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

ACUTE

without renal involvement

1st line – supportive care

Back
ACUTE
|
without renal involvement
1st line – 

supportive care

Most children with IgAV only require adequate analgesia and supportive treatment. IgAV commonly resolves spontaneously over weeks to a few months; complete recovery occurs in 94% of children.[35]​ The primary goal is therefore to provide symptomatic treatment.

The skin rash of IgAV can be managed conservatively in the majority of cases. A topical corticosteroid may be used for dermatological manifestations.

Joint pain can be treated with either paracetamol or a non-steroidal anti-inflammatory drug (NSAID) such as ibuprofen (both of which are considered equally efficacious); mild to moderate abdominal pain can be managed with paracetamol, rest, and supportive care.[31]​ Concerns about renal toxicity, cardiovascular events, and gastrointestinal (GI) bleeding often limit the use of NSAIDs, and they should not be used first line for pain management in patients with IgAV. NSAIDs should be used at the lowest effective dose for the shortest duration possible.

Primary options

paracetamol: consult product literature for guidance on dose

Secondary options

ibuprofen: 5-10 mg/kg orally every 6-8 hours when required, maximum 40 mg/kg/day

Consider – corticosteroid and/or immunosuppressant

Back
ACUTE
|
without renal involvement
Consider – 

corticosteroid and/or immunosuppressant

Additional treatment recommended for SOME patients in selected patient group

Patients with severe rash (such as skin blistering or necrotic areas), moderate to severe GI involvement, or orchitis are likely to respond to a short course of corticosteroid treatment and/or immunosuppressant.[33][42][43][44]​ In practice, immunosuppressants are rarely required for children without nephritis, and there is very little evidence to guide choice of treatment.

In any case of severe extrarenal symptoms such as organ or life-threatening vasculitis (including cerebral vasculitis, or pulmonary haemorrhage), hospital admission, intravenous corticosteroids, and immunosuppressants are recommended.[29]

In cases of severe abdominal pain or rectal bleeding, complications such as intestinal intussusception must be excluded.[33]

Consult a specialist for guidance on the best treatment regimen and doses.

Consider – plasmapheresis

Back
ACUTE
|
without renal involvement
Consider – 

plasmapheresis

Additional treatment recommended for SOME patients in selected patient group

In severe, immediately life-threatening disease, plasmapheresis may be considered; however, the lack of a causative antibody may make active monitoring more challenging than in other forms of vasculitis.[37][45]

Evidence on the use of plasmapheresis in patients with IgAV is limited. Case series suggest plasmapheresis may be beneficial for patients with life- or organ-threatening extrarenal complications of IgAV.[37]

mild nephritis: normal GFR and mild or moderate proteinuria

1st line – oral corticosteroid

Back
ACUTE
|
mild nephritis: normal GFR and mild or moderate proteinuria
1st line – 

oral corticosteroid

Children should be referred to a nephrologist when starting treatment for IgAV nephritis.

In mild nephritis that has been biopsy proven, for most clinicians the first-line treatment is oral prednisolone. Supporting evidence is, however, of low quality.[29]

Long-term corticosteroid therapy may be associated with serious adverse effects including growth retardation, adrenal suppression, Cushing syndrome, glaucoma, cataracts, increased risk of infection, osteoporosis, and osteonecrosis. Cardiovascular, gastrointestinal (GI), and neuropsychiatric adverse effects are also possible. Preventative measures should be taken where possible (e.g., gastroprotection, bone protection, Pneumocystis pneumonia prophylaxis). Monitor growth velocity.

Primary options

prednisolone: 1-2 mg/kg/day orally for 1-2 weeks, taper gradually according to response, maximum 75 mg/day

Consider – immunosuppressant

Back
ACUTE
|
mild nephritis: normal GFR and mild or moderate proteinuria
Consider – 

immunosuppressant

Additional treatment recommended for SOME patients in selected patient group

Azathioprine or mycophenolate may be considered as corticosteroid-sparing agents.[29]​ The immunosuppressant can be started while the patient is still receiving oral prednisolone, and up titrated as the corticosteroid dose is tapered.

