IgA vasculitis (Henoch-Schonlein purpura)
Summary
Definition
History and exam
Key diagnostic factors
- rash
- arthralgias
- abdominal pain
- signs of renal disease
Other diagnostic factors
- drug history
- scrotal pain or swelling
- headaches
- seizures
- pulmonary hemorrhage
Risk factors
- genetics
- allergy
- male sex
- age 2 to 10 years
- increasing age at onset
- winter season/prior infection
- medication
Diagnostic investigations
1st investigations to order
- blood pressure
- urinalysis
- serum creatinine and electrolyte levels
Investigations to consider
- serum IgA levels
- CBC and coagulation studies
- skin biopsy
- renal biopsy
- ultrasound, abdomen
- ultrasound, testicles
Treatment algorithm
without renal involvement
mild nephritis: normal GFR and mild or moderate proteinuria
moderate nephritis: <50% crescents on renal biopsy and impaired GFR or severe persistent proteinuria
severe nephritis: >50% crescents on renal biopsy and impaired GFR or severe persistent proteinuria
relapsed/refractory disease
Contributors
Authors
Louise Oni, MBChB, MRCPCH, MA, PhD
Senior Lecturer in Paediatric Nephrology and Honorary Consultant Paediatric Nephrologist
University of Liverpool
Department of Paediatric Nephrology
Alder Hey Children's NHS Foundation Trust Hospital
Liverpool
UK
Disclosures
In the past 12 months LO has contributed to expert advisory roles for Boehringer Ingelheim and Biocryst pharmaceuticals. LO has received a travel grant from Sandoz. These are unrelated to this article and all monetary payments are placed into an institutional educational charity account. LO is an author of references cited in this topic.
Peer reviewers
David Kershaw, MD
Professor of Pediatrics
University of Michigan
Ann Arbor
MI
Disclosures
DK is co-medical director for the board of directors of Nephcure.
Rajan Madhok, MD, FCRP
Consultant Physician and Rheumatologist
Centre for Rheumatic Diseases
Glasgow Royal Infirmary
Glasgow
UK
Disclosures
RM declares that he has no competing interests.
Richard A. Watts, MA, DM, FRCP
Consultant Rheumatologist
Ipswich Hospital NHS Trust
Ipswich
UK
Divulgaciones
RAW declares that he has no competing interests.
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Referencias
Artículos principales
Ozen S, Marks SD, Brogan P, et al. European consensus-based recommendations for diagnosis and treatment of immunoglobulin A vasculitis - the SHARE initiative. Rheumatology (Oxford). 2019 Sep 1;58(9):1607-16.Texto completo Resumen
UK Kidney Association. The initial management of IgA vasculitis (Henoch Schönlein Purpura) in children and young people. Dec 2022 [internet publication].Texto completo
UK Kidney Association. The management of complications associated with IgA vasculitis (Henoch Schönlein Purpura) in children and young people. Dec 2022 [internet publication].Texto completo
Hahn D, Hodson EM, Craig JC. Interventions for preventing and treating kidney disease in IgA vasculitis. Cochrane Database Syst Rev. 2023 Feb 28;(2):CD005128.Texto completo Resumen
Kidney Disease: Improving Global Outcomes (KDIGO) Glomerular Diseases Work Group. KDIGO 2021 clinical practice guideline for the management of glomerular diseases. Kidney Int. 2021 Oct;100(4S):S1-276.Texto completo
Artículos de referencia
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