Part 9A: Wegener’s Granulomatosis
BRONCHATLAS©
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This presentation is part of a
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Wegener Granulomatosis
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Wegener’s Granulomatosis is a necrotizing
vasculitis usually characterized by a triad of :
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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.
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Overview
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Wegener’s Granulomatosis
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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.
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Wegener’s Granulomatosis
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Clinical features
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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:
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Untreated, many patients may die within 1 year.
Serum c-ANCA is elevated in 95% of patients with active
generalized disease.
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Bronchoscopic Biopsy
Confirms
inflammation*
50% reveals
vasculitis
necrosis
microabscesses
giant cells
From: Am J Respir Crit Care Med 1995;151:522
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Photo courtesy E. Edell,
Mayo Clinic Rochester MN.
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Endobronchial Biopsy in Wegener’s
Granulomatosis
Courtesy N. Narula, UCIMC
Necrotic zones
Several multinucleated giant cells
Inflammation
Hemotoxylin – Eosin stain
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Inflammatory Lesions
May
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be accompanied by
Headaches, epistaxis
Nasal congestion
Arthralgias
Remission
possible after
treatment with steroids
and cytotoxic agents.
Tracheobronchitis
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Inflammatory Lesions
Pseudotumor Trachea
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Inflammatory Lesions
Ulcerative tracheobronchitis
Inflammatory cobblestoning
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Subglottic Inflammatory
Lesion
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Inflammatory Stenosis
Left main bronchial stricture
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Inflammatory lesions with evolving
segmental strictures
Photos courtesy H. Colt, UCIMC
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Chronic inflammation
Segmental strictures
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Persistent inflammatory plaques
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Inflammation, nasal plaques and
crusting may be present
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Chronic strictures
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Healed lesions
Web Stenosis
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Result from scarring of
previously symptomatic
acute inflammation?
Often diagnosed in patients
with other systemic
manifestations in remission
Unresponsive to
immunosuppression
Potential for complication
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Healed strictures
34yo woman with
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Nasal crusting
Epistaxis
Moderately controlled
using Prednisone and
cytotoxics.
Progressive dyspnea
present
RUL segments
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Healed lesions
Cicatricial Stenosis
Subglottic stenosis
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15-20% of WG patients
Occurs independently of
systemic WG activity
Cough, dyspnea, stridor
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Main bronchial strictures
Bilateral bronchial strictures
Photos courtesy H. Colt UCIMC
Complete bronchial occlusion
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Healed lesions with persistent scarring
and segmental stenosis
Note
yellowish
mucosa
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Inflammatory lesions evolve
unfavorably or favorably
Upon diagnosis
4 months later
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Treatment
Inflammatory Lesions
Biopsy
Exclude
infection
Exclude other diseases
Minimize inflammation
Systemic therapy
Role for inhaled
corticosteroids unclear
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Treatment Acute Lesion
Active disease
Post immunosuppression
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Chronic Lesions
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6 months post dilatation
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Chronic strictures
Chronic right bronchial stricture
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Chronic Stenosis
Beware friable mucosa and lobar bronchial strictures
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Silicone stents for chronic Stenosis
Left main
bronchus
3 months later
Beware granulation tissue
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and
recurrent obstruction
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