Azathioprine may be associated with GI adverse effects, dose-related haematological toxicities, hepatotoxicity, pancreatitis, and an increased risk of infections or malignancy.

Mycophenolate may be associated with GI adverse effects, bone marrow suppression, haematological toxicities, and an increased risk of infections and malignancy.

Primary options

azathioprine: consult specialist for guidance on dose

OR

mycophenolate mofetil: consult specialist for guidance on dose

Consider – ACE inhibitor or angiotensin-II receptor antagonist

Back
ACUTE
|
mild nephritis: normal GFR and mild or moderate proteinuria
Consider – 

ACE inhibitor or angiotensin-II receptor antagonist

Additional treatment recommended for SOME patients in selected patient group

An ACE inhibitor (e.g., enalapril) or an angiotensin-II receptor antagonist (e.g., losartan) should be added to prevent or limit glomerular injury in patients with persistent proteinuria.[7][29][33]​​

While these agents are typically used in combination with immunosuppressive therapy, UK guidance suggests that they may be considered as a first-line treatment without immunosuppressive therapy in mild cases associated with persistent proteinuria, even if they have not met the threshold for a kidney biopsy.[33]

Primary options

enalapril: 0.2 to 0.6 mg/kg/day orally, maximum 20 mg/day

OR

losartan: 0.35 to 1.4 mg/kg/day orally, maximum 100 mg/day

Consider – conservative measures for other organ involvement

Back
ACUTE
|
mild nephritis: normal GFR and mild or moderate proteinuria
Consider – 

conservative measures for other organ involvement

Additional treatment recommended for SOME patients in selected patient group

The skin rash of IgAV can be managed conservatively in the majority of cases. A topical corticosteroid may be used for dermatological manifestations.

Joint pain can be treated with either paracetamol or a non-steroidal anti-inflammatory drug (NSAID) such as ibuprofen (both of which are considered equally efficacious); mild to moderate abdominal pain can be managed with paracetamol, rest, and supportive care.[31]​ Concerns about renal toxicity, cardiovascular events, and GI bleeding often limit the use of NSAIDs, and they should not be used first line for pain management in patients with IgAV. NSAIDs should be used at the lowest effective dose for the shortest duration possible.

Patients with severe rash (such as skin blistering or necrotic area), moderate to severe GI involvement, or orchitis are likely to respond to the corticosteroid and/or immunosuppressant prescribed for mild nephritis.[33][42][43]​​​ 

Primary options

paracetamol: consult product literature for guidance on dose

Secondary options

ibuprofen: 5-10 mg/kg orally every 6-8 hours when required, maximum 40 mg/kg/day

2nd line – immunosuppressant

Back
ACUTE
|
mild nephritis: normal GFR and mild or moderate proteinuria
2nd line – 

immunosuppressant

Azathioprine or mycophenolate can be used as a second-line therapy without overlap with oral prednisolone.[29]

Azathioprine may be associated with GI adverse effects, dose-related haematological toxicities, hepatotoxicity, pancreatitis, and an increased risk of infections or malignancy.

Mycophenolate may be associated with GI adverse effects, bone marrow suppression, haematological toxicities, and an increased risk of infections and malignancy.

Primary options

azathioprine: consult specialist for guidance on dose

OR

mycophenolate mofetil: consult specialist for guidance on dose

Consider – ACE inhibitor or angiotensin-II receptor antagonist

Back
ACUTE
|
mild nephritis: normal GFR and mild or moderate proteinuria
Consider – 

ACE inhibitor or angiotensin-II receptor antagonist

Additional treatment recommended for SOME patients in selected patient group

An ACE inhibitor (e.g., enalapril) or an angiotensin-II receptor antagonist (e.g., losartan) should be added to prevent or limit glomerular injury in patients with persistent proteinuria.[7][29][33]​​

While these agents are typically used in combination with immunosuppressive therapy, UK guidance suggests that they may be considered as a first-line treatment without immunosuppressive therapy in mild cases associated with persistent proteinuria, even if they have not met the threshold for a kidney biopsy.[33]

