Wegener granulomatosis

A Triad of:

1. vasculitis of tissues of upper respiratory tract &
2. lower resp tract
3. FSGS (focal segmental glomerulonephritis)

• Look out for progressive upper or lower respiratory tract manifestations and oral/nasal ulcerations.
• small vessel vasculitis of arteries and veins; necrotizing, granulomatous
• airways
  • pain over sinus areas (see Sinusitis, Chronic)
  • nasal ulceration, purulent or bloody nasal discharge
  • destructive changes lead to saddle nose deformity
  • tracheal lesions, subglottic stenosis
• lungs
  • hemoptysis
  • cough
  • chest pain
• kidneys
  • hematuria, proteinuria, RBC casts
• Skin: nodules, purpura, ulcerations
• nonspecific: fever, malaise, wt loss, arthralgia, myalgias, chronic rhinitis or worsening sinusitis

Who?

• usually young or middle aged adults

Diagnosis:

• c-ANCA (circle the wagons), in 80%
• p-ANCA in a minority (usually in microscopic polyangitis - age of onset 50 years.)
• biopsy should be done

DDX

• r/o sepsis, fungal, mycobacterial
• Churg-Strauss may have similar triad of organ involvement
• angiocentric T-cell lymphomas (lymphomatoid granulomatosis) produce similar destructive lesions

Primer of the Rheumatic diseases. 11th ed.