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A case of destructive Wegener's granulomatosis complicated by cytomegalovirus infection

Abstract

Background A 63-year-old man with a 4-year history of Wegener's granulomatosis presented with hemoptysis, palatal ulceration and sensorimotor polyneuropathy.

Investigations Physical examination, serologic testing, proteinase 3 enzyme-linked immunosorbent assay, IgG and IgM specific to Epstein–Barr virus enzyme-linked immunosorbent assay, MRI of the middle face, an ear, nose and throat consultation, immunohistochemical staining of an esophageal sample obtained from esophagogastroduodenoscopy, polymerase chain reaction of cytomegalovirus (CMV) DNA from esophageal and blood samples, and measurement of pp65 early antigen.

Diagnosis Wegener's granulomatosis with progressive palatal ulceration and osseous destruction complicated by CMV-related esophagitis during induction treatment.

Management Induction treatment with cyclophosphamide pulse therapy was discontinued and antiviral therapy with ganciclovir was started. Cyclophosphamide pulse therapy was later reintroduced, but there was subsequent CMV reactivation, and, therefore, cyclophosphamide pulse therapy was suspended again and ganciclovir and intravenous immunoglobulin were started. After control of CMV, cyclophosphamide pulse therapy was reinitiated. Prolonged immunosuppressive therapy controlled disease activity, without CMV reactivation.

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Figure 1: Wegener's granulomatosis with ear, nose and throat involvement and palatal destruction.
Figure 2: Wegener's granulomatosis with ear, nose and throat manifestations and beginning mucosal ulcerations.

References

  1. 1

    Leavitt RY et al. (1990) The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis. Arthritis Rheum 33: 1101–1107

    CAS  PubMed  Google Scholar 

  2. 2

    Jennette JC et al. (1994) Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 37: 187–192

    CAS  Article  Google Scholar 

  3. 3

    Jayne D (2001) Update on the European Vasculitis Study Group trials. Curr Opin Rheumatol 13: 48–55

    CAS  PubMed  Google Scholar 

  4. 4

    Rasmussen N (2001) Management of the ear, nose, and throat manifestations of Wegener granulomatosis: an otorhinolaryngologist's perspective. Curr Opin Rheumatol 13: 3–11

    CAS  PubMed  Google Scholar 

  5. 5

    Knecht K and Mishriki YY (1999) More than a mouth ulcer. Oral ulcer due to Wegener's granulomatosis. Postgrad Med 105: 200–203

    CAS  PubMed  Google Scholar 

  6. 6

    Torres HA et al. (2005) Gastrointestinal cytomegalivirus disease in patients with cancer: a two decade experience in a tertiary care cancer center. Eur J Cancer 41: 2268–2279

    PubMed  Google Scholar 

  7. 7

    Bottomley WW et al. (1992) Esophageal involvement in Behcet's disease. Is endoscopy necessary? Dig Dis Sci 37: 594–597

    CAS  PubMed  Google Scholar 

  8. 8

    Fauci AS et al. (1983) Wegener's granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med 98: 76–85

    CAS  PubMed  Google Scholar 

  9. 9

    de Groot K and Jayne D (2005) What is new in the therapy of ANCA-associated vasculitides? Take home messages from the 12th workshop on ANCA and systemic vasculitides. Clin Nephrol 64: 480–484

    CAS  PubMed  Google Scholar 

  10. 10

    Aries PM et al. (2005) Intravenous immunoglobulin therapy in vasculitis: speculation or evidence? Clin Rev Allergy Immunol 29: 237–245

    CAS  PubMed  Google Scholar 

  11. 11

    Aries PM et al. (2006) Lack of efficacy of rituximab in Wegener's granulomatosis with refractory granulomatous manifestations. Ann Rheum Dis 65: 853–858

    CAS  PubMed  PubMed Central  Google Scholar 

  12. 12

    Centers for Disease Control and Prevention; Infectious Disease Society of America; American Society of Blood and Marrow Transplantation (2000) Guidelines for preventing opportunistic infections among hematopoietic stem cell transplant recipients. Biol Blood Marrow Transplant 6: 659–713; 715; 717–727

  13. 13

    Hodson EM et al. (2005) Antiviral medications to prevent cytomegalovirus disease and early death in reciepiens of solid-organ transplants: a systematic review of randomised controlled trials. Lancet 365: 2105–2115

    Google Scholar 

  14. 14

    Markham A and Faulds D (1994) Ganciclovir. An update of its therapeutic use in cytomegalovirus infection. Drugs 48: 455–484

    CAS  PubMed  Google Scholar 

  15. 15

    Blanshard C et al. (1995) Treatment of AIDS-associated gastrointestinal cytomegalovirus infection with foscarnet and ganciclovir: a randomized comparison. J Infect Dis 172: 622–628

    CAS  PubMed  Google Scholar 

  16. 16

    Whitley RJ et al. (1998) Guidelines for the treatment of cytomegalovirus disease in patients with AIDS in the era of potent antiretroviral therapy: recommendations of an international panel. International AIDS Society-USA. Arch Intern Med 158: 957–969

    CAS  PubMed  Google Scholar 

  17. 17

    Blanshard C (1992) Treatment of HIV-related cytomegalovirus disease of the gastrointestinal tract with foscarnet. J Acquir Immune Defic Syndr 5 (Suppl 1): S25–S28

    PubMed  Google Scholar 

  18. 18

    Jacobson MA (2006) AIDS-related cytomegalovirus gastrointestinal disease. [www.uptodate.com] (accessed 30 June 2006)

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Correspondence to Peer M Aries.

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Aries, P., Ullrich, S. & Gross, W. A case of destructive Wegener's granulomatosis complicated by cytomegalovirus infection. Nat Rev Rheumatol 2, 511–515 (2006). https://doi.org/10.1038/ncprheum0269

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