Primary options

enalapril: 0.2 to 0.6 mg/kg/day orally, maximum 20 mg/day

OR

losartan: 0.35 to 1.4 mg/kg/day orally, maximum 100 mg/day

Consider – conservative measures for other organ involvement

Back
ACUTE
|
mild nephritis: normal GFR and mild or moderate proteinuria
Consider – 

conservative measures for other organ involvement

Additional treatment recommended for SOME patients in selected patient group

The skin rash of IgAV can be managed conservatively in the majority of cases. A topical corticosteroid may be used for dermatological manifestations.

Joint pain can be treated with either paracetamol or an NSAID such as ibuprofen (both of which are considered equally efficacious); mild to moderate abdominal pain can be managed with paracetamol, rest, and supportive care.[31]​ Concerns about renal toxicity, cardiovascular events, and GI bleeding often limit the use of NSAIDs, and they should not be used first line for pain management in patients with IgAV. NSAIDs should be used at the lowest effective dose for the shortest duration possible.

Patients with severe rash (such as skin blistering or necrotic area), moderate to severe GI involvement, or orchitis are likely to respond to the immunosuppressant prescribed for mild nephritis.[33][42][43]​​​

Primary options

paracetamol: consult product literature for guidance on dose

Secondary options

ibuprofen: 5-10 mg/kg orally every 6-8 hours when required, maximum 40 mg/kg/day

moderate nephritis: <50% crescents on renal biopsy and impaired GFR or severe persistent proteinuria

1st line – intravenous and/or oral corticosteroid

Back
ACUTE
|
moderate nephritis: <50% crescents on renal biopsy and impaired GFR or severe persistent proteinuria
1st line – 

intravenous and/or oral corticosteroid

Children should be referred to a nephrologist when starting treatment for IgAV nephritis.

Pulsed-dose intravenous methylprednisolone and/or oral prednisolone should be used as first-line treatment in patients with moderate IgAV nephritis.[29]​ Pulsed-dose intravenous methylprednisolone is indicated for severe organ or life-threatening manifestations of IgAV.[29]​ 

Long-term corticosteroid therapy may be associated with serious adverse effects including growth retardation, adrenal suppression, Cushing syndrome, glaucoma, cataracts, increased risk of infection, osteoporosis, and osteonecrosis. Cardiovascular, gastrointestinal (GI), and neuropsychiatric adverse effects are also possible. Preventative measures should be taken where possible (e.g., gastroprotection, bone protection, Pneumocystis pneumonia prophylaxis). Monitor growth velocity.

Primary options

methylprednisolone sodium succinate: 10-30 mg/kg intravenously every 24 hours for 3 days (followed by oral prednisolone), maximum 1000 mg/day

and/or

prednisolone: 1-2 mg/kg/day orally for 1-2 weeks, taper gradually according to response, maximum 75 mg/day

Consider – immunosuppressant

Back
ACUTE
|
moderate nephritis: <50% crescents on renal biopsy and impaired GFR or severe persistent proteinuria
Consider – 

immunosuppressant

Additional treatment recommended for SOME patients in selected patient group

Azathioprine, mycophenolate, a calcineurin inhibitor (e.g., ciclosporin, tacrolimus), or intravenous cyclophosphamide may be used as part of first-line treatment as a corticosteroid-sparing agent, subject to clinical features and histopathological findings of renal biopsy.​[29][33]​ The immunosuppressant can be started while the patient is still receiving oral prednisolone, and up titrated as the corticosteroid is tapered. Intravenous cyclophosphamide is usually reserved for more severe cases.​

Cyclophosphamide may be associated with bone marrow suppression, pulmonary toxicity, hepatotoxicity, haemorrhagic cystitis, and an increased risk of infections and malignancy. Doses should be taken in the morning with increased fluid intake to prevent haemorrhagic cystitis; mesna may be prescribed in some patients, if appropriate.

