title: Life-threatening or organ-impairing Henoch-Schönlein purpura: plasmapheresis may save lives and limit organ damage.
abstract: 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.
authors.affiliation: Department of Dermatology, University Hospital of Zürich, Zürich, Switzerland. davide.donghi@usz.ch
authors.forename: Davide
authors.initials: D
authors.lastname: Donghi
authors.forename: Urs
authors.initials: U
authors.lastname: Schanz
authors.forename: Ulrike
authors.initials: U
authors.lastname: Sahrbacher
authors.forename: Mike
authors.initials: M
authors.lastname: Recher
authors.forename: Ralph M
authors.initials: RM
authors.lastname: Trüeb
authors.forename: Beat
authors.initials: B
authors.lastname: Müllhaupt
authors.forename: Lars E
authors.initials: LE
authors.lastname: French
authors.forename: Jürg
authors.initials: J
authors.lastname: Hafner
mesh_terms: 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