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Life-threatening or organ-impairing Henoch-Schönlein purpura: plasmapheresis may save lives and limit organ damage


Donghi, D; Schanz, U; Sahrbacher, U; Recher, M; Trüeb, R M; French, L E; Hafner, J (2009). Life-threatening or organ-impairing Henoch-Schönlein purpura: plasmapheresis may save lives and limit organ damage. Dermatology, 219(2):167-170.

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.

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.

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Additional indexing

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Clinic for Hematology
04 Faculty of Medicine > University Hospital Zurich > Clinic for Gastroenterology and Hepatology
04 Faculty of Medicine > University Hospital Zurich > Dermatology Clinic
Dewey Decimal Classification:610 Medicine & health
Language:English
Date:2 June 2009
Deposited On:25 Jan 2010 14:11
Last Modified:20 Feb 2018 13:59
Publisher:Karger
ISSN:1018-8665
Additional Information:© 2010 S. Karger AG
OA Status:Green
Free access at:Publisher DOI. An embargo period may apply.
Publisher DOI:https://doi.org/10.1159/000223237
PubMed ID:19494483

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