Azathioprine may be associated with GI adverse effects, dose-related haematological toxicities, hepatotoxicity, pancreatitis, and an increased risk of infections and malignancy.

Mycophenolate may be associated with GI adverse effects, bone marrow suppression, haematological toxicities, and an increased risk of infections and malignancy.

Calcineurin inhibitors may be associated with nephrotoxicity, neurotoxicity, hepatotoxicity, gingival hyperplasia, hypertension, and an increased risk of infections and malignancy. Serum drug monitoring is recommended.

Primary options

azathioprine: consult specialist for guidance on dose

OR

mycophenolate mofetil: consult specialist for guidance on dose

OR

ciclosporin: consult specialist for guidance on dose

OR

tacrolimus: consult specialist for guidance on dose

OR

cyclophosphamide: consult specialist for guidance on dose

Consider – ACE inhibitor or angiotensin-II receptor antagonist

Back
ACUTE
|
moderate nephritis: <50% crescents on renal biopsy and impaired GFR or severe persistent proteinuria
Consider – 

ACE inhibitor or angiotensin-II receptor antagonist

Additional treatment recommended for SOME patients in selected patient group

An ACE inhibitor (e.g., enalapril) or an angiotensin-II receptor antagonist (e.g., losartan) should be added to prevent or limit glomerular injury in patients with persistent proteinuria.[7][29][33]​​

Primary options

enalapril: 0.2 to 0.6 mg/kg/day orally, maximum 20 mg/day

OR

losartan: 0.35 to 1.4 mg/kg/day orally, maximum 100 mg/day

Consider – conservative measures for other organ involvement

Back
ACUTE
|
moderate nephritis: <50% crescents on renal biopsy and impaired GFR or severe persistent proteinuria
Consider – 

conservative measures for other organ involvement

Additional treatment recommended for SOME patients in selected patient group

The skin rash of IgAV can be managed conservatively in the majority of cases. A topical corticosteroid may be used for dermatological manifestations.

Joint pain can be treated with either paracetamol or a non-steroidal anti-inflammatory drug (NSAID) such as ibuprofen (both of which are considered equally efficacious); mild to moderate abdominal pain can be managed with paracetamol, rest, and supportive care.[31]​ Concerns about renal toxicity, cardiovascular events, and GI bleeding often limit the use of NSAIDs, and they should not be used first line for pain management in patients with IgAV. NSAIDs should be used at the lowest effective dose for the shortest duration possible.

Patients with severe rash (such as skin blistering or necrotic area), moderate to severe GI involvement, or orchitis are likely to respond to the corticosteroid and/or immunosuppressant prescribed for moderate nephritis.[33][42][43]​​​

Primary options

paracetamol: consult product literature for guidance on dose

Secondary options

ibuprofen: 5-10 mg/kg orally every 6-8 hours when required, maximum 40 mg/kg/day

2nd line – immunosuppressant

Back
ACUTE
|
moderate nephritis: <50% crescents on renal biopsy and impaired GFR or severe persistent proteinuria
2nd line – 

immunosuppressant

Azathioprine, mycophenolate, a calcineurin inhibitor (e.g., ciclosporin, tacrolimus), or intravenous cyclophosphamide may be used as a second-line treatment, subject to clinical features and histopathological findings of renal biopsy.[29][33]​​ The immunosuppressant can be used as a second line therapy without overlap with oral prednisolone. Intravenous cyclophosphamide is usually reserved for more severe cases.

Cyclophosphamide may be associated with bone marrow suppression, pulmonary toxicity, hepatotoxicity, haemorrhagic cystitis, and an increased risk of infections and malignancy. Doses should be taken in the morning with increased fluid intake to prevent haemorrhagic cystitis; mesna may be prescribed in some patients, if appropriate.

Azathioprine may be associated with GI adverse effects, dose-related haematological toxicities, hepatotoxicity, pancreatitis, and an increased risk of infections and malignancy.

Mycophenolate may be associated with GI adverse effects, bone marrow suppression, haematological toxicities, and an increased risk of infections and malignancy.

