Life-threatening or organ-impairing Henoch-Schönlein purpura: plasmapheresis may save lives and limit organ damage.
Adult-onset Henoch-Schonlein purpura (HSP) tends to become chronic-relapsing, yet rarely leads to organ impairment, e.g. due to chronic glomerulonephritis. Bed rest, compression and nonsteroidal anti-inflammatory drugs are usually sufficient to control the active phases. We report 2 cases of adult HSP with an unusually severe evolution. One patient required intensive-care treatment for hypovolemic shock caused by hemorrhagic pancolitis; the other had progressive and extremely extensive vasculitic leg ulcers. Both were refractory to common immunosuppression with systemic corticosteroids (oral and pulse) and additive steroid-sparing immunosuppressive drugs. Only after the introduction of plasmapheresis did both patients show a dramatic improvement in the disease, with rapid and almost complete healing. Plasmapheresis is a rarely used therapeutic tool in the treatment of severe HSP, but the growing literature on its highly beneficial effect underlines its potential usefulness.
Donghi
Davide
D
Department of Dermatology, University Hospital of Zürich, Zürich, Switzerland. davide.donghi@usz.ch
Schanz
Urs
U
Sahrbacher
Ulrike
U
Recher
Mike
M
Trüeb
Ralph M
RM
Müllhaupt
Beat
B
French
Lars E
LE
Hafner
Jürg
J
D000328:Adult; D016638:Critical Illness / Q000628:therapy; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D005921:Glomerulonephritis / Q000209:etiology / Q000517:prevention & control*; D006801:Humans; D011695:IgA Vasculitis / Q000150:complications / Q000175:diagnosis* / Q000628:therapy*; D007871:Leg Ulcer / Q000175:diagnosis* / Q000628:therapy*; D010956:Plasmapheresis / Q000379:methods*; D018570:Risk Assessment; D016896:Treatment Outcome; D014945:Wound Healing