3wks he reports to have coughed up blood too. What is the single most likely positive antibody?
a. P ANCA
b. C ANCA
c. Anti Ro
d. Anti DS DNA
e. Anti centromere
answer: B
GRANULOMATOSIS WITH POLYANGIITIS (GPA, formerly known as Wegener’s Granulomatosis)
Definition
• granulomatous inflammation of vessels that may affect the upper airways (rhinitis, sinusitis), lungs (pulmonary nodules, infiltrates), and kidneys (glomerulonephritis, renal failure).
Classification Criteria for GPA
1. Nasal or oral involvement: Inflammation, ulcers, epistaxis
2. Abnormal findings on CXR: Nodules, cavitations, etc.
3. Urinary sediment: Microscopic hematuria ± RBC casts
4. Biopsy of involved tissue: Lungs show granulomas, kidneys show necrotizing segmental glomerulonephritis
Signs and Symptoms
• systemic
malaise, fever, weakness, weight loss
• HEENT
- sinusitis or rhinitis, nasal crusting and bloody nasal discharge, nasoseptal perforation, saddle nose deformity
- proptosis due to: inflammation/vasculitis involving extra-ocular muscles, granulomatous retrobulbar space occupying lesions or direct extension of masses from the upper respiratory tract
- hearing loss due to involvement of CN VIII
• pulmonary
- cough, hemoptysis, granulomatous upper respiratory tract masses
• renal
- hematuria
• other
- joint, skin, eye complaints, vasculitic neuropathy
Investigations:
• highly associated with c-ANCA
• biopsy: renal (segmental necrotizing glomerulonephritis), lung (granulomas, tracheobronchial
erosion)
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