MANAGEMENT OF IGA

VASCULITIS NEPHRITIS
(HENOCH-SCHONLEIN
PURPURA NEPHRITIS) IN
CHILDREN
Introduction

■ Renal Impairment is a major complication of immunoglobulin A

Vasculitis
■ This systematic review focused on the following questions:
– Can biomarkers and genes predict renal involvement?
– Can these predictors be used to prevent progression of
renal disease?
– Are there any new recommendation for Ig A vasculitis
management??
Nomenclature

■ International Chapel Hill Consensus Conference (CCHC 2012)

– Henoch-Schonlein Purpura (HSP)  Ig A vasculitis
(IgAV).
– Better define the pathophysiological features observed in
this condition
– Henoch-Schonlein Purpura Nephritis  Ig A Vasculitis
Nephritis (IgAVN)
Diagnosis of Immunoglobulin
A Vasculitis
■ American College of Rheumatology (ACR), at least 2 of :
– Age ≤ 20 years of disease onset
– Palpable purpura
– Acute Abdominal Pain
– Biopsy showing granulocytes in the walls of small
arterioles/venules
Diagnosis of Immunoglobulin
A Vasculitis
■ 2010, European League against Rheumatism and the Pediatric Rheumatology
European Society
■ Purpura or Erythema predominantly observed over the lower extremities, plus one
of these four :
– Diffuse abdominal pain
– Histopathological findings of typical leukocytoclastic vasculitis with
predominant IgA deposits or proliferative glomerulonephritis with
predominant igA deposits
– Arthirits or arthalgia
– Renal involvement
■ Proteinuria > 0.3g/24 hours, > 30 mmol/mg of urine albumin to creatinine ratio
in a spot urine morning sample
■ Hematuria
Renal Involvement in IgA
Vasculitis
■ 20 – 50 % in patients with IgA Vasculitis
■ Most common findings  microscopic hematuria
■ Nephritis rarely precedes purpura onset
■ 97% patients with nephritis, develop urinary abnormality within 6
months after the onset of other symptoms.
■ Urinalysis
– Weekly during active phase of Ig A Vasculitis
– Monthly for 6 month after resolution of active phase
– 24 month follow up despite resolution of urinary abnormalities
Risk Factors associated with renal involvement in IgAV

■ Clinical risk factor

– Persisten purpura and GI bleeding, associated with sever e abdominal pain 
predictors of nephritis
– Recurrence of Purpura  higher risk than monocyclic purpura to develop
nephritis
■ Genes and biomarkers
– Reduced activity of the C1GALT1 gene  abberation of glycosylation of IgA1
– Nitric oxide synthase polymorphisms
– Angiotensin-converting enzyme (ACE) gene
■ Proteomic analysis
– Angiotensionogen concentration was positively associated with ig A Vasculitis
Nephritis
Long term Prognosis of Ig A
Vasculitis Nephritis
■ The prognosis of IgAVN is better in children than in adults
■ The previous cohort studies indicated that pediatric IgAVN is
not a mild disease and require long-term follow up during
adulthood.
■ Proteinuria and hematuria risk : 15%
■ Progression to ESKD : 50%
 Prevention of progression to severity

■ A report did not describe any significant initial features that could help
to identify children at risk of disease progression, not corticosteroid
nor cytotoxic agents
■ Early treatment might prevent progression of cellular crescent
deposition to fibrosis and consequent chronic kidney disease.
■ Plasmapheresis effectively removes IgA-containing complexes and
prevents the development of and exacerbation
Treatment of IgAVN

■ KDIGO 2012 and SHARE 2019 guidelines

■ KDIGO 2012 : 3–6 month administration of ACE inhibitors
(ACEi) or angiotensin receptor blockers (ARBs) in children with
proteinuria >0.5 g/day (including those with nephrotic
syndrome) and glucocorticoid therapy is necessary in patients
refractory to this treatment.
Conclusion

■ The progress of the disease differs between children and adults.

Following the KDIGO recommendation in 2012, the 2019
SHARE group established guidelines for the treatment of
IgAVN in children.
■ Future research on the pathophysiology of IgAN and clinical
trials that are currently underway are expected to result in the
development of novel drugs in patients with IgAVN.