Calcineurin inhibitors may be associated with nephrotoxicity, neurotoxicity, hepatotoxicity, gingival hyperplasia, hypertension, and an increased risk of infections and malignancy. Serum drug monitoring is recommended.

Primary options

azathioprine: consult specialist for guidance on dose

OR

mycophenolate mofetil: consult specialist for guidance on dose

OR

ciclosporin: consult specialist for guidance on dose

OR

tacrolimus: consult specialist for guidance on dose

OR

cyclophosphamide: consult specialist for guidance on dose

Consider – ACE inhibitor or angiotensin-II receptor antagonist

Back
ACUTE
|
moderate nephritis: <50% crescents on renal biopsy and impaired GFR or severe persistent proteinuria
Consider – 

ACE inhibitor or angiotensin-II receptor antagonist

Additional treatment recommended for SOME patients in selected patient group

An ACE inhibitor (e.g., enalapril) or an angiotensin-II receptor antagonist (e.g., losartan) should be added to prevent or limit glomerular injury in patients with persistent proteinuria.[7][29][33]​​

Primary options

enalapril: 0.2 to 0.6 mg/kg/day orally, maximum 20 mg/day

OR

losartan: 0.35 to 1.4 mg/kg/day orally, maximum 100 mg/day

Consider – conservative measures for other organ involvement

Back
ACUTE
|
moderate nephritis: <50% crescents on renal biopsy and impaired GFR or severe persistent proteinuria
Consider – 

conservative measures for other organ involvement

Additional treatment recommended for SOME patients in selected patient group

The skin rash of IgAV can be managed conservatively in the majority of cases. A topical corticosteroid may be used for dermatological manifestations.

Joint pain can be treated with either paracetamol or an NSAID such as ibuprofen (both of which are considered equally efficacious); mild to moderate abdominal pain can be managed with paracetamol, rest, and supportive care.[31]​ Concerns about renal toxicity, cardiovascular events, and GI bleeding often limit the use of NSAIDs, and they should not be used first line for pain management in patients with IgAV. NSAIDs should be used at the lowest effective dose for the shortest duration possible.

Patients with severe rash (such as skin blistering or necrotic area), moderate to severe GI involvement, or orchitis are likely to respond to the immunosuppressant prescribed for moderate nephritis.[33][42][43]​​​

Primary options

paracetamol: consult product literature for guidance on dose

Secondary options

ibuprofen: 5-10 mg/kg orally every 6-8 hours when required, maximum 40 mg/kg/day

severe nephritis: >50% crescents on renal biopsy and impaired GFR or severe persistent proteinuria

1st line – induction therapy: immunosuppressant + intravenous and/or oral corticosteroid

Back
ACUTE
|
severe nephritis: >50% crescents on renal biopsy and impaired GFR or severe persistent proteinuria
1st line – 

induction therapy: immunosuppressant + intravenous and/or oral corticosteroid

Children should be referred to a nephrologist when starting treatment for IgAV nephritis. Patients with severe IgAV nephritis are admitted to hospital.

Severe IgAV nephritis is managed in a similar manner to severe systemic small vessel vasculitides, such as ANCA-associated vasculitis. An immunosuppressant in combination with an intravenous and/or oral corticosteroid is recommended to induce remission.[29]

Evidence from uncontrolled observational studies suggests that intravenous pulsed-dose methylprednisolone may improve outcomes.[39][40]

There is a lack of evidence to support a preferred immunosuppressant.[33][36][38]​ The SHARE (Single Hub and Access point for paediatric Rheumatology in Europe) guideline recommends cyclophosphamide first line for remission induction.[29] Azathioprine, a calcineurin inhibitor (e.g., ciclosporin, tacrolimus), or mycophenolate may be used as second-line options.[29][33]​​

There is some evidence to suggest that patients with severe IgAV nephritis have more severe extrarenal symptoms.[8]​ In any case of organ or life-threatening vasculitis (including cerebral vasculitis, or pulmonary haemorrhage), hospital admission, intravenous corticosteroids, and immunosuppressants are recommended.[29]

Long-term corticosteroid therapy may be associated with serious adverse effects including growth retardation, adrenal suppression, Cushing syndrome, glaucoma, cataracts, increased risk of infection, osteoporosis, and osteonecrosis. Cardiovascular, gastrointestinal (GI), and neuropsychiatric adverse effects are also possible. Preventative measures should be taken where possible (e.g., gastroprotection, bone protection, Pneumocystis pneumonia prophylaxis). Monitor growth velocity.

