1. Part 9A: Wegener’s Granulomatosis
BRONCHATLAS©
Prepared By BI

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3. 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 procedure-related training on patients. BI

4. 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

5. 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

6. 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

7. Bronchoscopic Biopsy Confirms inflammation* 50% reveals vasculitis
necrosis
microabscesses
giant cells
Photo courtesy E. Edell, Mayo Clinic Rochester MN.
From: Am J Respir Crit Care Med 1995;151:522 BI

8. Endobronchial Biopsy in Wegener’s Granulomatosis
Necrotic zones
Several multinucleated giant cells
Inflammation
Hemotoxylin – Eosin stain
Courtesy N. Narula, UCIMC BI

9. Inflammatory Lesions May be accompanied by
Headaches, epistaxis
Nasal congestion
Arthralgias
Remission possible after treatment with steroids and cytotoxic agents.
Tracheobronchitis BI

10. Inflammatory Lesions
Pseudotumor Trachea BI

11. Ulcerative tracheobronchitis Inflammatory cobblestoning
Inflammatory Lesions
Ulcerative tracheobronchitis
Inflammatory cobblestoning BI

12. Subglottic Inflammatory LesionBI

13. Inflammatory Stenosis
Left main bronchial stricture BI

14. Inflammatory lesions with evolving segmental strictures
Photos courtesy H. Colt, UCIMC BI

15. Chronic inflammation
Segmental strictures BI

16. Persistent inflammatory plaquesBI

17. Inflammation, nasal plaques and crusting may be presentBI

18. Chronic strictures BI

19. 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

20. Healed strictures 34yo woman with Nasal crusting Epistaxis
Moderately controlled using Prednisone and cytotoxics.
Progressive dyspnea present
RUL segments BI

21. Healed lesions Cicatricial Stenosis
Subglottic stenosis
15-20% of WG patients
Occurs independently of systemic WG activity
Cough, dyspnea, stridor BI

22. Main bronchial strictures
Bilateral bronchial strictures
Complete bronchial occlusion
Photos courtesy H. Colt UCIMC BI

23. Healed lesions with persistent scarring and segmental stenosis
Note yellowish mucosa BI

24. Inflammatory lesions evolve unfavorably or favorably
Upon diagnosis
4 months later BI

25. Treatment Inflammatory Lesions Biopsy Exclude infection
Exclude other diseases
Minimize inflammation
Systemic therapy
Role for inhaled corticosteroids unclear BI

26. Treatment Acute Lesion
Active disease
Post immunosuppression BI

27. Chronic Lesions
6 months post dilatation BI

28. Chronic strictures
Chronic right bronchial stricture BI

29. Beware friable mucosa and lobar bronchial strictures
Chronic Stenosis
Beware friable mucosa and lobar bronchial strictures BI

30. Silicone stents for chronic Stenosis
3 months later
Beware granulation tissue and recurrent obstruction
Left main bronchus BI

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5/26/17
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5/26/17
All Rights Reserved, 2017