EULAR Recommendations for Management of Henoch-Schönlein Purpura

MADRID — Despite being the most common form of childhood vasculitis, there is a poor base of evidence available to guide clinical management of Henoch-Schönlein purpura. Updated interdisciplinary treatment recommendations and guidelines for management were presented at the 2019 European League Against Rheumatism (EULAR) Congress, held June 12-15, in Madrid, Spain.

Following a systematic meta-analysis of available literature, researchers graded evidence and recommendations and created a treatment algorithm. Treatments were tailored to each patient’s individual pathology and disease severity.

The recommendations are summarized below:

Joint symptoms: Bed rest and nonsteroidal anti-inflammatory drugs; avoid nonsteroidal anti-inflammatory drugs if renal disease is present. Steroids may be used in severe cases.

Abdominal pain: Abdominal pain typically settles within 72 hours of onset. Simple analgesia is adequate, including 5 to 10 mg/kg ibuprofen, 3 times/day with or without the addition of 10 to 15 mg/kg once-daily paracetamol. If abdominal pain is severe, the use of steroids (2 mg/kg daily) may be appropriate. If abdominal pain is acute, severe, and/or associated with bloody stool, consider intussusception and seek a surgical opinion.

Skin manifestations: Skin manifestations are typically benign and self-limited. Severe cases may be treated with colchicine or dapsone.

Henoch-Schönlein purpura nephritis in children: Children with Henoch-Schönlein purpura nephritis and persistent proteinuria (0.5 g/d to 1 g/d) should be treated with an angiotensin-converting enzyme inhibitor or an angiotensin II receptor blocker. Following treatment with either an angiotensin-converting enzyme inhibitor or an angiotensin II receptor blocker, if patients still present with persistent proteinuria (>1 g/d) and glomerular filtration rate of >50 mL/minute per 1.73 m², they should be treated with a 6-month course of corticosteroid therapy.

Crescentic Henoch-Schönlein purpura nephritis in children: For children with crescentic Henoch-Schönlein purpura nephritis, clinicians should consider treatment with pulse steroids, cyclophosphamide, and azathioprine. For children resistant to first-line therapy, cyclosporine A may be an effective treatment. In steroid-resistant cases, clinicians may consider mycophenolate mofetil; in resistant cases, plasmapheresis and intravenous immunoglobulin may be considered. The safety of rituximab has not been established in this patient population.

Reference

Abu-Zaid MH, El Miedany Y, Hassan W, et al. Updates on the evidence based interdisciplinary guidelines for Henock-Schönlein purpura. Presented at: European League Against Rheumatism (EULAR) Congress 2019; June 12-15, 2019; Madrid, Spain. Abstract AB0922.