A 75yo man presents with ARF. He has been troubled by recurrent epistaxis but over the last
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
- Nasal or oral involvement: Inflammation, ulcers, epistaxis
- Abnormal findings on CXR: Nodules, cavitations, etc.
- Urinary sediment: Microscopic hematuria ± RBC casts
- 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)