second kidney biopsy may be considered.
Patients with rapidly progressive IgAN should be offered treatment with cyclophosphamide and systemic glucocorticoids in accordance with the KDIGO 2024 Clinical Practice Guideline for the Management of Antineutrophil Cytoplasmic Antibody (ANCA)–Associated Vasculitis.87
Prophylactic measures that should accompany immunosuppression are discussed in Chapter 1 of the KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases.15
There is insufficient evidence to support the use of rituximab for the treatment of rapidly progressive IgAN.

Practice Point 1.5.4: IgAN and pregnancy planning:

IgAN is a disease predominantly of young adults, and all women of childbearing potential should be offered preconception counseling when appropriate.
Preconception counseling should include a discussion on cessation of RASi, SGLT2i, sparsentan, Nefecon, and systemic glucocorticoids. Blood pressure control should be optimized with alternative antihypertensive medications prior to conception.
In women at risk of progressive loss of kidney function, a trial of treatments to optimally address the immunopathogenesis of IgAN prior to conception may be preferable and should be discussed with patients.
Neither SGLT2i nor sparsentan should be used while breastfeeding. Enalapril can be used if a RASi is clinically indicated.
The evidence to date suggests that systemic glucocorticoid use in the first trimester may confer a small increase in the odds of a cleft lip with or without a cleft palate, although data are conflicting, and it is unknown to what extent the underlying maternal disease may contribute. Systemic glucocorticoid use in pregnancy does not increase the risk of preterm birth, low birth weight, or preeclampsia.
The use of Nefecon in pregnancy is not advised; however, studies examining the use of budesonide by pregnant women with inflammatory bowel disease have not identified any harmful effects. Budesonide has a Food and Drug Administration Pregnancy Category C risk designation, so risk cannot be ruled out.

Practice Point 1.5.5: IgAN in children:

General considerations for children with IgAN

A more extensive review of the management of IgAN in children can be found in the 2025 International Pediatric Nephrology Association clinical practice recommendations for the diagnosis and management of children with IgA nephropathy and IgA vasculitis nephritis.1
In this guideline, we define children as those aged <18 years, but it is acknowledged that postpubertal children may in some respects have a similar course and response to treatment as adults with IgAN. However, there are insufficient data currently to recommend that they be managed as adults with IgAN.
Visible hematuria is more frequent in children than in adults, and this may account for earlier diagnosis in children.42
Children generally have higher eGFR, lower urine protein excretion, and more hematuria than adults at diagnosis.41

Kidney biopsy in children with IgAN

A kidney biopsy is usually performed at presentation of symptoms (hematuria, proteinuria, and normal C3) to confirm the diagnosis (and rule out other diagnoses) and assess the degree of inflammation/presence of necrosis.
In particular, a kidney biopsy should be performed promptly in children with persistent (>2–3 weeks) or recurrent hematuria and nephrotic-range proteinuria and/or reduced eGFR.88
A kidney biopsy should also be performed in children with persistent or recurrent hematuria and protein-to-creatinine ratio (PCR) >500 mg/g (50 mg/mmol) in ≥2 measurements 1–2 weeks apart.
In children with persistent or recurrent hematuria and PCR 200–500 mg/g (20–50 mg/mmol) in ≥3 measurements on clear urine 1–2 weeks apart, a kidney biopsy should be considered.
Inflammation, mesangial cell proliferation, and endocapillary hypercellularity tend to be more prevalent in kidney biopsies of IgAN in children than in those of adults.89–92

