Hostname: page-component-76fb5796d-5g6vh Total loading time: 0 Render date: 2024-04-26T15:40:24.408Z Has data issue: false hasContentIssue false
  • English
  • Français

Case of acute, resistant fulminant Wegener's granulomatosis successfully treated by rituximab

Published online by Cambridge University Press:  12 March 2013

A K Ebrahim  and
J W Loock
A K Ebrahim*
Affiliation:
Division of Otorhinolaryngology, Faculty of Health Sciences, University of Stellenbosch/Tygerberg Hospital, Cape Town, South Africa
J W Loock
Affiliation:
Division of Otorhinolaryngology, Faculty of Health Sciences, University of Stellenbosch/Tygerberg Hospital, Cape Town, South Africa
*
Address for correspondence: Dr A K Ebrahim, Division of Otorhinolaryngology, Faculty of Health Sciences, University of Stellenbosch/Tygerberg Hospital, Private Bag X3, Tygerberg, 7505, Cape Town, South Africa E-mail: akebrahim@gmail.com
Get access Rights & Permissions [Opens in a new window]

Abstract

Background:

Wegener's granulomatosis is generally a chronic, indolent, inflammatory condition, treated with cytotoxics (cyclophosphamide) and corticosteroids.

Objective:

This paper reports an unusual case of acute fulminant Wegener's granulomatosis that failed to respond to conventional treatment, but showed a dramatic response to rituximab, which is a relatively new form of treatment for resistant cases.

Method:

As well as describing the case (with photographic illustrations), the current paper provides a review of the literature, focusing on acute Wegener's granulomatosis and frequency of resistance to ‘conventional’ forms of treatment. There is also an evaluation of the evidence for the effectiveness of rituximab in resistant Wegener's granulomatosis.

Results:

The patient responded remarkably well to rituximab and had no disease recurrence at 24 months' follow up.

Conclusion:

Clinicians should be aware of the acute fulminant form of Wegener's granulomatosis, as a delay in diagnosis and treatment may have fatal consequences. The paper also highlights the dramatic response to rituximab experienced by the patient.

Keywords

Wegener's GranulomatosisOtolaryngologyRituximab
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 127 , Issue 4 , April 2013 , pp. 411 - 414
Copyright
Copyright © JLO (1984) Limited 2013

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

Footnotes

Presented as a poster at the South African ENT Society Annual Congress, 24–27 October 2010, Drakensberg, South Africa.

