Clinical analysis of 34 patients of Wegener's granulomatosis

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Author:
DUAN Xin-wang(Department of Rheumatology,Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Science,Beijing, 100032, China.)
MENG Qing-liang(Department of Rheumatology,Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Science,Beijing, 100032, China.)
WANG Hui(Department of Rheumatology,Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Science,Beijing, 100032, China.)
WU Ai-yu(Department of Rheumatology,Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Science,Beijing, 100032, China.)
WANG Qian(Department of Rheumatology,Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Science,Beijing, 100032, China.)
LI Meng-tao(Department of Rheumatology,Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Science,Beijing, 100032, China.)
ZENG Xiao-feng(Department of Rheumatology,Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Science,Beijing, 100032, China.)
Journal Title:
Chinese Journal of Emergency Medicine
Issue:
Volume 21, Issue 10, 2012
DOI:
10.3760/cma.j.issn.1671-0282.2012.10.021
Key Word:
Wegener's granulomatosis ; Vasculitis ; Lung imaging; Anti-neutrophil cytoplasmic antibody : Infection; Diagnosis times ; Treatment

Abstract: Objective To analyze the clinical data of diagnosis and treatment of Wegener' s granulomatosis (WG) in order to understand the nature of this disease. Methods The clinical data including clinical manifestations,laboratory findings imaging features,pathological changes and efficacy of treatment of 34 patients with WG admitted from January 2002 to March 2012 were analyzed.Results Of the 34 patients,male to female ratio was 18 to 16,and the average age subjected to WG onset was (45 ± 15)years old ranged from 18 to 81 years old.The average duration before the diagnosis confirmed was ( 140 ±72) mouths,while 29.4% was diagnosed within 3 months. The presenting symptoms included initial involvements in lung (41.1%),nose (38.2% ),eyes ( 11.7% ),ear (8.8% ) and constitutional symptoms such as fever (26.4% ). Throughout the whole disease course,the incidence of systemic impairments were as follows:lung (76.4%),nose (73.5%),kidney (67.6),eyes (58.5%),ear (47.0%),nervous system (41.1%),oral ulcer (20.5% ).Of laboratory findings,C - ANCA/PR3 -ANCA was positive in 61.7% patients,P - ANCA/MPO - ANCA positive in 20.5% patients,ANCA negative in 11.7% patients,P- ANCA positive with MPO- ANCA negative in one patient (2.9 % ) and C -ANCA positive with PR3 and MPO positive in one patient (2.9% ).Of imaging findings,nodules or masses were most commonly observed (34%), followed by fibrotic lesions (20.5% ), cavitations ( 17.6% )、opacities ( 17.6% ).Typically pathological triad ( vasculitis,necross and granuloma formation)was found in one patient ( 4.3% ),while two pathological changes of the triad occurred in 10 patients (43.4%). Pulmonary infection was highly prevalent (50%), and of them,20.5% patients were misdiagnosed as tuberculosis,malignancy or abscess.After admission,high dose of corticosteroids and cyclophosphamide were administered as bolus and maintenance therapy. Plasma exchange,intravenous immunoglobulin,and rituximab were added for refractory cases and optimal response were obtained in most cases.Conclusions Wegeners granulomatosis is a clinically complicated entity and sometimes can have progression,leading to misdiagnosis. Early and prompt use of steroids and cyclophosphamide may confer good therapeutic efficacy and avoid life -threatening complications.

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