Cyclophosphamide may be associated with bone marrow suppression, pulmonary toxicity, hepatotoxicity, haemorrhagic cystitis, and an increased risk of infections and malignancy. Doses should be taken in the morning with increased fluid intake to prevent haemorrhagic cystitis; mesna may be prescribed in some patients, if appropriate.

Azathioprine may be associated with GI adverse effects, dose-related haematological toxicities, hepatotoxicity, pancreatitis, and an increased risk of infections and malignancy.

Calcineurin inhibitors may be associated with nephrotoxicity, neurotoxicity, hepatotoxicity, gingival hyperplasia, hypertension, and an increased risk of infections and malignancy. Serum drug monitoring is recommended.

Mycophenolate may be associated with GI adverse effects, bone marrow suppression, haematological toxicities, and an increased risk of infections and malignancy.

Expert supervision is required with respect to treatment duration, weaning, and therapeutic response.[29]

Primary options

cyclophosphamide: consult specialist for guidance on dose

-- AND --

methylprednisolone sodium succinate: 10-30 mg/kg intravenously every 24 hours for 3 days (followed by oral prednisolone), maximum 1000 mg/day

and/or

prednisolone: 1-2 mg/kg/day orally for 1-2 weeks, taper gradually according to response, maximum 75 mg/day

Secondary options

azathioprine: consult specialist for guidance on dose

or

ciclosporin: consult specialist for guidance on dose

or

tacrolimus: consult specialist for guidance on dose

or

mycophenolate mofetil: consult specialist for guidance on dose

-- AND --

methylprednisolone sodium succinate: 10-30 mg/kg intravenously every 24 hours for 3 days (followed by oral prednisolone), maximum 1000 mg/day

and/or

prednisolone: 1-2 mg/kg/day orally for 1-2 weeks, taper gradually according to response, maximum 75 mg/day

Plus – maintenance therapy: immunosuppressant + oral corticosteroid

Back
ACUTE
|
severe nephritis: >50% crescents on renal biopsy and impaired GFR or severe persistent proteinuria
Plus – 

maintenance therapy: immunosuppressant + oral corticosteroid

Treatment recommended for ALL patients in selected patient group

Once remission is achieved with induction therapy, maintenance therapy is recommended.

Azathioprine, a calcineurin inhibitor (e.g., ciclosporin, tacrolimus), or mycophenolate may be used in combination with a low-dose oral corticosteroid for maintenance treatment.[29][33]

Long-term corticosteroid therapy may be associated with serious adverse effects including growth retardation, adrenal suppression, Cushing syndrome, glaucoma, cataracts, increased risk of infection, osteoporosis, and osteonecrosis. Cardiovascular, GI, and neuropsychiatric adverse effects are also possible. Preventative measures should be taken where possible (e.g., gastroprotection, bone protection, Pneumocystis pneumonia prophylaxis). Monitor growth velocity.

Azathioprine may be associated with GI adverse effects, dose-related haematological toxicities, hepatotoxicity, pancreatitis, and an increased risk of infections and malignancy.

Calcineurin inhibitors may be associated with nephrotoxicity, neurotoxicity, hepatotoxicity, gingival hyperplasia, hypertension, and an increased risk of infections and malignancy. Serum drug monitoring is recommended.

Mycophenolate may be associated with GI adverse effects, bone marrow suppression, haematological toxicities, and an increased risk of infections and malignancy.

