Transcript PowerPoint: 8.4MB - Bronchoscopy International

Part 9A: Wegener’s Granulomatosis
BRONCHATLAS©
Prepared By
Bronchoscopy International
Contact us at
[email protected]
BI
1
How to use this presentation




At anytime you may click anywhere with the left
mouse button to advance to the next slide.
This presentation contains NO video or Audio.
This presentation can be viewed FULL SCREEN
by right clicking on the slide and selecting Full
Screen on the menu bar.
To exit Full Screen, press the ESCAPE key.
BI
2
This presentation is part of a
comprehensive curriculum for
Flexible Bronchoscopy. Our goals are
to help health care workers become
better at what they do, and to
decrease the burden of procedurerelated training on patients.
BI
3
Wegener Granulomatosis

Wegener’s Granulomatosis is a necrotizing
vasculitis usually characterized by a triad of :



acute necrotizing granulomas of the upper respiratory tract (
ear ,nose, sinuses ,throat ), the lower respiratory tract ( lung )
,or both. May cause saddle-nose deformity.
Necrotizing or Granulomatosis vasculitis affecting small to
medium-sized vessels (e.g., capillaries, venules, arterioles,
and arteries), most prominent in the lungs and upper airways
but affecting other sites as well.
Renal disease in the form of focal necrotizing, and
cresenteric glomerulonephritis.
BI
4
Overview

Wegener’s Granulomatosis





incidence 8.5/million
2300 cases/year
Affects tracheobronchial tree in nearly 60% pts
Disease activity in airway poorly correlates with
PR3 ANCA.
Early recognition and treatment of airway
involvement can prevent untoward affects of
improper therapy.
BI
5
Wegener’s Granulomatosis

Clinical features







Persistent pneumonitis with bilateral nodular and cavity
infiltrates (95%).
Chronic sinusitis (90%).
Mucosal ulcerations of the nasopharynx (75%).
Renal disease (80%).
Other features includes skin rashes, muscle pains ,articular
involvement, mononeuritis or polyneuritis and fever.
Disease may be limited to one or more organ systems.
Prognosis:


Untreated, many patients may die within 1 year.
Serum c-ANCA is elevated in 95% of patients with active
generalized disease.
BI
6
Bronchoscopic Biopsy
Confirms
inflammation*
50% reveals
vasculitis
necrosis
microabscesses
giant cells
From: Am J Respir Crit Care Med 1995;151:522
BI
Photo courtesy E. Edell,
Mayo Clinic Rochester MN.
7
Endobronchial Biopsy in Wegener’s
Granulomatosis
Courtesy N. Narula, UCIMC
Necrotic zones
Several multinucleated giant cells
Inflammation
Hemotoxylin – Eosin stain
BI
8
Inflammatory Lesions
May



be accompanied by
Headaches, epistaxis
Nasal congestion
Arthralgias
Remission
possible after
treatment with steroids
and cytotoxic agents.
Tracheobronchitis
BI
9
Inflammatory Lesions
Pseudotumor Trachea
BI
10
Inflammatory Lesions
Ulcerative tracheobronchitis
Inflammatory cobblestoning
BI
11
Subglottic Inflammatory
Lesion
BI
12
Inflammatory Stenosis
Left main bronchial stricture
BI
13
Inflammatory lesions with evolving
segmental strictures
Photos courtesy H. Colt, UCIMC
BI
14
Chronic inflammation
Segmental strictures
BI
15
Persistent inflammatory plaques
BI
16
Inflammation, nasal plaques and
crusting may be present
BI
17
Chronic strictures
BI
18
Healed lesions
Web Stenosis




Result from scarring of
previously symptomatic
acute inflammation?
Often diagnosed in patients
with other systemic
manifestations in remission
Unresponsive to
immunosuppression
Potential for complication
BI
19
Healed strictures
34yo woman with




Nasal crusting
Epistaxis
Moderately controlled
using Prednisone and
cytotoxics.
Progressive dyspnea
present
RUL segments
BI
20
Healed lesions
Cicatricial Stenosis
Subglottic stenosis



15-20% of WG patients
Occurs independently of
systemic WG activity
Cough, dyspnea, stridor
BI
21
Main bronchial strictures
Bilateral bronchial strictures
Photos courtesy H. Colt UCIMC
Complete bronchial occlusion
BI
22
Healed lesions with persistent scarring
and segmental stenosis
Note
yellowish
mucosa
BI
23
Inflammatory lesions evolve
unfavorably or favorably
Upon diagnosis
4 months later
BI
24
Treatment
Inflammatory Lesions
Biopsy
Exclude
infection
Exclude other diseases
Minimize inflammation
Systemic therapy
Role for inhaled
corticosteroids unclear
BI
25
Treatment Acute Lesion
Active disease
Post immunosuppression
BI
26
Chronic Lesions
BI
6 months post dilatation
27
Chronic strictures
Chronic right bronchial stricture
BI
28
Chronic Stenosis
Beware friable mucosa and lobar bronchial strictures
BI
29
Silicone stents for chronic Stenosis
Left main
bronchus
3 months later
Beware granulation tissue
BI
and
recurrent obstruction
30
Step by Step©
A new curriculum
Assured competency and proficiency
1.
2.
3.
4.
5.
Web-based Self-learning study guide.
Computer-based simulations, didactic lectures,
and image encyclopedia.
Bronchoscopy step-by-step©: Practical exercises,
skills and tasks, competency testing.
Guided apprenticeship.
Learning the art of Bronchoscopy.
BRONCHATLAS©
DEMOCRATIZATION AND
GLOBALIZATION OF KNOWLEDGE
BI
31
All efforts are made by Bronchoscopy International
to maintain currency of online information. All
published multimedia slide shows, streaming videos,
and essays can be cited for reference as:
Bronchoscopy International: BronchAtlas©, an Electronic On-Line
Multimedia Slide Presentation.
http://www.Bronchoscopy.org/Bronchatlas/. Published 2007 (Please
add “Date Accessed”).
BRONCHATLAS©
Thank you
BI
32