What is Henoch-Schönlein purpura (HSP)?

From the Guidelines

Henoch-Schönlein purpura (HSP) is a form of vasculitis that affects blood vessels and is characterized by the deposition of immune complexes containing IgA antibodies, leading to inflammation and damage to various organs, including the skin, kidneys, and gastrointestinal tract.

Key Characteristics of HSP

• HSP can present with a range of symptoms, including purpura (bleeding under the skin), arthritis, abdominal pain, and renal involvement 1.
• The disease can affect both children and adults, but the prognosis and treatment approach may differ between the two groups 1.
• Renal involvement in HSP can range from mild proteinuria to nephrotic syndrome or nephritic syndrome, and may require treatment with ACE inhibitors, ARBs, or corticosteroids 1.

Treatment Approach

• The treatment approach for HSP depends on the severity of the disease and the presence of renal involvement 1.
• Children with HSP and persistent proteinuria may be treated with ACE inhibitors or ARBs 1.
• Adults with HSP may be treated similarly to children, but the long-term benefit of steroid therapy for nephrotic-range or persistent proteinuria has not been proven 1.

Ongoing Research and Controversies

• There is a need for randomized controlled trials to determine the effectiveness of different treatment approaches for HSP, including the use of noncorticosteroid immunosuppressive agents such as CNIs or MMF 1.
• The optimal duration of steroid therapy for children with moderately severe HSP nephritis is also unclear and requires further study 1.

From the Research

Definition and Overview of Henoch-Schönlein Purpura (HSP)

• Henoch-Schönlein purpura (HSP) is an acute, self-limited small-vessel vasculitis commonly affecting children aged 3-10 2.
• It is also known as anaphylactoid purpura, leukocytoclastic vasculitis, allergic vasculitis, and rarely, as rheumatoid purpura 3.
• HSP is an IgA-mediated systemic small-vessel vasculitis with a predilection for the skin, gastrointestinal tract, joints, and kidneys 4.

Clinical Manifestations of HSP

• The classic tetrad of HSP includes palpable non-thrombocytopenic purpura, abdominal pain, polyarthralgia, and renal involvement 2.
• The diagnosis should be based on the finding of palpable purpura in the presence of at least one of the following criteria, namely, diffuse abdominal pain, arthritis or arthralgia, renal involvement (hematuria and/or proteinuria), and a biopsy showing predominant IgA deposition 4.
• Urological manifestations of HSP involve the kidney, ureter, bladder, prostate, scrotum, testicle, and penis 5.

Incidence and Prognosis of HSP

• The incidence of HSP is 10 to 20 cases per 100,000 children per year, with approximately 90% of cases occurring in children between 2 and 10 years of age 4.
• Most cases are self-limited, with an average duration of the disease being 4 weeks 4.
• Long-term complications are rare and include persistent hypertension and end-stage kidney disease 4.
• The long-term prognosis of HSP is directly dependent on the severity of renal involvement 6.

Treatment and Management of HSP

• Therapy consists of general and supportive measures as well as treatment of the sequelae of the vasculitis 4.
• Current evidence does not support the universal treatment of HSP patients with corticosteroids 4.
• Oral corticosteroids may be considered for HSP patients with severe gastrointestinal pain and gastrointestinal hemorrhage 4.
• Treatment options vary from conservative to invasive depending on the severity of the disease and time frame of diagnosis 5.