Table 4

The recommendations for the management of complications associated with IgA vasculitis (IgAV) in children and young people (taken from The UK Kidney Association100)

NumberRecommendationGrade
Section 1: Clinically suspected IgAV nephritis
1.1We recommend that a kidney biopsy is undertaken to confirm a diagnosis of severe nephritis in children and young people with IgAV, where the definition of severe nephritis includes persisting severe proteinuria (UP: UC>250 mg/mmol for up to 4 weeks), persisting moderate proteinuria (UP: UC 100–250 mg/mmol for 3 months), AKI stage 1 or greater (at any time), or nephrotic syndrome (at any time; also see Section 2.2).1C
Section 2: Management of histologically proven IgAV nephritis
2.1We recommend that the kidney histology should be classified using the ISKDC classification criteria for children and young people with IgAV.1C
2.2We recommend that a combination of clinical features and the histological ISKDC classification should guide decisions about treatment choices to offer children and young people with IgAV nephritis.
The following are clinical indications;

  • Persisting severe proteinuria (UP: UC>250 mg/mmol for up to 4 weeks)

  • Persisting moderate proteinuria (UP: UC 100–250 mg/mmol for up to 3 months)

  • AKI stage 1 or greater (serum creatinine>1.5 × previous baseline (if known) or>1.5 × upper limit of normal for age)

  • Nephrotic syndrome (clinical signs of oedema, serum albumin<30 g/L, severe proteinuria UP: UC>250 mg/mmol).


The following are histological indications;

  • Class II ISKDC classification with persisting clinical indications

  • Class III or above ISKDC classification with clinical indications

1C
2.3We suggest that the management of biopsy proven IgAV nephritis should be directed by, or in conjunction with, a paediatric nephrologist2B
2.4We suggest using the following disease-modifying drugs, or a combination of corticosteroids together with a disease-modifying drug, depending on the clinical and histological features in children and young people with biopsy proven IgAV nephritis.
Corticosteroids;

  • Prednisolone 1–2 mg/kg/day (maximum 60 mg/day) for 2–4 weeks then weaned according to clinical response

  • In severe nephritis with adverse clinical and histological features (impaired kidney function, nephrotic syndrome, or crescentic features), or where oral absorption is potentially compromised, intravenous methylprednisolone 10–30 mg/kg (maximum of 1 g/day) once daily for three consecutive days may be used followed by the use of oral prednisolone.


Disease-modifying drug (listed in alphabetical order);

  • Azathioprine

  • Calcineurin inhibitors (ciclosporin or tacrolimus)

  • Cyclophosphamide

  • Mycophenolate mofetil.


Rapidly progressive glomerulonephritis is managed more aggressively with preference for intravenous treatment.		2B
2.5We suggest that in cases of IgAV nephritis with persisting proteinuria (UP: UC>100 mg/mmol for 3 months or UP: UC>50 mg/mmol for 6 months) the use of an angiotensin converting enzyme inhibitor (ACEi) or an angiotensin receptor blocker (ARB) should be considered as adjunctive or monotherapy under the guidance of a nephrologist, even if they haven’t met the threshold for performing a kidney biopsy (section 1.1).2C
Section 3: Management of acute GI bleeding
3.1We suggest that corticosteroids (prednisolone 1–2 mg/kg/day for 1–2 weeks) are considered for children and young people with IgAV within 3 days of onset of severe abdominal pain (defined as pain requiring hospital admission) or acute GI bleeding after appropriate clinical review and the exclusion of other causes including intussusception.2B
Section 4: Management of suspected or proven intussusception
4.1We recommend that specialist surgical and(/or)radiological advice is sought for children and young people with IgAV and abdominal symptoms suggestive of intussusception.1C
Section 5: Management of suspected or proven testicular involvement
5.1We suggest that testicular involvement (orchiditis) should be considered in boys with IgAV who develop painful scrotal oedema that is associated with palpable purpuric lesions.2D
5.2We suggest that treatment with corticosteroids (prednisolone 1–2 mg/kg/day for 1–2 weeks) should be considered in boys with IgAV who develop orchiditis after appropriate specialist advice such as a surgical opinion has been sought.2D
Section 6: Management of cases with atypical features
6.1We suggest that a skin biopsy is undertaken in children and young people with IgAV who have an atypical purpuric/petechial rash or to exclude alternative diagnoses.2B
6.2We suggest that when a skin biopsy is performed the histological analysis should specifically include evaluation of IgA deposition using immunofluorescence (fresh specimen) or immunohistochemistry (fixed tissue).2C
Section 7: Definition of persisting or recurrent disease
7.1We suggest that children and young people with IgAV and a typical purpuric/petechial rash persisting for more than 1 month should be defined as having persisting disease.2B
7.2We suggest that children and young people with IgAV who present with a reappearance of the typical purpuric/petechial rash after a symptom-free period of greater than 1 month should be defined as having recurrent disease.2B
Section 8: Long term follow up in IgAV
8.1We recommend that children and young people with IgAV should have follow up whilst there is evidence of nephritis and for at least 3 years if they have experienced biopsy proven nephritis.1C

  • AKI, acute kidney injury; ISKDC, International Study of Kidney Diseases in Children; UP:UC, urine protein to urine creatinine ratio.