Treatment of children with IgAN

There is strong evidence suggesting a benefit of RAS blockade in children.64 All children with IgAN and proteinuria >200 mg/d or PCR >200 mg/g (>20 mg/mmol) should receive RAS blockade, advice on moderating dietary salt intake below 3–5 g/d, and optimal lifestyle and blood pressure control (systolic blood pressure [SBP] <90th percentile for age, sex, and height).
It is widely acknowledged that treatment of IgAN with immunosuppression differs between adults and children and that in children, the use of immunosuppressants is more widespread, particularly the use of systemic glucocorticoids. However, randomized controlled trials and expert consensus–driven indications are lacking.
Evidence derived mostly from retrospective studies suggests that treatment with systemic glucocorticoids (plus second-line immunosuppression) leads to improved kidney survival.42,89,91–97
The risk-benefit balance of glucocorticoid side effects must be considered. Systemic oral glucocorticoids are used in selected settings in children with clinical risk of progression, as evidenced by one of the following: (i) PCR 500–1000 mg/g (50–100 mg/mmol) despite 3–6 months of RASi, (ii) PCR >1000 mg/g (>100 mg/mmol) despite 4 weeks of RASi, or (iii) active MEST-C scores (≥1 of the following scores: M1, E1, S1 with podocyte lesions, and/or C1) and/or PCR consistently (i.e., persisting over 2–3 weeks in ≥2 measurements 1–2 weeks apart) >1000 mg/g (100 mg/mmol) in addition to RAS blockade.
- Duration of treatment is not established, but usually 2 mg/kg/d (maximum 60 mg/m2/d) of oral prednisone/prednisolone (or equivalent) for a maximum of 4 weeks followed by alternate-day dosing tapered over 5–6 months is given.
- Further extension of the duration may be useful in some cases. Lower doses, such as those emerging from the adult Therapeutic Effects of Steroids in IgA Nephropathy Global (TESTING) trial (0.4 mg/kg/d of prednisone/prednisolone [or equivalent] for 2 months, tapering over 6 months) should be considered.
Regimens including intravenous methylprednisolone are also used on an individual basis in patients with higher clinical and histologic risk of progression, such as in (i) children with acute onset of IgAN and worsening of kidney function (eGFR <90 ml/min per 1.73 m2) and/or PCR >1000 mg/g (100 mg/mmol) with active severe MEST-C scores (≥2 of the following scores: M1, E1, S1 with podocyte lesions, and/or C1) or (ii) children with crescentic forms of IgAN (C2).
- In cases with C1 or C2 in the absence of any other MEST-C score >0, the level of proteinuria must be considered.41,89,91,98
- In cases with C2, irrespective of proteinuria, treatment of rapidly progressive IgAN is suggested (see below). Dosing regimens may be as follows: 3 methylprednisolone intravenous pulses given at the dose of 15 mg/kg/d each (maximum dose 500 mg) on 3 consecutive or alternate days followed by oral prednisone/prednisolone as indicated above.
  - Alternatively, the intravenous pulses can be repeated 3 times at 2-month intervals, with oral prednisone/prednisolone given at 0.5 mg/kg/d for 2 months between pulse cycles for a total of 6 months.99,100
Children with IgAN not benefiting from an adequate diet, RAS blockade, and glucocorticoids alone should, whenever possible, be enrolled in clinical trials. Another potential approach in these children is the use of immunosuppressants (e.g., calcineurin inhibitors, cyclophosphamide, mizoribine where available, mycophenolate mofetil, or rituximab) in addition to glucocorticoids.
As for adults, IgAN with MCD may be found, and it should be treated as steroid-sensitive nephrotic syndrome (KDIGO 2025 Clinical Practice Guideline for the Management of Nephrotic Syndrome in Children).100a
As in adults, children with rapidly progressive IgAN have a poor outcome, and despite limited evidence, this subgroup should be offered treatment with systemic glucocorticoids (usually as methylprednisolone pulses) and cyclophosphamide.89,91,101

Follow-up of children with IgAN

Aim for proteinuria ≤200 mg/d (≤400 mg/1.73 m2 per day) or PCR ≤200 mg/g (≤20 mg/mmol).
Aim for SBP at <90th percentile for age, sex, and height.
Continue to follow patients after complete remission, as they can relapse even after many years.102 In particular, yearly monitoring of blood pressure and urinalysis for patients with a history of pediatric IgAN is necessary.

