🔬 Other

Henoch-Schönlein Purpura

Also known as: IgA Vasculitis, Anaphylactoid Purpura

Henoch-Schönlein Purpura (HSP), also known as IgA vasculitis, is a systemic small vessel vasculitis characterized by deposition of IgA-containing immune complexes in small vessels, leading to inflammation. It primarily affects the skin, intestines, kidneys, and joints. HSP is more common in children but can occur at any age. The exact cause is unknown, but it often follows an upper respiratory infection.

ICD-10: D69.0 Orphanet: 778 OMIM: 141000
👥 3-26.7 per 100,000 children Prevalence
🔬 25 Active Trials

Imagine your body's defense system is a bit confused and starts attacking tiny blood vessels, especially in your skin, tummy, kidneys, and joints. This causes a rash with small, raised spots, tummy aches, sore joints, and sometimes problems with your pee. It usually gets better on its own, but doctors might give you medicine to help you feel better.

Signs & Symptoms

  • Palpable purpura (typically on buttocks and lower extremities)
  • Arthritis or arthralgia (joint pain), especially in knees and ankles
  • Abdominal pain (colicky, may be severe)
  • Gastrointestinal bleeding
  • Kidney involvement (hematuria, proteinuria)
  • Edema (swelling), especially of the scalp, hands, and feet

Treatment Options

SUPPORTIVE

Supportive Care

SUPPORTIVE
MEDICATION FDA Approved

Pain Management (NSAIDs)

MODERATELY EFFECTIVE
MEDICATION FDA Approved

Corticosteroids (e.g., Prednisone)

MODERATELY EFFECTIVE
MEDICATION FDA Approved

Immunosuppressants (e.g., Azathioprine, Cyclophosphamide)

MODERATELY EFFECTIVE
MEDICATION FDA Approved

Angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs)

MODERATELY EFFECTIVE
MEDICATION FDA Approved

Rituximab

MODERATELY EFFECTIVE

Diagnosis

  • Clinical evaluation (based on characteristic symptoms)
  • Skin biopsy (to confirm IgA deposition in blood vessels)
  • Urinalysis (to assess kidney involvement)
  • Blood tests (CBC, renal function tests, coagulation studies)
  • Stool occult blood test
  • Kidney biopsy (if kidney involvement is severe or atypical)

History

Henoch-Schönlein Purpura was first described by Johann Schönlein in 1837, who noted the association of purpura and joint pain. Eduard Henoch, his student, later described the abdominal symptoms and renal involvement in 1874, leading to the eponymous name.

Recent Breakthroughs

2022

Biomarkers for Predicting Renal Involvement in Henoch-Schönlein Purpura

Research has identified potential biomarkers, such as specific cytokines and chemokines, that may predict the risk of renal involvement in children with HSP, allowing for earlier intervention.

2023

Efficacy of Rituximab in Severe Refractory Henoch-Schönlein Purpura

A study demonstrated the potential efficacy of rituximab, an anti-CD20 monoclonal antibody, in patients with severe, refractory HSP who have failed conventional therapies.