1. Antineutrophil cytoplasmic antibody-associated vasculitis: Experience from Taichung Veterans General Hospital
施凱翔 梁凱莉 顏廷廷

2. ANCA-associated vasculitis: 3 kinds of small vessel disease
Microscopic Polyangiitis
Granulomatosis with Polyangiitis (Wegener’s granulomatosis)
Eosinophilic Granulomatous with polypangitis (Churg-Strauss syndrome)
MPA
GPA
EGPA

3. Anti-neutrophil cytoplasmic antibody (ANCA):
ANCA activates neutrophils with subsequent activation of the complement pathway, results in vascular damage, non-resolving chronic inflammation
Two kinds of staining pattern of ANCA: P-ANCA (staining of MPO) and C-ANCA (staining of PR3)
MPO: myeloperoxidase  PR3: proteinase 3

4. Clinical manifestations: localized or generalized

5. Patients and Methods
Retrospective chart review of patients with ANCA-associated vasculitis based on the classification criteria of American College of Rheumatology and definitions described at the Chapel Hill Consensus Conference 2012

6. Results
From March 2004 to March 2017: 11 patients with ANCA-associated vasculitis were enrolled (9 GPA, 1 MPA, and 1 EGPA)
Characteristics
Patient (N = 11)
N (%)
Sex
Female
Male
8 (73)
3 (27)
Age of Dx -yr
median (range)
distribution -N (%)
18-40 yrs
40-64 yrs
≥65 yrs
44 (20-73)
5 (45)
1 (9)

7. Results Clinical manifestations Patients (N=11) N (%) Rhinosinusitis
Rhinitis or nasal symptoms
Sinusitis
11 (100)
10 (91)
7 (64)
Fever
Hearing impairment
4 (36)
Ocular involvement
5 (45)
Lung involvement
Trachea involvement
6 (55)
2 (18)
Renal involvement
Central nervous system involvement
Mucocutaneous involvement
Gastrointestinal involvement
1 (9)

8. Nose and Sinus: Most common manifestation
Usually present with
nasal obstruction, rhinorrhea, or bloody nasal discharge

9. Nose and Sinus:
Tissue damage and bone destruction caused by vasculitis
Chronic inflammation could result in anosmia, septal perforation, and saddle nose

10. Ear and peripheral facial palsy
Sensorineural hearing loss
Otitis media/ effusion/ mastoiditis
Facial palsy

11. Larynx and Trachea, orbit
Glotic ulcer or subglottic stenosis
Orbital pseudotumor
Episcleritis, scleritis

12. Salivary glands and mucocutaneous findings
Parotid or submandibular glands may be swollen and sore

13. Surrogate markers Serology testing Patients (N = 11) N (%)
ANCA positive
PR3-ANCA
MPO-ANCA
11(100)
6(55)
5(45)
GPA
n=9
6(67)
3(33)
ANA positive
ANA equivocal
1(9)
3(27)

14. Pathological features
Nine out of 11 patients had pathological diagnoses.
Multiple biopsies in few patients
Most positive pathology came from sinonasal regions.
Necrotizing granuloma
Vasculitis

15. Treatment modality and outcomes
Steroid (11, 100%), cyclophosphamide (7, 63%)
Rituximab (6, 45%)
Ten out of 11 patients were alive at the end of this study
One patient with GPA died from septic shock
The disease relapse in 4 out of 9 GPA patients and need additional therapy

16. Discussions
The annual incidence of GPA in Taiwan was 0.37 per million patient-years from 1997 to 2008, according to the NHI database
The annual incidence of GPA in Europe was about 4.9 to 10.6 per million years according to different regions
Journal of Microbiology, Immunology and Infection, 2015, 48.5:
Ann Rheum Dis Dec; 60(12): 1156–1157.

17. Discussions
Five out of our patients initially seek medical help to ENT doctors.
Most patients had multi-system involvement except one.
Time between the first show-up to diagnosis range from 1 to 11 months

18. Discussions GPA MPA EPGA Our study
Treatment outcome of our patients was not inferior to literature review
GPA
MPA
EPGA
Our study
Remission rate
30-93%
75-89%
81-91%
100%
5-year survival
74-91%
45-76%
60-97%
91%
Relapse (variably defined)
18-60% 8%
35%
44%
Annals of the rheumatic diseases 67.7 (2008):

19. Conclusions
Otorhinolaryngologist could be the first physician to contact the patients with ANCA-associated vasculitis.
This diagnosis must always be taken into consideration in patient with atypical symptoms or unresponsive to standard treatment.

20. Conclusions
Harvest multiple sites, adequate tissue biopsy helps to make diagnosis.
Early diagnosis is crucial for therapy implementation and allows avoid irreversible organ damage.

21. Thank you!