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1.9 Treatment

1.9.1 Prevention of nephritis in IgAV
Practice Point 1.9.1.1: Considerations for the management of all patients with IgAVN who are at risk of progressive loss of kidney function and do not have RPGN:

Proteinuria ≥0.5 g/d (while on or off treatment of IgAVN) identifies a patient with IgAVN at increased risk of progressive loss of kidney function.
The goal for managing IgAVN, like IgAN, should be to simultaneously:
- Prevent or reduce IgA-IC formation, mesangial deposition, and IgA-IC–mediated glomerular injury
- Manage the consequences of existing IgAVN-induced nephron loss
Unlike IgAN, there are no treatments proven to prevent or reduce IgA-IC formation in IgAVN.
Prevention of IgA-IC–mediated injury should incorporate treatments with proven anti-inflammatory effects and ideally should be used in combination with, and not as a replacement for, treatments that prevent or reduce IgA-IC formation.
- In all patients in whom systemic glucocorticoids are being considered, a detailed discussion of the risks and benefits of each drug should be undertaken with the patient.
- In patients who wish to try systemic glucocorticoids, a reduced-dose regimen as described for IgAN should be employed with antimicrobial prophylaxis.
Management of the consequences of IgAVN-induced nephron loss should include:
- Lifestyle advice, including information on dietary sodium restriction (<2 g/d), smoking and vaping cessation, weight control, and endurance exercise, as appropriate
- Control blood pressure with a target of ≤120/70 mm Hg using a RASi as the first-choice drug intervention
- Measures to reduce glomerular hyperfiltration and the impact of proteinuria on the tubulointerstitium, using RASi and SGLT2i, singly or in combination
- A thorough cardiovascular risk assessment and commencement of appropriate interventions, as necessary
Offer participation in a clinical trial if one is available.
There is insufficient evidence to support the use of the Oxford Classification MEST-C score in determining which drug should be commenced in patients with IgAVN.
There is insufficient evidence to base treatment decisions on the presence and number of crescents in the kidney biopsy.
The International IgAN Prediction Tools cannot be used to determine the likely impact of any particular treatment regimen.
Dynamic assessment of patient risk over time should be performed, as decisions regarding immunosuppression may change.

1.10 Special situations

Practice Point 1.10.1: IgAV with RPGN:

The potential risks and benefits of immunosuppression should be evaluated at the individual patient level and discussed with the patient.
Patients agreeing to the treatment should be treated in accordance with the KDIGO 2024 Clinical Practice Guideline for the Management of Antineutrophil Cytoplasmic Antibody (ANCA)–Associated Vasculitis.87
IgAVN with RPGN, as well as other presentations of IgAVN, may be associated with significant extrarenal involvement (pulmonary, gastrointestinal, and skin), which may dictate alternative immunosuppressive strategies.
There are insufficient data to determine the efficacy of plasma exchange in IgAVN with RPGN. However, uncontrolled case series describe the potential role of the addition of plasma exchange to glucocorticoid therapy to accelerate recovery in patients with life- or organ-threatening extrarenal complications of IgAV.116

1.10.1 IgAVN in children

Practice Point 1.10.1.1: In this guideline, we define children as those aged <18 years, but it is acknowledged that postpubertal children in some respects may have a similar course and response to treatment as adults with IgAN. However, there are insufficient data currently to recommend that they be managed as adults with IgAN.

