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PubMed This is a summary of 54 peer-reviewed journal articles Updated
Rheumatology

Immunoglobulin A Vasculitis (IgAV / HSP): Your Complete Guide

At a Glance

Immunoglobulin A Vasculitis (IgAV), formerly Henoch-Schönlein Purpura (HSP), causes inflammation in small blood vessels and a distinct purple rash. While it often resolves on its own in children, adults face higher risks. Long-term kidney monitoring via urinalysis is essential for all patients.

Welcome to your comprehensive guide on Immunoglobulin A Vasculitis (IgAV), formerly known as Henoch-Schönlein Purpura (HSP).

Hearing that you or your child has a condition with a long, complicated name like “vasculitis” can be terrifying. It is entirely normal to feel overwhelmed, especially when the hallmark of the disease—a sudden, dramatic purple rash—looks so alarming. But take a deep breath: this condition is highly studied, and the medical community has very clear guidelines on how to manage it.

IgAV is a condition where a specific immune system protein (IgA) accidentally builds up in the small blood vessels, causing them to become inflamed and leak [1]. While it often resolves completely on its own in children [2], adults require much closer monitoring due to a higher risk of complications [3].

This guide is designed to help you navigate every step of the journey, from understanding the initial diagnosis to managing the long-term monitoring required to keep your kidneys safe. It translates complex immunological concepts into plain language so you can actively participate in your care.

Navigating This Guide

01

Understanding Your Diagnosis: An Introduction to IgAV

Learn about Immunoglobulin A Vasculitis (IgAV), formerly known as HSP. Understand the classic tetrad of symptoms, kidney monitoring, and what to expect.

02

Recognizing Symptoms and Warning Signs

Learn to recognize the classic symptoms of Immunoglobulin A Vasculitis (IgAV). Understand palpable purpura, joint pain, kidney issues, and emergency red flags.

03

Biology, Pathology, and Finding the Right Diagnosis

Understand the biology of Immunoglobulin A Vasculitis (IgAV). Learn when a biopsy is needed, how to read your pathology report, and how mimics are ruled out.

04

Managing IgAV: Treatment Strategies and Guidelines

Learn about treatment options for Immunoglobulin A Vasculitis (IgAV). Understand supportive care, when steroids are used, and why you should avoid NSAIDs.

05

The Road to Recovery: Renal Monitoring and Long-Term Health

Learn about the long-term recovery process for Immunoglobulin A Vasculitis (IgAV), including the 6-12 month kidney monitoring schedule and recurrence risks.

Common questions in this guide

What is the difference between IgAV and HSP?
There is no difference. Immunoglobulin A Vasculitis (IgAV) is simply the updated medical name for the condition that was formerly known as Henoch-Schönlein Purpura (HSP).
Why are urine tests necessary after the IgAV rash goes away?
Even after the visible rash completely fades, IgAV can cause silent inflammation in your kidneys. Long-term urinalysis monitoring is critical to detect and protect against potential kidney damage early on.
Is IgAV treated differently in adults than in children?
Yes. While IgAV often resolves completely on its own in children, adults have a higher risk of severe complications and require much closer monitoring by their healthcare team.
What specialists should be on an IgAV care team?
Because IgAV involves blood vessel inflammation and possible kidney complications, your care team should typically include a rheumatologist (an expert in inflammatory conditions) and a nephrologist (a kidney specialist).

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Which specialists (e.g., Rheumatologist, Nephrologist) should be on my (or my child's) care team?
  2. 2.Will you coordinate the long-term urinalysis monitoring, or should we see a specialist for that?
  3. 3.Does our current care plan follow the European SHARE guidelines for IgAV management?

Questions For You

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References

References (3)
  1. 1

    Predictive role of laboratory markers and clinical features for recurrent Henoch-Schönlein Purpura in childhood: A study from Turkey.

    Gökçe Ş, Kurugöl Z, Koturoğlu G, Aslan A

    Modern rheumatology 2020; (30(6)):1047-1052 doi:10.1080/14397595.2019.1690966.

    PMID: 31711347
  2. 2

    Henoch-Schönlein Purpura in Children: An Updated Review.

    Leung AKC, Barankin B, Leong KF

    Current pediatric reviews 2020; (16(4)):265-276 doi:10.2174/1573396316666200508104708.

    PMID: 32384035
  3. 3

    Clinical Characteristics of Biopsy-Proven IgA Vasculitis in Children and Adults: A Retrospective Cohort Study.

    Villatoro-Villar M, Crowson CS, Warrington KJ, et al.

    Mayo Clinic proceedings 2019; (94(9)):1769-1780 doi:10.1016/j.mayocp.2019.04.034.

    PMID: 31486380

This guide provides educational information about Immunoglobulin A Vasculitis (IgAV/HSP) and does not replace professional medical advice. Always consult your rheumatologist or nephrologist about your specific treatment and kidney monitoring plan.

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