What are treatment options for Henoch-Schönlein purpura (IgA vasculitis)?

Updated: Jan 08, 2021
  • Author: Rajendra Bhimma, MBChB, MD, PhD, DCH (SA), FCP(Paeds)(SA), MMed(Natal); Chief Editor: Craig B Langman, MD  more...
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Answer

To date, no form of therapy has been found to shorten the duration of IgA vasculitis (IgAV; Henoch-Schönlein purpura) to any significant degree. Therefore, treatment remains primarily supportive in most cases. This is consonant with the understanding that IgAV is a self-limited disease. The majority of patients recover quickly (ie, within several weeks) without treatment. [98]

Management of IgAV includes adequate hydration; immediate discontinuation of any exposure to antigenic stimulants (eg, drugs); and follow-up each week for the first month, every other week for the second month, and monthly thereafter until abnormal urinary findings subside.

Hospital admission and monitoring for complications should be considered. Hospitalization should be strongly considered for IgAV patients with severe abdominal pain, significant gastrointestinal (GI) bleeding, or marked kidney insufficiency. Complications such as acute abdomen, acute scrotum, and acute kidney injury may be severe and may occur precipitously.

IgAV may mimic an abdominal emergency and, in its most severe form, result in small-bowel infarction, perforation, or both.

In adults with IgAV, permanent kidney involvement is not uncommon. All pregnant women with even mild kidney symptoms at the onset of IgAV should be carefully observed during and after pregnancy. Hematuria at disease onset and persistence of kidney manifestations during the course of IgAV are significant predictors of possible development of kidney sequelae. Other features associated with the development of kidney sequelae include the following:

  • Onset in summer
  • Anemia at disease onset
  • Relapse 

Nephropathy is treated supportively, with monitoring of fluid and electrolyte balance, restriction of salt intake, and administration of antihypertensives when needed. Various drugs (eg, corticosteroids, azathioprine, and cyclophosphamide) and plasmapheresis have been used to prevent progression of kidney disease, but the results have been inconsistent, and no data from controlled studies are available.

Dietary restrictions have no clear role in the management of IgAV. Activities can be performed as tolerated.


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