Hostname: page-component-cd9895bd7-gbm5v Total loading time: 0 Render date: 2024-12-28T02:18:49.511Z Has data issue: false hasContentIssue false
  • English
  • Français

Wegener's granulomatosis presenting as acute systemic vasculitis following 20 years of limited tracheobronchial disease

Published online by Cambridge University Press:  28 January 2009

J E Peters ,
A D Salama  and
P W Ind
J E Peters*
Affiliation:
Department of Respiratory Medicine, Hammersmith Hospital, London, UK
A D Salama
Affiliation:
Department of Renal Medicine, Hammersmith Hospital, London, UK
P W Ind
Affiliation:
Department of Respiratory Medicine, Hammersmith Hospital, London, UK
*
Address for correspondence: Dr James Peters, Department of Respiratory Medicine, Hammersmith Hospital, Du Cane Road, London W12 0HS, UK. Fax: 0208 743 9733 E-mail: james.peters@imperial.nhs.uk
Get access Rights & Permissions [Opens in a new window]

Abstract

Objective:

We report a patient with a 20-year history of apparently idiopathic airways stenoses, who presented with an antineutrophil cytoplasmic antibody (ANCA) associated, acute, systemic vasculitis with necrotising glomerulonephritis, subsequently diagnosed as Wegener's granulomatosis.

Methods:

We present a case report and a review of the world literature on airway stenosis in Wegener's granulomatosis.

Results:

To our knowledge, this is the first report of Wegener's granulomatosis manifesting as local airway disease for such a prolonged period, before transforming into a systemic vasculitis.

Conclusions:

This case highlights the need for physicians to be alert to the possibility of Wegener's granulomatosis as a cause of apparently idiopathic airway stenosis, and to be aware that systemic disease may occur in very long-standing, limited Wegener's granulomatosis.

Keywords

Wegener's GranulomatosisTracheal StenosisVasculitisAntineutrophil Cytoplasmic AntibodiesGlomerulonephritis
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 123 , Issue 12 , December 2009 , pp. 1375 - 1377
Copyright
Copyright © JLO (1984) Limited 2009

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.)

References

1Marh, AD, Neogi, T, Merkel, PA. Epidemiology of Wegener's granulomatosis: lessons from descriptive studies and analyses of genetic and environmental risk determinants. Clin Exp Rheumatol 2006;24(suppl 41):S82–91Google Scholar
2Carrington, CB, Liebow, AA. Limited forms of angiitis and granulomatosis of Wegener's type. Am J Med 1966;41:497–527CrossRefGoogle ScholarPubMed
3McDonald, TJ, Neel, HB, DeRemee, RA. Wegener's granulomatosis of the subglottis and the upper portion of the trachea. Ann Otol Rhinol Laryngol 1982;91:593–6CrossRefGoogle ScholarPubMed
4Screaton, NJ, Sivasothy, P, Flower, CD, Lockwood, CM. Tracheal involvement in Wegener's granulomatosis: evaluation using spiral CT. Clin Radiol 1998;53:809–15CrossRefGoogle ScholarPubMed
5Nouraei, SAR, Obholzer, R, Ind, PW, Salama, AD, Pusey, CD, Porter, F et al. Results of endoscopic surgery and intralesional steroid therapy for airway compromise due to tracheobronchial Wegener's granulomatosis. Thorax 2008;63:49–52CrossRefGoogle ScholarPubMed
6Arauz, JC, Fonseca, R. Wegener's granulomatosis appearing initially in the trachea. Ann Otol Rhinol Laryngol 1982;91:588–92CrossRefGoogle ScholarPubMed
7Strange, C, Halstead, L, Baumann, M, Sahn, SA. Subglottic stenosis in Wegener's granulomatosis: development during cyclophosphamide treatment with response to carbon dioxide laser therapy. Thorax 1990;45:300–1CrossRefGoogle ScholarPubMed
8Boubenider, SA, Akhtar, M, Alfurayh, O, Algazian, S, Taibah, K, Qunibi, W. Late recurrence of Wegener's granulomatosis presenting as tracheal stenosis in a renal transplant patient. Clin Transplant 1994;8:5–9Google Scholar
9Fienburg, R. The protracted superficial phenomenon in pathergic (Wegener's) granulomatosis. Hum Path 1981;12:459–67Google Scholar
10Mathison, DJ, Wright, CD, Ashiku, SK, Wain, JC. Successful treatment of idiopathic laryngotracheal stenosis by resection and primary anastomosis. Ann Otol Rhinol Laryngol 2003;112:798–9Google Scholar