Practice Point 1.10.1.2: A more extensive review of the management of IgAVN in children can be found in the 2025 International Pediatric Nephrology Association clinical practice recommendations for the diagnosis and management of children with IgA nephropathy and IgA vasculitis nephritis.1
Briefly:

The majority of children who will develop nephritis do so within 3 months of presentation. Urinary monitoring is necessary at the onset of vasculitis and then at least monthly for ≥6 months from the initial presentation of systemic disease.
A kidney biopsy should be promptly performed in children with nephrotic-range proteinuria or impaired kidney function (GFR <90 ml/min per 1.73 m2).
In children with IgAV and PCR 1000–2000 mg/g (100–200 mg/mmol) for 2–4 weeks or PCR 200–500 mg/g (20–50 mg/mmol) for >4 weeks, a kidney biopsy should be considered.
In children with confirmed IgAVN, a pediatric nephrologist should be consulted.
In children with IgAVN and persistent proteinuria for >3 months, ACEi or ARB treatment should be considered.
There are no data supporting the use of glucocorticoids to prevent nephritis in children with IgAV and no evidence of kidney involvement or with isolated microhematuria.117,118
Oral prednisone/prednisolone for 3–6 months or pulsed intravenous methylprednisolone should be considered in children with IgAVN and nephrotic-range proteinuria (PCR >2000 mg/g or 200 mg/mmol) or RPGN and histologic risk of progression (International Study of Kidney Disease in Children [ISKDC] criteria ≥II).
Other immunosuppressive agents in addition to glucocorticoids (e.g., calcineurin inhibitors, cyclophosphamide, mizoribine where available, mycophenolate mofetil, or rituximab) should be considered in the following indications: to reduce the glucocorticoid dose and/or if the PCR is >2000 mg/g (200 mg/mmol) and/or if there is insufficient response to glucocorticoids.
Children with IgAVN with nephrotic syndrome and/or rapidly deteriorating kidney function are treated in the same way as those with rapidly progressive IgAN.
Monitoring children with IgAVN with the evaluation of urinalysis, eGFR, and blood pressure should be considered for ≥5 years after the initial episode. Lifelong monitoring, individualized according to the severity and response to treatment, appears prudent for children who received therapy for their IgAVN.

Nomenclature and Description for Rating Guideline Recommendations

Strength of Recommendation and Implications
Grade	Patients	Clinicians	Policy
Level 1 "We recommend"	Most people in your situation would want the recommended course of action, and only a small proportion would not.	Most patients should receive the recommended course of action.	The recommendation can be evaluated as a candidate for developing a policy or a performance measure.
Level 2 "We suggest"	The majority of people in your situation would want the recommended course of action, but many would not.	Different choices will be appropriate for different patients. Each patient needs help to arrive at a management decision consistent with her or his values and preferences.	The recommendation is likely to require substantial debate and involvement of stakeholders before policy can be determined.

Quality of Evidence
Grade	Quality of evidence	Meaning
A	High	We are confident that the true effect is close to the estimate of the effect.
B	Moderate	The true effect is likely to be close to the estimate of the effect, but there is a possibility that it is substantially different.
C	Low	The true effect may be substantially different from the estimate of the effect.
D	Very low	The estimate of effect is very uncertain, and often it will be far from the true effect.

Document Overview

Document Title: Management of Immunoglobulin A Nephropathy (IgAN) and Immunoglobulin A Vasculitis (IgAV)

Authoring Society: Kidney Disease Improving Global Outcomes

Document Publication Date: Sep 18, 2025

Page Last Reviewed/Updated: May 6, 2026

Document Type: Guideline

Country of Publication: United States

Full Text Freely Available: Yes

Full Text Guideline: www.kidney-international.org/article/S0085-2538(25)00279-0/fulltext

Source Citation: Brad H. Rovin, Jonathan Barratt, H. Terence Cook, Irene L. Noronha, Heather N. Reich, Yusuke Suzuki, Sydney C.W. Tang, Hernán Trimarch, Jürgen Floege, KDIGO 2025 Clinical Practice Guideline for the Management of Immunoglobulin A Nephropathy (IgAN) and Immunoglobulin A Vasculitis (IgAV), Kidney International, Volume 108, Issue 4, Supplement, 2025, Pages S1-S71, ISSN 0085-2538, https://doi.org/10.1016/j.kint.2025.04.004.

