The Bottom Line: “Diagnostic testing is required only to exclude other etiologies of purpura, to identify renal involvement, and, if indicated, to determine its extent with biopsy. Imaging or endoscopy may be needed to assess organ complications. IgA vasculitis spontaneously resolves in 94% of children and 89% of adults, making supportive treatment the primary management strategy… Systematic reviews have shown that steroids do not prevent complications and should not be used prophylactically. However, randomized trials have demonstrated success with high-dose steroids, cyclosporine, and mycophenolate in treating glomerulonephritis and other complications. Long-term prognosis depends on the extent of renal involvement.” (Reamy)
“Immunoglobulin A (IgA) vasculitis, formerly known as Henoch-Schönlein purpura, is defined as a systemic, immune complex–mediated, small-vessel leukocytoclastic vasculitis characterized by nonthrombocytopenic palpable purpura, abdominal pain, and arthritis. It is the most common vasculitis in children. IgA vasculitis is typically self-limited, but a subset of patients experience a remitting-relapsing course. Glomerulonephritis and gastrointestinal bleeding are the most commonly associated complications.” (Reamy)
Diagnostic Criteria for (IgA) vasculitis from the European League Against Rheumatism/Paediatric Rheumatology International Trials Organisation/Paediatric Rheumatology European Society (EULAR/PRINTO/PRES):
Recommendations for practice with evidence from Reamy et al. 2020 Review:
| Clinical recommendation | Evidence rating | Comment |
| Suspect IgA vasculitis in patients with palpable purpura, arthralgias, and abdominal pain.The initial diagnostic evaluation should include a complete blood count, a coagulation and metabolic profile, urinalysis, and a serum albumin test.13,14,19 | C | Expert opinion and literature review |
| Do not prescribe prednisone or antiplatelet agents to prevent renal disease.33 | A | Systematic review of patient-oriented evidence |
| Early treatment with prednisone reduces the intensity of joint and abdominal pain in children.30–32 | B | High-quality blinded, randomized trials |
| Monthly urinalysis, creatinine, and blood pressure screening should be performed for six months after diagnosis to monitor for relapse and poor renal outcomes.23,25 | C | Meta-analysis and five-year retrospective review |
| Consider evaluation for lung, kidney, and prostate cancers in patients older than 60 years who have IgA vasculitis.47 | C | Literature review and expert opinion |
A = consistent, good-quality patient-oriented evidence; B = inconsistent or limited-quality patient-oriented evidence; C = consensus, disease-oriented evidence, usual practice, expert opinion, or case series. For information about the SORT evidence rating system, go to https://www.aafp.org/afpsort.
Reamy, Brian V et al. “Henoch-Schönlein Purpura (IgA Vasculitis): Rapid Evidence Review.” American family physician vol. 102,4 (2020): 229-233. Free Full Text at AFP.
DynaMed “Henoch-Schonlein Purpura: Diagnosis” EBSCO Information Services. Accessed May 21, 2024.
The Bottom Line 