Korean J Otorhinolaryngol-Head Neck Surg.  2008 Dec;51(12):1109-1118.

Clinical Experiences of Diagnosis and Treatment for Wegener's Granulomatosis

• Park KT
• Kong IG
• Han DH
• Kim DW
• Kim SH
• Rhee CS
• Lee CH
• Min YG
• Kim DY

Abstract

BACKGROUND AND OBJECTIVES: Because most patients with Wegener's granulomatosis (WG) visit otolaryngologists complaining of head and neck symptoms, otolaryngologists may play an important role in early diagnosis. In this study, we investigated clinical features, laboratory findings and treatment outcomes of 33 patients with WG, and propose a new algorithm for the diagnosis of WG.
SUBJECTS AND METHOD
Thirty-three patients with WG who visited the clinic between January 1980 and December 2007 were included. The medical records of 13 male and 20 female patients were retrospectively reviewed. The mean follow-up duration was 68 months.
RESULTS
Most WG patients had ENT symptoms such as nasal obstruction, rhinorrhea, otorrhea, hearing loss, dyspnea and hoarseness. The mean duration from ENT examination to diagnosis was 6.7 months (3 days- 102 months) and tissue biopsy was performed on an average of 2.1 times to confirm the diagnosis. While 16 patients could be diagnosed by the first biopsy, the others were required to repeat the biopsy. ANCA was positive in 17 patients (50%). Twentysix patients (78.7%) had multiple organ involvements such as lung, kidney, joint and skin. Twenty-eight patients received a combination of prednisolone and cyclophosphamide with the remission rate of 87%.
CONCLUSION
Based on this study, we propose a better algorithm for the diagnosis of WG to avoid unnecessary delay in the diagnosis and treatment of WG.

Keyword

Wegener granulomatosis; Diagnosis; ANCA; Biopsy

MeSH Terms

Antibodies, Antineutrophil Cytoplasmic
Biopsy
Cyclophosphamide
Dyspnea
Early Diagnosis
Female
Follow-Up Studies
Head
Hearing Loss
Hoarseness
Humans
Joints
Kidney
Lung
Male
Medical Records
Nasal Obstruction
Neck
Prednisolone
Retrospective Studies
Skin
Wegener Granulomatosis
Antibodies, Antineutrophil Cytoplasmic
Cyclophosphamide
Prednisolone