References

1Oristrell, J, Bejarano, G, Jordana, R, Monteagudo, M, Marí, B, Casanovas, A et al. Effectiveness of rituximab in severe Wegener's granulomatosis: report of two cases and review of the literature. Open Respir Med J 2009;3:94–9CrossRefGoogle ScholarPubMed
2Del Pero, M, Chaudhry, A, Jones, RB, Sivasothy, P, Jani, P, Jayne, D.B-cell depletion with rituximab for refractory head and neck Wegener's granulomatosis: a cohort study. Clin Otolaryngol 2009;34:328–35CrossRefGoogle Scholar
3Popa, ER, Tervaert, JW.The relation between Staphylococcus aureus and Wegener's granulomatosis: current knowledge and future directions. Intern Med 2003;42:771–80CrossRefGoogle ScholarPubMed
4Erickson, VR, Hwang, PH.Wegener's granulomatosis: current trends in diagnosis and management. Curr Opin Otolaryngol Head Neck Surg 2007;15:170–6CrossRefGoogle ScholarPubMed
5Khan, A, Lawson, CA, Quinn, MA, Isdale, AH, Green, MJ.Successful treatment of ANCA-negative Wegener's granulomatosis with rituximab. Int J Rheumatol 2010. Epub 2010 Oct 26CrossRefGoogle ScholarPubMed
6Sanchez-Cano, D, Callejas-Rubio, JL, Ortego-Centeno, N.Effect of rituximab on refractory Wegener's granulomatosis with predominant granulomatous disease. J Clin Rheumatol 2008;14:92–3CrossRefGoogle ScholarPubMed
7Stone, JH, Merkel, PA, Spiera, R, Seo, P, Langford, CA, Hoffman, GS et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med 2010;363:221–32CrossRefGoogle ScholarPubMed
8de Groot, K, Rasmussen, N, Bacon, PA, Tervaert, JWC, Feighery, C, Gregorini, G et al. Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic ANCA-associated vasculitis. Arthritis Rheum 2005;52:2461–9CrossRefGoogle Scholar
9Jayne, D, Rasmussen, N, Andrassy, K, Bacon, P, Tervaert, JWC, Dadoniené, J.A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med 2003;349:36–44CrossRefGoogle ScholarPubMed
10Ntatsaki, E, Watts, RA, Scott, DG.Epidemiology of ANCA-associated vasculitis. Rheum Dis Clin North Am 2010;36:447–61CrossRefGoogle ScholarPubMed
11Thapar, A, George, A, Pfleiderer, A.Fulminant Wegener's granulomatosis presenting as epistaxis. Emerg Med J 2007;24:e14CrossRefGoogle ScholarPubMed
12Elizabeth, M, Hernandez, C, Lovrekovic, G, Schears, G, Helfaer, M, Friedman, D et al. Acute onset of Wegener's granulomatosis and diffuse alveolar hemorrhage treated successfully by extracorporeal membrane oxygenation. Pediatr Crit Care Med 2002;3:63–6Google Scholar
13Miszalski-Jamka, T, Szczeklik, W, Sokołowska, B, Miszalski-Jamka, K, Karwat, K, Grzadziel, G et al. Cardiac involvement in Wegener's granulomatosis resistant to induction therapy. Eur Radiol 2011;21:2297–304CrossRefGoogle ScholarPubMed
14Haubitz, M, Schellong, S, Gobel, U, Schurek, HJ, Schaumann, D, Koch, KM et al. Intravenous pulse administration of cyclophosphamide versus daily oral treatment in patients with antineutrophil cytoplasmic antibody-associated vasculitis and renal involvement: a prospective, randomized study. Arthritis Rheum 1998;41:1835–443.0.CO;2-Q>CrossRefGoogle ScholarPubMed
15Guillevin, L, Cordier, JF, Lhote, F, Cohen, P, Jarrousse, B, Royer, I et al. A prospective, multicenter, randomized trial comparing steroids and pulse cyclophosphamide versus steroids and oral cyclophosphamide in the treatment of generalized Wegener's granulomatosis. Arthritis Rheum 1997;40:2187–98CrossRefGoogle ScholarPubMed
16Fortin, PM, Tejani, AM, Bassett, K, Musini, VM.Intravenous immunoglobulin as adjuvant therapy for Wegener's granulomatosis. Cochrane Database Syst Rev 2009;(3):CD007057CrossRefGoogle ScholarPubMed
17Henes, JC, Fritz, J, Koch, S, Klein, R, Horger, M, Risler, T et al. Rituximab for treatment-resistant extensive Wegener's granulomatosis–additive effects of a maintenance treatment with leflunomide. Clin Rheumatol 2007;26:1711–15CrossRefGoogle ScholarPubMed
18Keogh, KA, Ytterberg, SR, Fervenza, FC, Carlson, KA, Schroeder, DR, Specks, U.Rituximab for refractory Wegener's granulomatosis. Am J Respir Crit Care Med 2006;173:180–7CrossRefGoogle ScholarPubMed
19Edwards, JC, Szczepanski, L, Szechinski, J, Filipowicz-Sosnowska, A, Emery, P, Close, DR et al. Efficacy of B-cell-targeted therapy with rituximab in patients with rheumatoid arthritis. N Engl J Med 2004;350:2572–81CrossRefGoogle ScholarPubMed
20Jones, RB, Tervaert, JWC, Hauser, T, Luqmani, R, Morgan, MD, Peh, CA et al. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. N Engl J Med 2010;363:211–20CrossRefGoogle ScholarPubMed
21Guerry, MJ, Brogan, P, Bruce, IN, D'Cruz, DP, Harper, L, Luqmani, R et al. Recommendations for the use of rituximab in anti-neutrophil cytoplasm antibody-associated vasculitis. Rheumatology (Oxford) 2012;51:634–43CrossRefGoogle ScholarPubMed