eMedicine Specialties > Otolaryngology and Facial Plastic Surgery > Granulomatosis Diseases

Wegener Granulomatosis: Follow-up

Author: Neil Tanna, MD, MBA, Staff Physician, Division of Otolaryngology-Head and Neck Surgery, The George Washington University
Coauthor(s): Charles A Elmaraghy, MD, Assistant Professor, Department of Otolaryngology-Head and Neck Surgery, Ohio State University Medical Center; Douglas R Sidell, MD, Resident Physician, Department of Otolaryngology-Head and Neck Surgery, University of California at Los Angeles Medical Center; John Boone, MD, Consulting Staff, Department of Otolaryngology, Naval Hospital Oak Harbor
Contributor Information and Disclosures

Updated: Mar 24, 2008

Follow-up

Prognosis

Untreated WG has a rapid fatal course with a mean survival of 5 months. Death most frequently results from renal failure. Pulmonary complications are the second leading cause of mortality. Therapy with glucocorticoids and cytotoxic agents produces clinical improvement in more than 90% of patients; complete remission occurs in 75%. However, relapses are common, and WG is still associated with a mortality rate of 20%.

Miscellaneous

Medicolegal Pitfalls

The early diagnosis of WG can be difficult, similar to that in many multisystem diseases. Symptoms can be nonspecific at initial presentation; however, early diagnosis is important to prevent renal and pulmonary complications. A high index of suspicion combined with generous biopsy samples of available tissue and the use of c-ANCA best ensures that the diagnosis is not missed and treatment not delayed.

 


More on Wegener Granulomatosis

Overview: Wegener Granulomatosis
Differential Diagnoses & Workup: Wegener Granulomatosis
Treatment & Medication: Wegener Granulomatosis
Follow-up: Wegener Granulomatosis
References

References

  1. Gubbels SP, Barkhuizen A, Hwang PH. Head and neck manifestations of Wegener's granulomatosis. Otolaryngol Clin North Am. Aug 2003;36(4):685-705. [Medline].

  2. Cadoni G, Prelajade D, Campobasso E. Wegener's granulomatosis: a challenging disease for otorhinolaryngologists. Acta Otolaryngol. Oct 2005;125(10):1105-10. [Medline].

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  4. Cummings CW, Haughey BH, Thomas JR, et al. Otolaryngology - Head and Neck Surgery. 4th ed. St. Louis, MO: Mosby, Inc; 2005:934-936; 1493-1508.

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  6. Finley JC, Bloom DC, Thiringer JK. Wegener granulomatosis presenting as an infiltrative retropharyngeal mass with syncope and hypoglossal paresis. Arch Otolaryngol Head Neck Surg. Mar 2004;130(3):361-5. [Medline].

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  8. Leavitt RY, Fauci AS, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis. Arthritis Rheum. Aug 1990;33(8):1101-7. [Medline].

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  13. Illei GG, Austin HA, Crane M. Combination therapy with pulse cyclophosphamide plus pulse methylprednisolone improves long-term renal outcome without adding toxicity in patients with lupus nephritis. Ann Intern Med. Aug 21 2001;135(4):248-57. [Medline].

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Further Reading

Keywords

Wegener granulomatosis, WG, Wegener's granulomatos, granuloma, respiratory tracts, disseminated vasculitis, glomerulonephritis

Contributor Information and Disclosures

Author

Neil Tanna, MD, MBA, Staff Physician, Division of Otolaryngology-Head and Neck Surgery, The George Washington University
Neil Tanna, MD, MBA is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Medical Association, and Medical Society of the District of Columbia
Disclosure: Nothing to disclose.

Coauthor(s)

Charles A Elmaraghy, MD, Assistant Professor, Department of Otolaryngology-Head and Neck Surgery, Ohio State University Medical Center
Charles A Elmaraghy, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Medical Association, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Douglas R Sidell, MD, Resident Physician, Department of Otolaryngology-Head and Neck Surgery, University of California at Los Angeles Medical Center
Disclosure: Nothing to disclose.

John Boone, MD, Consulting Staff, Department of Otolaryngology, Naval Hospital Oak Harbor
John Boone, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery
Disclosure: Nothing to disclose.

Medical Editor

David J Terris, MD, FACS, Porubsky Professor and Chairman, Department of Otolaryngology, Medical College of Georgia
David J Terris, MD, FACS is a member of the following medical societies: Alpha Omega Alpha, American Academy of Otolaryngology-Head and Neck Surgery, American Association for the Advancement of Science, American Bronchoesophagological Association, American College of Surgeons, American Head and Neck Society, Federation of American Societies for Experimental Biology, International Association of Endocrine Surgeons, Phi Beta Kappa, Radiation Research Society, Society of University Otolaryngologists-Head and Neck Surgeons, and Triological Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Peter S Roland, MD, Professor, Department of Neurological Surgery, Professor and Chairman, Department of Otolaryngology-Head and Neck Surgery, Director of Clinical Center for Auditory, Vestibular and Facial Nerve Disorders, Chief of Pediatric Otology, University of Texas Southwestern Medical Center; Adjunct Professor of Communicative Disorders, School of Human Development.
Peter S Roland, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Otolaryngic Allergy, American Academy of Otolaryngology-Head and Neck Surgery, American Auditory Society, American Laryngological Rhinological and Otological Society, American Neurotology Society, American Otological Society, North American Skull Base Society, and Society of University Otolaryngologists-Head and Neck Surgeons
Disclosure: Alcon labs Honoraria Speaking and teaching; GSK Honoraria Speaking and teaching; Advanced Bionics Honoraria Board membership; Cochlear corp Honoraria Board membership; Med El corp travel grants Speaking and teaching; Insight vision Consulting fee Consulting

CME Editor

Christopher L Slack, MD, Otolaryngology-Facial Plastic Surgery, Private Practice, Associated Coastal ENT; Medical Director, Treasure Coast Sleep Disorders
Christopher L Slack, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Arlen D Meyers, MD, MBA, Professor, Department of Otolaryngology-Head and Neck Surgery, University of Colorado School of Medicine
Arlen D Meyers, MD, MBA is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Head and Neck Society
Disclosure: Advanced Headache Intervention Consulting fee Consulting; Covidien Corp Consulting fee Consulting

 
 
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