Adult-onset Henoch-Schönlein 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.

Keywords:
Henoch-Schönlein purpura, Pancolitis, Plasmapheresis, Immunosuppression, Leg ulcers, extensive
1.
Roberts PF, Waller TA, Brinker TM, Riffe IZ, Sayre JW, Bratton RL: Henoch-Schönlein purpura: a review article. South Med J 2007;100:821–824.
2.
Saulsbury FT: Epidemiology of Henoch-Schönlein purpura. Cleve Clin J Med 2002;69:SII87–SII89.
3.
Zurada JM, Ward KM, Grossman ME: Henoch-Schönlein purpura associated with malignancy in adults. J Am Acad Dermatol 2006;55(suppl 5):S65–S70.
4.
Dosa S, Cairns SA, Mallick NP, Lawler W, Williams G: Relapsing Henoch-Schönlein syndrome with renal involvement in a patient with an IgA monoclonal gammopathy: a study of the results of immunosuppressant and cytotoxic therapy. Nephron 1980;26:145–148.
5.
Zickerman AM, Allen AC, Talwar V, Olczak SA, Brownlee A, Holland M, Furness PN, Brunskill NJ, Feehally J: IgA myeloma presenting as Henoch-Schönlein purpura with nephritis. Am J Kidney Dis 2000;36:E19.
6.
Van Der Helm-Van Mil AH, Smith AC, Pouria S, Tarelli E, Brunskill NJ, Eikenboom HC: Immunoglobulin A multiple myeloma presenting with Henoch-Schönlein purpura associated with reduced sialylation of IgA1. Br J Haematol 2003;122:915–917.
7.
Birchmore D, Sweeney C, Choudhury D, Konwinski MF, Carnevale K, D’Agati V: IgA multiple myeloma presenting as Henoch-Schönlein purpura/polyarteritis nodosa overlap syndrome. Arthritis Rheum 1996;39:698–703.
8.
Kawasaki Y, Suzuki J, Murai M, Takahashi A, Isome M, Nozawa R, Suzuki S, Suzuki H: Plasmapheresis therapy for rapidly progressive Henoch-Schönlein nephritis. Pediatr Nephrol 2004;19:920–923.
9.
Sanders JT, Wyatt RJ: IgA nephropathy and Henoch-Schönlein purpura nephritis. Curr Opin Pediatr 2008;20:163–170.
10.
Sunderkötter C, Roth J, Bonsmann G: Leukozytoklastische Vaskulitis. Hautarzt 2004; 55:759–785.
11.
Gedalia A: Henoch-Schönlein purpura. Curr Rheumatol Rep 2004;6:195–202.
12.
Fridey JL, Kaplan AA: Prescription and technique of therapeutic plasma exchange (review version 16.1). Jan 31, 2008. www. uptodate.com.
13.
Smith JW, Weinstein R, AABB Hemapheresis Committee KL, AABB Hemapheresis Committee, American Society for Apheresis: Therapeutic apheresis: a summary of current indication categories endorsed by the AABB and the American Society for Apheresis. Transfusion 2003;43:820–822.
14.
Eming R, Hertl M: Immunoadsorption in pemphigus. Autoimmunity 2006;39:609–616.
15.
Chen TC, Chung FR, Lee CH, Huang SC, Chen JB, Hsu KT: Successful treatment of crescentic glomerulonephritis associated with adult-onset Henoch-Schoenlein purpura by double-filtration plasmapheresis.Clin Nephrol 2004;61:213–216.
16.
Chen CL, Chiou YH, Wu CY, Lai PH, Chung HM: Cerebral vasculitis in Henoch-Schönlein purpura: a case report with sequential magnetic resonance imaging changes and treated with plasmapheresis alone. Pediatr Nephrol 2000;15:276–278.
17.
Kauffmann RH, Houwert DA: Plasmapheresis in rapidly progressive Henoch-Schoenlein glomerulonephritis and the effect on circulating IgA immune complexes. Clin Nephrol 1981;16:155–160.
18.
Gaskell H, Searle M, Dathan JR: Henoch-Schönlein purpura with severe ileal involvement responding to plasmapheresis. Int J Artif Organs1985;8:163–164.
19.
Rech J, Fuchs F, Kallert S, Hueber AJ, Requadt C, Manger B, Kalden JR, Amann K, Strauss R, Schulze-Koops H: Plasmapheresis therapy in an elderly patient with rapidly progressive Henoch-Schönlein purpura with disseminated organ involvement. Clin Rheumatol 2007;26:112–114.
20.
Lee J, Clayton F, Shihab F, Goldfarb-Rumyantzev A: Successful treatment of recurrent Henoch-Schönlein purpura in a renal allograft with plasmapheresis. Am J Transplant 2008;8:228–231.
21.
Wortmann SB, Fiselier TJW, Van de Kar NCAJ, Aarts RAHM, Warris A, Draaisma JMT: Refractory severe intestinal vasculitis due to Henoch-Schönlein purpura: successful treatment with plasmapheresis. Acta Paediatr 2006;95:622–623.
22.
Wen YK, Yang Y, Chang CC: Cerebral vasculitis and intracerebral hemorrhage in Henoch-Schönlein purpura treated with plasmapheresis. Pediatr Nephrol2005;20:223–225.
23.
Shenoy M, Ognjanovic MV, Coulthard MG: Treating severe Henoch-Schönlein and IgA nephritis with plasmapheresis alone. Pediatr Nephrol 2007;22:1167–1171.
24.
Hattori M, Ito K, Konomoto T, Kawaguchi H, Yoshioka T, Khono M: Plasmapheresis as the sole therapy for rapidly progressive Henoch-Schönlein purpura nephritis in children. Am J Kidney Dis 1999;33:427–433.
© 2009 S. Karger AG, Basel
2009
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.