Supplemental Implementation Resources

Document Scope, Criteria, and Use Cases

Document Objectives: IgAN is the most common primary glomerular disease and a leading cause of chronic kidney disease and kidney failure. IgAV, historically known as Henoch–Schönlein purpura, also carries significant risks for kidney damage, particularly in children. The new guideline integrates evidence from randomized controlled trials through August 2024 to provide clinicians with the most up-to-date recommendations for diagnosis, prognosis, and treatment. A significant advance is the focus of management to simultaneously prevent or reduce IgA-containing immune complex (IgA-IC) formation and IgA-IC injury while treating the complications of IgAN-related kidney damage, including glomerular hyperfiltration and systemic hypertension. Key recommendations include optimized use of ACEi or ARB for all patients, targeted-release budesonide (Nefecon) for those at risk of progressive loss of kidney function, and newer agents such as sparsentan (a dual endothelin angiotensin receptor antagonist) and sodium-glucose cotransporter-2 (SGLT2) inhibitors to manage responses to IgAN-induced nephron loss. The guideline also provides guidance for special situations such as IgAN with nephrotic syndrome, acute kidney injury, or rapidly progressive glomerulonephritis. Additional guidance for pregnancy planning and managing IgAN in children is also provided. The guideline also highlights global challenges. Many regions with the highest prevalence of IgAN, such as Asia, remain underrepresented in clinical trials and face barriers to accessing newly approved therapies. KDIGO emphasizes the need for equity so that advances benefit all patients, not only those in resource-rich settings.

Scope: Assessment and Screening, Diagnosis, Management

Diseases/Conditions (MeSH)

D007070 - Immunoglobulin A

Keywords: IgAN, IgAV, Immunoglobulin A Nephropathy, Immunoglobulin A Vasculitis, Immunoglobulin A nephropathy (IgAN), glomerular diseases

Inclusion Criteria: Male, Female, Adolescent, Adult, Child, Older Adult

Health Care Settings: Ambulatory, Outpatient

Intended Users: Nurse, Nurse Practitioner, Physician, Physician Assistant

Recommendation Development Processes & Methodology

Supplemental Methodology Resource: IgAN/IgAV Guideline Website

Management of Immunoglobulin A Nephropathy (IgAN) and Immunoglobulin A Vasculitis (IgAV)

Summary of recommendation statements and practice points

Immunoglobulin A nephropathy

Practice Points

1.2 Diagnosis

1.3 Prognosis

1.4 Treatment

1.5 Special situations

Recommendations

Recommendation 1.4.3.1

Recommendation 1.4.3.2

Recommendation 1.4.4.1

Recommendation 1.4.4.2

Recommendation 1.4.4.3

Figure 1. Data elements included in the International Immunoglobulin A Nephropathy (IgAN) Prediction Tools

Figure 2. Initial assessment and management of the patient with immunoglobulin A nephropathy (IgAN)

Figure 3. Treatment targets in immunoglobulin A nephropathy (IgAN) and the positioning of drugs included in this guideline

Figure 4. Other pharmacologic therapies evaluated in immunoglobulin A nephropathy (IgAN)

Table 1. Factors to consider when choosing a treatment and/or treatment combinations for patients with IgAN at risk of progressive loss of kidney function

Table 2. Baseline characteristics and key inclusion criteria for recently reported trials in IgAN and including trials with significant numbers of patients with IgAN

Immunoglobulin A vasculitis

Practice Points

1.7 Diagnosis

1.8 Prognosis

1.9 Treatment

1.10 Special situations

Recommendations

Recommendation 1.9.1.1

Nomenclature and Description for Rating Guideline Recommendations

Document Overview

Supplemental Implementation Resources

Document Scope, Criteria, and Use Cases

Recommendation Development Processes & Methodology