What is the efficacy of drug treatment for Henoch-Schönlein purpura (IgA vasculitis)?

Updated: Oct 28, 2020
  • 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

Of those, only cyclophosphamide has been shown to be effective in a randomized controlled trial. Although some studies have reported success, cyclosporine does not have sufficient clinical data to establish its utility in this setting. [106]

Azathioprine, mycophenolate mofetil, and urokinase must be tested before their use is consistently advocated. Guidelines for prescribing azathioprine in dermatology have been established. [107] No convincing studies have yet been conducted regarding the use of IVIg, factor XIII administration, antioxidant vitamin E, and fish oil to treat IgAV. [108]

A randomized clinical trial comparing cyclosporine with methylprednisolone pulses in IgAV with nephritis found that cyclosporine had superior efficacy and many fewer complications. [109] A study of 12 patients with severe IgAV nephritis indicated that patients did well with methylprednisolone at 30 mg/kg/day for 3 days followed by oral corticosteroids at 2 mg/kg/day for 2 months, cyclophosphamide at 2 mg/kg/day for 2 months, and dipyridamole at 5 mg/kg/day for 6 months. [110]

Some have noted that parvovirus B19–associated IgAV must be recognized in adults because the treatment of choice is IV gamma globulin combined with tumor necrosis factor (TNF)–α inhibitor therapy. Immunosuppressive therapy may lead to a persistent or worsening disease course in these patients.

Massive GI hemorrhage in isolated intestinal IgAV that is responsive to IVIg infusion has been reported. [111] IVIg was used in a complex case of IgAV with brain hemorrhage, but more work must be done to validate the use of this treatment. [112]

Dapsone has been used to treat associated purpuras and arthralgias. Iqbal and Evans found it to be effective for IgAV. [113]

Rituximab has been noted to be a successful treatment for severe refractory chronic IgAV. [114]

Factor VIII concentrate has been used to relieve abdominal pain when corticosteroids are contraindicated. Recombinant factor VIIa has been used in patients with very pronounced factor XIII deficiency and compartment syndrome.

Treatment of complicated IgAV with mycophenolate mofetil has been reported in a series of patients. [115, 116]


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