2. Definition
• WG is an necrotising granulomatous diseases
leading to
– inflammation of the upper and lower respiratory
tracts
– Systemic vasculitis of small arteries and veins
– Focal glomerulonephritis
3. Epidemiology
• prevalence of 5 per 100,000
• males > females (except in laryngeal
manifestations)
• wide age range but increased in 4-5th decade
• all racial groups affected but predominantly
whites
4. Aetiology
• cause of WG is unknown• ? viral related activation of ANCA
• Increasing circumstantial evidence supports
the concept that WG is an autoimmune
5. Pathogenesis
• (ANCA) no direct evidence
• Anti Neutrophil surface antibody → degranulation of
the leukocytes and the release of toxic oxygen radicals
and lysosomal enzymes
• neutrophils activated by ANCA → directly damage
endothelial cells → vasculitis
• Titres for ANCA correlate with disease activity and
predict relapses
• granular cytoplasmic staining pattern ANCA (c-ANCA)
has been strongly associated with WG
• a smaller number of patients with WG have a
perinuclear staining pattern ANCA (p-ANCA)
6. pathology
• necrotising granulomatous vasculitis
• vasculitis of small to medium-sized vessels and
polymorphous inflammatory infiltrate composed
of lymphocytes, histiocytes and less
frequently eosinophils and neutrophils
• “ischaemic” or “geographic” type necrosis with
basophilic smudgy appearance
• multinucleated giant cells or non-necrotising
granulomas
7. Clinical
• May be localised or systemic
• H+N manifestations = initial symptoms in 75%
• laryngx:
– Upper airway findings include oedema, ulceration of
larynx (25%) and significant subglottic stenosis (8.5%)
– Hoarseness, cough, haemoptysis, dyspnoea, stridor
and wheeze
– Flattening of both insp and exp phase in flow-volume
loop
8. Clinical (ear)
•
•
External
– Rarely involves external ear
– OE 2ry to OM
– Auricular chondritis similar to RP
Middle
– Serous otitis media – most
common otologic manifestation
• 2ry to ETD
– CHL
– Suppurative otitis media possibly
with granulation tissue
– CNVII palsy 2ry to otomastoiditis
– TM perforation?
• Inner
– SNHL = 2nd most
common L-T morbitidy
(renal = 1st)
• 35% affected
• May develop rapidly
• ? 2ry to vasculitis of
cochlear vasculature
• ? Immune complex
deposition within
cochlea
– NB vertigo = rare
– ? Tinnitus
9. Clinical (nose)
• Nose:
– Non-specific
• Nasal congestion, rhinorrhoea, anosmia, epistaxis
– Cobblestone mucosa
– Crusting, septal perforation, saddle nose deformity, dorsal
nasal pain (suggesting chondritis) and recurrent sinusitis
– Anterior nasal septum in Kiesselbach’s plexus = commonly
involved
– Nasal airway cicatricial stenosis
– Nasal cavity > maxillary > ethmoid > frontal > sphenoid
– NB often mistaken for NK cell lymphoma
10. Clinical
• Throat:
– Oral cavity manifestations
(rare)
– hyperplasia of gingiva and
gingivitis
– Strawberry gingival
hyperplasia
– Upper airway findings
include oedema, ulceration
of larynx (25%) and
significant subglottic
stenosis (8.5%)
• H+N:
– Salivary involvement
Sjögren syndrome
– Can have massive
enlargement of the SMG
or parotid glands
11. Clinical
• Pulmonary:
– Classic chest
radiographic findings
include bilateral multiple
parenchymal nodes (+/cavitation), or airway
disease that simulates
pneumonia
• Renal:
– Usual cause of death in
WG and the most
important prognostic
feature
12. Investigation
• Haematological
– Elevated ESR
– ANCA (C-ANCA & p-ANCA)
– WG → c-ANCA is +ve in 70% and p-ANCA in 25%
– Patients with polyarteritis nodosa and Kawasaki disease may also
test positive
• Radiological
– CXR: for pulmonary infiltrates
• Pathological
– Biopsy vasculitis of small vessels, granulomatous changes, and
focal necrosis
• Urinalysis
– Performed to evaluate urinary sediment (eg, RBC casts,
haematuria, proteinuria)
13. Diagnosis
• Criteria
– Nasal or oral
inflammation
– Chest x-ray showing
nodules, infiltrates
(fixed), or cavities
– Microscopic haematuria
or red cell casts in urine
– Granulomatous
inflammation on biopsy
(within vessel wall or
perivascular)
• New Criteria – all of:
– Lack of eosinophilia
– Biopsy verified
necrotising vasculitis
– Glomerulonephritis
– Granulomatous
inflammation –
surrogate
– PR3 c-ANCA +ve
14. Treatment
• untreated mortality rate of 90% at two years
• consists of meticulous dental and nasal care, removal of crusts from
the nose and ET orifices and ME drainage
• limited disease → prednisolone and methotrexate or prednisolone
and cyclophosphamide for 3 months → methotrexate and
azathioprine
• systemic disease → prednisolone and cyclophosphamide for 3-12
months → methotrexate and azathioprine
• other options include mycophenolate, anti-TNF and IV Ig
• for isolated sinus disease, treatment includes low dose steroids,
topical steroids, saline irrigations and antibiotics as needed
• airway compromise is alleviated with systemic steroids and
subglottic stenosis may warrant tracheotomy
15. Treatment
• AIRWAY
– Dilation and intralesional/inhaled steroid or
mitomycin C
– CO2 laser treatment of stenosis
– Silicone stenting of stenosis
– laryngotracheal reconstruction
– endoscopic longitudinal incision of stenosis
– Tracheostomy / Tracheal resection
