Wegeners granulomatosis

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Wegener's Granulomatosis

General Considerations
Hallmarks
Small to medium sized systemic vasculitis
Granulomatous inflammation
Necrosis
Unknown etiology
Incidence of 1 case per 30,000
Affects predominantly whites (91%)
M>F
Mean age at diagnosis = 45

Sites of Involvement:
Lung (>90%)
Renal (75%)
Rapidly progressing glomerulonephritis, chronic renal failure
Trachea (15-60%)
Subglottic Stenosis
Other
Paranasal sinuses, nose (saddle nose deformity)
Ears, eyes, oral cavity
Skin, joints, nervous system
Rarely
Heart, GI tract and brain
Can potentially involve any organ in the body
Diagnosis:
American College of Rheumatology Classification
(2 out of 4 criteria is 88% sensitive and 92% specific for the diagnosis)
Nasal or oral inflammation
Abnormal chest X-ray
Urinary sediment
Biopsy
Imaging Findings
Conventional Radiography
Pulmonary nodules
Most common finding (40-70%)
Typically multiple and bilateral with a tendency to cavitate (50%)
Cavitary lesions may lead to atelectasis or pneumothorax
Thick or thin walled, well or ill circumscribed
Tendency to wax and wane
Size varies (1.5-10cm)
Air space consolidation
Waxing and waning infiltrates
May be mistaken for pneumonia
Pulmonary hemorrhage or edema
Hilar lypmhadenopathy
Normal in 20%
CT
Useful in further defining extent of disease seen in plain film, and revealing lesions not seen on plain film including:
Interstitial abnormalities
Tracheobronchial Abnormalities
Findings suggestive of vasculitis
Differential Diagnosis
For cavitary lung lesions
Infarction
Septic pulmonary emboli
Carcinoma
Squamous cell carcinoma
Infection
TB, Fungal, Bacterial
Rheumatoid nodules
Prognosis and Treatment
Tracheostomy may be required for tracheal strictures
Independent Risk factors of mortality
Older age
Absence of ear, nose and throat involvement
Renal or Cardiac involvement