Expert supervision is required with respect to treatment duration, weaning, and therapeutic response.[29]

Primary options

azathioprine: consult specialist for guidance on dose

or

ciclosporin: consult specialist for guidance on dose

or

tacrolimus: consult specialist for guidance on dose

or

mycophenolate mofetil: consult specialist for guidance on dose

-- AND --

prednisolone: 1-2 mg/kg/day orally for 1-2 weeks, taper gradually according to response, maximum 75 mg/day

Consider – ACE inhibitor or angiotensin-II receptor antagonist

Back
ACUTE
|
severe nephritis: >50% crescents on renal biopsy and impaired GFR or severe persistent proteinuria
Consider – 

ACE inhibitor or angiotensin-II receptor antagonist

Additional treatment recommended for SOME patients in selected patient group

An ACE inhibitor (e.g., enalapril) or an angiotensin-II receptor antagonist (e.g., losartan) should be added to prevent or limit glomerular injury in patients with persistent proteinuria.[7][29][33]​​

Primary options

enalapril: 0.2 to 0.6 mg/kg/day orally, maximum 20 mg/day

OR

losartan: 0.35 to 1.4 mg/kg/day orally, maximum 100 mg/day

Consider – conservative measures for other organ involvement

Back
ACUTE
|
severe nephritis: >50% crescents on renal biopsy and impaired GFR or severe persistent proteinuria
Consider – 

conservative measures for other organ involvement

Additional treatment recommended for SOME patients in selected patient group

The skin rash of IgAV can be managed conservatively in the majority of cases. A topical corticosteroid may be used for dermatological manifestations.

Joint pain can be treated with either paracetamol or a non-steroidal anti-inflammatory drug (NSAID) such as ibuprofen (both of which are considered equally efficacious); mild to moderate abdominal pain can be managed with paracetamol, rest, and supportive care.[31]​ Concerns about renal toxicity, cardiovascular events, and GI bleeding often limit the use of NSAIDs, and they should not be used first line for pain management in patients with IgAV. NSAIDs should be used at the lowest effective dose for the shortest duration possible.

Patients with severe rash (such as skin blistering or necrotic area), moderate to severe GI involvement, or orchitis are likely to respond to the corticosteroid and/or immunosuppressant prescribed for severe nephritis.[33][42][43]​​​ In cases of severe abdominal pain or rectal bleeding, complications such as intestinal intussusception must be excluded.[33]

Primary options

paracetamol: consult product literature for guidance on dose

Secondary options

ibuprofen: 5-10 mg/kg orally every 6-8 hours when required, maximum 40 mg/kg/day

Consider – plasmapheresis

Back
ACUTE
|
severe nephritis: >50% crescents on renal biopsy and impaired GFR or severe persistent proteinuria
Consider – 

plasmapheresis

Additional treatment recommended for SOME patients in selected patient group

In severe, immediately life-threatening disease, plasmapheresis may be considered; however, the lack of a causative antibody may make active monitoring more challenging than in other forms of vasculitis.[37][45]​​​

Evidence on the use of plasmapheresis in patients with IgAV is limited. One systematic review assessed the use of plasmapheresis in patients with IgAV and rapidly progressive glomerulonephritis. In the included case reports and case series, 76.3% (29/38 patients) achieved remission and 23.6% (9/38 patients) progressed to end-stage kidney disease.[45]​ Case series also suggest plasmapheresis may be beneficial for patients with life- or organ-threatening extrarenal complications of IgAV.[37][46]

2nd line – renal transplant

Back
ACUTE
|
severe nephritis: >50% crescents on renal biopsy and impaired GFR or severe persistent proteinuria
2nd line – 

renal transplant

Renal transplant may be required in patients who progress to end-stage renal disease; the risk of relapse in the graft is low.[41]

ONGOING

relapsed/refractory disease

1st line – specialist referral

Back
ONGOING
|
relapsed/refractory disease
1st line – 

specialist referral

Relapsing/refractory disease is rare, and there is limited evidence for management.[33]​ Consult a specialist for guidance on managing children with relapsing/refractory disease.

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Choose a patient group to see our recommendations

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

Use of this content is subject to our disclaimer