Guideline sources The following summarized guidelines for the evaluation and management of henoch-Sch nlein purpura (HSP) are prepared by our editorial team based on guidelines from the Single Hub and Access Point for Pediatric Rheumatology in Europe (SHARE initiative 2019) and the Kidney Disease: Improving Global Outcomes Foundation (KDIGO 2012). 1 2 Calculator Calculator EULAR/PRINTO/PRES criteria for Severity grading for IgA vasculiti Guidelines 1. Classification and risk stratification https://web.pathway.md/diseases/reciVsOBWLsaejtOa 1/4 6/24/23, 12:31 PM Henoch-Sch nlein purpura Pathway Classification: use the EULAR/PRINTO/PReS-endorsed Ankara 2008 criteria for the classification of IgA vasculitis (formerly known as HSP). B 2. Diagnostic investigations Evaluation for renal involvement: obtain estimated GFR measurement and urinalysis (for hematuria and urine protein/urine creatinine ratio or urine albumin/urine creatinine ratio) for the evaluation of renal involvement in patients with IgA vasculitis. B Abdominal ultrasound: obtain abdominal ultrasound by a specialist with pediatric expertise to exclude intestinal intussusception in patients with severe abdominal pain. B 3. Diagnostic procedures Skin biopsy: perform skin biopsy with specific immunofluorescence stating for IgA in patients with atypical rash and/or to exclude alternative diagnosis. B Show 2 more Kidney biopsy: Perform renal biopsy in patients with IgA vasculitis with: severe proteinuria (> 250 mg/mmol for at least 4 weeks) persistent moderate proteinuria (100-250 mg/mmol) impaired GFR. B Consider performing renal biopsy in patients with IgA vasculitis with a shorter duration of severe proteinuria (> 250 mg/mmol for < 4 weeks). C 4. Medical management Corticosteroids: Initiate corticosteroids in patients with IgA vasculitis for the treatment of: orchitis cerebral vasculitis pulmonary hemorrhage other severe organ- or life-threatening vasculitis manifestations. B Show 3 more Analgesics: Prescribe adequate analgesia in patients with IgA vasculitis-associated arthropathy or abdominal pain. B Recognize that NSAIDs are not contraindicated if the renal function is normal. B 5. Specific circumstances https://web.pathway.md/diseases/reciVsOBWLsaejtOa 2/4 6/24/23, 12:31 PM Henoch-Sch nlein purpura Pathway Patients with IgA nephritis, ACEIs and ARBs: As per SHARE initiative 2019 guidelines, consider administering ACEIs to prevent/limit secondary glomerular injury in patients with IgA nephritis with persistent proteinuria. C As per KDIGO 2012 guidelines, consider administering ACEIs or ARBs in patients with HSP nephritis and persistent proteinuria > 0.5-1 g/day/1.73 m . C Patients with IgA nephritis, corticosteroids: As per SHARE initiative 2019 guidelines, administer oral prednisone as first-line therapy in patients with mild IgA nephritis. B Show 2 more As per KDIGO 2012 guidelines, consider administering a 6-month course of corticosteroid therapy in patients with HSP nephritis with GFR > 50 ml/min/1.73 m and persistent proteinuria > 1 g/day after a trial of ACEIs or ARBs. C Patients with IgA nephritis (immunosuppressants): consider administering azathioprine or mycophenolate mofetil as second-line therapy in patients with IgA nephritis following renal biopsy. C Show 2 more Patients with IgA nephritis (combination therapy): Administer IV cyclophosphamide with pulsed methylprednisolone and/or oral prednisolone as first-line therapy in patients with severe IgA nephritis. B Consider administering azathioprine or mycophenolate mofetil in combination with corticosteroids as maintenance therapy in patients with severe IgA nephritis. C Patients with crescentic nephritis: consider treating patients with crescentic HSP and nephrotic syndrome and/or deteriorating kidney function using the same treatment regimens as for crescentic IgA nephropathy. C 6. Preventative measures Prevention of IgA nephritis: As per SHARE initiative 2019 guidelines, do not administer prophylactic corticosteroids to prevent the development of IgA vasculitis-associated nephritis. D As per KDIGO 2012 guidelines, do not administer corticosteroids for the prevention of HSP nephritis. D 7. Follow-up and surveillance Indications for specialist consultation: Consult with a pediatric nephrologist: if the patient with IgA vasculitis has moderate proteinuria and/or impaired GFR when starting treatment for IgA nephritis. B https://web.pathway.md/diseases/reciVsOBWLsaejtOa 3/4 6/24/23, 12:31 PM Henoch-Sch nlein purpura Pathway References 1. KDIGO. Chapter 11 Henoch-Sch nlein purpura nephritis. Kidney Int Suppl 2011 . 2012 Jun;2 2 218 220. Open 2. Seza Ozen, Stephen D Marks, Paul Brogan et al. European consensus-based recommendations for diagnosis and treatment of immunoglobulin A vasculitis-the SHARE initiative. Rheumatology Oxford). 2019 Sep 1;58 9 1607 1616. Open 3. Priyank Yagnik, Apurva Jain, Jason K Amponsah et al. National Trends in the Epidemiology and Resource Use for Henoch-Sch nlein Purpura IgA Vasculitis) Hospitalizations in the United States From 2006 to 2014. Hosp Pediatr. 2019 Nov;9 11 888 896. Open 4. Louise Oni, Sunil Sampath. Childhood IgA Vasculitis Henoch Schonlein Purpura)-Advances and Knowledge Gaps. Front Pediatr. 2019 Jun 27;7 257. Open 5. Alexander Tracy, Anuradhaa Subramanian, Nicola J Adderley et al. Cardiovascular, thromboembolic and renal outcomes in IgA vasculitis Henoch-Sch nlein purpura): a retrospective cohort study using routinely collected primary care data. Ann Rheum Dis. 2019 Feb;78 2 261 269. Open 6. Seza Ozen, Angela Pistorio, Silvia M Iusan et al. EULAR/PRINTO/PRES criteria for Henoch-Sch nlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II Final classification criteria. Ann Rheum Dis. 2010 May;69 5 798 806. Open 7. Anand Padmanabhan, Laura Connelly-Smith, Nicole Aqui et al. Guidelines on the Use of Therapeutic Apheresis in Clinical Practice Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Eighth Special Issue. J Clin Apher. 2019 Jun;34 3 171 354. Open https://web.pathway.md/diseases/reciVsOBWLsaejtOa 4/4