Thrombotic thrombocytopenic purpura (TTP) occurring in patients with systemic lupus erythematosus (SLE) is rare and can be difficult to diagnose because of overlapping features of the two disorders. The aim of this study is to further characterize this uncommon association in terms of presenting features, diagnostic difficulties and treatment outcome. This is the largest series from a single centre with 6 patients diagnosed over a 6-year period. Two thirds of the patients had a simultaneous diagnosis of TTP and SLE. Half of the patients had a positive Coombs test along with clear features of TTP. Five patients received plasmapheresis as initial treatment while 1 patient received plasma infusions only. Four out of 5 patients responded to plasmapheresis and only 1 patient required cytotoxic therapy. TTP in association with SLE appears to be underdiagnosed and a positive Coombs test is not against the diagnosis of TTP in this setting. Most of the patients respond well to plasmapheresis. In case of a poor response, cytotoxic drugs should be considered early.

1.
Moschowitz E: Hyaline thrombosis of terminal arterioles and capillaries: a hitherto undescribed disease. Proc NY Pathol Soc 1924;24:21–24.
2.
Amorosi EL, Ultmann JE: Thrombotic thrombocytopenic purpura: Report of 16 cases and review of the literature. Medicine 1996;45:139–159.
3.
Rock GA, Shumak KH, Buskard NA, Blanchette VS, Kelton JG, Nair RC, Spasoff RA: Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura: Canadian Apheresis Study group. N Engl J Med 1991;325:393–397.
4.
Torok TJ, Holman RC, Chorba TL: Increasing mortality from thrombotic thrombocytopenic purpura in the United States – analysis of national mortality data, 1968–1991. Am J Hematol 1995;50:84–90.
5.
Hochberg MC: Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1997;40:1725 (letter).
6.
Porta C, Caporali R, Montecucco C: Thrombotic thrombocytopenic purpura and autoimmunity: a tale of shadows and suspects. Haematologica 1999;84:260–269.
7.
Furlan M, Robles R, Galbusera M, Remuzzi G, Kyrle PA, Brenner B, Krause M, Scharrer I, Aumann V, Mittler U, Solenthaler M, Lammle B: von-Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. N Engl J Med 1998;339:1578–1584.
8.
Tsai HM, Lian EC: Antibodies to von-Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med 1998;339:1585–1594.
9.
Caramaschi P, Riccetti MM, Pasini AF, Savarin T, Biasi D, Todeschini G: Systemic lupus erythematosus and thrombotic thrombocytopenic purpura. Report of three cases and review of the literature. Lupus 1998;7:37–41.
10.
Stricker RB, Davis JA, Gershow J, Yamamoto KS, Kiprov DD: Thrombotic thrombocytopenic purpura complicating systemic lupus erythematosus. Case report and literature review from the plasmapheresis era. J Rheumatol 1992;19:1469–1473.
11.
Porta C, Bobbio-Pallavicini E, Centurioni R, Caporali R, Montecucco CM: Thrombotic thrombocytopenic purpura in systemic lupus erythematosus. J Rheumatol 1993;20:1625–1626 (letter).
12.
Musio F, Bohen EM, Yuan CM, Welch PG: Review of thrombotic thrombocytopenic purpura in the setting of systemic lupus erythematosus. Semin Arthritis Rheum 1998;28:1–19.
13.
Manadan AM, Harris C, Schwartz MM, Block JA: The frequency of thrombotic thrombocytopenic purpura in patients with systemic lupus erythematosus undergoing kidney biopsy. J Rheumatol 2003;30:1227–1230.
14.
Devinski O, Petit C, Alonso D: Clinical and neuropathological findings in SLE: the role of vasculitis, heart emboli and TTP. Ann Neurol 1998;23:380–384.
15.
Mannucci PM, Vanoli M, Forza I, Canciani MT, Scorza R: Von Willebrand factor cleaving protease (ADAMTS-13) in 123 patients with connective tissue diseases (systemic lupus erythematosus and systemic sclerosis). Haematologica 2003;88:914–918.
16.
Long Zheng X, Kaufman RM, Goodnough LT, Evan Sadler J: Effect of plasma exchange on plasma ADAMTS13 metalloprotease activity, inhibitor level, and clinical outcome in patients with idiopathic and nonidiopathic thrombotic thrombocytopenic purpura. Blood; 103:4043–4049.
17.
Matsuda J, Sanaka T, Gohchi K, Matsui K, Uchida J, Matsumoto M, Fujimura Y: Occurrence of thrombotic thrombocytopenic purpura in a systemic lupus erythematosus patient with antiphospholipid antibodies in association with a decreased activity of von Willebrand factor-cleaving protease. Lupus 2002;11:463–464 (letter).
18.
Rick ME, Austin H, Leitman SF, Krizek DM, Aronson DL: Clinical usefulness of a functional assay for the von Willebrand factor cleaving protease (ADAMTS 13) and its inhibitor in a patient with thrombotic thrombocytopenic purpura. Am J Hematol 2004;75:96–100.
19.
Moore JC, Hayward CPM, Warkentin TE, Kelton JG: Decreased von Willebrand factor protease activity associated with thrombocytopenic disorders. Blood 2001;98:1842–1846.
20.
Vesely SK, George JN, Lammle B, Studt J-D, Alberio L, El-Haraki MA, Raskob GE: ADAMTS13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: relation to presenting features and clinical outcomes in prospective cohort of 142 patients. Blood 2003;102:60–68.
21.
Brunner HI, Freedman M, Silverman ED: Close relationship between systemic lupus erythematosus and thrombotic thrombocytopenic purpura in childhood. Arthritis Rheum 1999;42:2346–2355.
22.
Coppo P, Bengoufa D, Veyradier A, Wolf M, Bussel A, Millot GA, Malot S, Heshmati F, Mira JP, Boulanger E, Galicier L, Durey-Dragon MA, Fremeaux-Bacchi V, Ramakers M, Pruna A, Bordessoule D, Gouilleux V, Scrobohaci ML, Vernant JP, Moreau D, Azoulay E, Schlemmer B, Guillevin L, Lassoued K; Reseau d’Etude des Microangiopathies Thrombotiques de l’Adulte: Severe ADAMTS13 deficiency in adult idiopathic thrombotic microangiopathies defines a subset of patients characterized by various autoimmune manifestations, lower platelet count and mild renal involvement. Medicine (Baltimore) 2004;83:233–244.
23.
Vasoo S, Thumboo J, Fong K-Y: Thrombotic thrombocytopenic purpura in systemic lupus erythematosus: disease activity and the use of cytotoxic drugs. Lupus 2002;11:443–450.
24.
Vaidya S, Abul-ezz S, Lipsmeyer E: Thrombotic thrombocytopenic purpura and systemic lupus erythematosus. Scand J Rheumatol 2001;30:308–310.
25.
Allford SL, Hunt BJ, Rose P, Machin SJ, on behalf of the Haemostasis and Thrombosis Task Force of the British Committee for Standards in Haematology: Guidelines on the diagnosis and management of the thrombotic microangiopathic haemolytic anaemias. Br J Haematol 2003;120:556–573.
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.