Henoch-Schönlein Purpura (IgA Vasculitis): Rapid Evidence Review

 

Am Fam Physician. 2020 Aug 15;102(4):229-233.

  Patient information: A handout on this topic is available at https://familydoctor.org/condition/henoch-schonlein-purpura/.

Author disclosure: No relevant financial affiliations.

Henoch-Schönlein purpura, now called immunoglobulin A (IgA) vasculitis, is a systemic, immune complex–mediated, small-vessel leukocytoclastic vasculitis characterized by nonthrombocytopenic palpable purpura, arthritis, and abdominal pain. It is the most common vasculitis in children but can also occur in adults. 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. However, a subset of patients experience renal involvement that can persist and relapse years later. Additional complications can include gastrointestinal bleeding, orchitis, and central nervous system involvement. 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. Six months of follow-up is prudent to assess for disease relapse or remission.

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.

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SORT: KEY RECOMMENDATIONS FOR PRACTICE

Clinical recommendationEvidence ratingComment

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.3032

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


IgA = immunoglobulin A.

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.

SORT: KEY RECOMMENDATIONS FOR PRACTICE

Clinical recommendationEvidence ratingComment

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.3032

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


IgA = immunoglobulin A.

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.

Epidemiology

  • IgA vasculitis occurs in 3.0 to 26.7 out of 100,000 children and 0.8 to 1.8 out of 100,000 adults each year.1

  • The mean age of onset in children is six years; onset ranged from 32 to 50 years of age in several adult case series.14 More than

The Authors

show all author info

BRIAN V. REAMY, MD, FAAFP, is a professor in the Department of Family Medicine and the senior associate dean for academic affairs at the Uniformed Services University of the Health Sciences, Bethesda, Md....

JESSICA T. SERVEY, MD, MHPE, FAAFP, is an associate professor in the Department of Family Medicine and the associate dean for faculty development at the Uniformed Services University of the Health Sciences.

PAMELA M. WILLIAMS, MD, FAAFP, is an associate professor in the Department of Family Medicine and the associate dean for student affairs at the Uniformed Services University of the Health Sciences.

Address correspondence to Brian V. Reamy, MD, Uniformed Services University of the Health Sciences, 4301 Jones Bridge Rd., Room D3013, Bethesda, MD 21037 (email: brian.reamy@usuhs.edu). Reprints are not available from the authors.

Author disclosure: No relevant financial affiliations.

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show all references

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