Diagnostic and Management Strategies of IgA Vasculitis Nephritis/Henoch-Schönlein Purpura Nephritis in Pediatric Patients: Current Perspectives

Figures & data

Figure 1 Diagnostic approach to IgAV and IgAVN. Data from Ozen et al.³⁵ Adapted from Jelusic M, Sestan M, Giani T, Cimaz R. New insights and challenges associated with IgA vasculitis and IgA vasculitis with nephritis-is it time to change the paradigm of the most common systemic vasculitis in childhood? Front Pediatr. 2022;10:853724. Creative commons.³⁶

Table 1 Comparison of the SHARE and KDIGO Recommendations for Treatment of IgAVN

Parameter	SHARE Recommendations	KDIGO Recommendations
Isolated Hematuria	Follow-Up	No Recommendations
0.5–1 g/day proteinuria with normal eGFR	Oral prednisone angiotensin converting enzyme inhibitors or angiotensin receptor blockers if persists more than 3 months	Angiotensin converting enzyme inhibitors or angiotensin receptor blockers
1–2.5 g/day proteinuria with normal eGFR	Oral prednisone angiotensin converting enzyme inhibitors or angiotensin receptor blockers if persists more than 3 months	Angiotensin converting enzyme inhibitors or angiotensin receptor blockers and then glucocorticoids
>2.5 g/day proteinuria with no crescents or <50% of cresecents	Oral prednisone and/or glucocorticoid pulses angiotensin converting enzyme inhibitors or angiotensin receptor blockers if persists more than 3 months	Angiotensin converting enzyme inhibitors or angiotensin receptor blockers and then glucocorticoids if no crescents glucocorticoids and cyclophosphamide if crescents
>2.5 g/day proteinuria with >50% of crescents	Cyclophosphamide pulses, glucocorticoid pulses then oral glucocorticoids angiotensin converting enzyme inhibitors or angiotensin receptor blockers if persists more than 3 months	Glucocorticoids and cyclophosphamide
eGFR <80 mL/min/1.73 m^2 or deteriorating kidney function	Oral prednisone and/or glucocorticoid pulses if <50% of crescents cyclophosphamide pulses, glucocorticoid pulses then oral glucocorticoids if >50% of crescents	Glucocorticoids and cyclophosphamide

Abbreviations: eGFR, glomerular filtration rate; IgAVN, IgA vasculitis nephritis; KDIGO, Kidney Disease Improving Global Outcomes; SHARE, Single Hub and Access point for pediatric Rheumatology in Europe.

Figure 2 The SHARE recommendations for IgAVN treatment. Data from Ozen et al.³⁵ Jelusic M, Sestan M, Giani T, Cimaz R. New insights and challenges associated with IgA vasculitis and IgA vasculitis with nephritis-is it time to change the paradigm of the most common systemic vasculitis in childhood? Front Pediatr. 2022;10:853724. Creative Commons.³⁶

Ozen S, Marks SD, Brogan P, et al. European consensus-based recommendations for diagnosis and treatment of immunoglobulin A vasculitis-the SHARE initiative. Rheumatology. 2019;58:1607–1616.

 PubMed Web of Science ®Google Scholar

Jelusic M, Sestan M, Giani T, Cimaz R. New insights and challenges associated with IgA vasculitis and IgA vasculitis with nephritis-is it time to change the paradigm of the most common systemic vasculitis in childhood? Front Pediatr. 2022;10:853724.

 PubMed Web of Science ®Google Scholar