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Henoch Schönlein Purpura — Observations from a Large Cohort in Northwest Spain

Summary and Comment |
May 15, 2014

Henoch Schönlein Purpura — Observations from a Large Cohort in Northwest Spain

  1. Jeffrey P. Callen, MD

Characteristics and outcome after long-term follow-up

  1. Jeffrey P. Callen, MD

Henoch-Schönlein purpura (HSP), a small vessel vasculitis, is characterized by palpable purpura, nephropathy, arthritis, and gastrointestinal (GI) bleeding or colicky pain. More frequent in children, it also occurs in adults. Association with IgA deposition in tissue has been suggested but is not included among criteria for diagnosis, nor do authorities classify IgA deposition with cutaneous vasculitis as HSP, absent other findings.

These authors conducted long-term follow-up of a large cohort of patients with vasculitis. Included were 417 patients classified as having HSP per American College of Rheumatology (ACR) criteria. Three of the following criteria will lead to correct diagnosis of HSP 87.1% of the time: palpable purpura, bowel angina, gastrointestinal bleeding, hematuria, age younger than 20 years, and absence of medication-related etiology.

Of the 417 patients, 315 were children, and 240 were male. The median age was 7.5 years (range, 8 months to 87 years). A drug-related cause was possible in 77 patients (18.5%). Five had hepatitis (4, hepatitis B; 1, hepatitis C). Initial manifestations included palpable purpura (55.9%), nephropathy (24%), GI involvement (13.7%), arthritis/arthralgia (9.1%), and fever (6.2%). Developing manifestations involved skin (100%), GI (64.5%), joints (63.1%), and nephropathy (41.2%; mild microscopic hematuria or proteinuria below the nephrotic range in most). The only other notable laboratory finding was elevated serum IgA in roughly one third of patients.

Only half of the patients received disease-modifying agents. Outcome was generally favorable. By 12 months after onset, disease had resolved in 83.2% of patients. About one third experienced relapse. Renal involvement occurred in 32 patients (7.7%), but renal insufficiency occurred only in 6.

Comment

Results of this largest published study of HSP are not surprising. Limitations include use of the ACR criteria for diagnosis, the retrospective analysis, and that not all patients had a skin biopsy. The latter flaw is common, as children with vasculitis are not often biopsied.

  • Disclosures for Jeffrey P. Callen, MD at time of publication Consultant / Advisory board XOMA; Eli Lilly Equity Various trust accounts Editorial boards JAMA Dermatology; UpToDate; Journal of Rheumatology; Psoriasis Forum; Journal of Drugs in Dermatology; eMedicine

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