Granulomatosis with Polyangiitis (Wegener’s granulomatosis)

Granulomatosis with Polyangiitis (GPA) is a rare multisystem autoimmune disease. Typically, the upper and lower respiratory tract and the kidneys are affected, but it may affect any organs.

• Small vessel vasculitis
• granulomatous inflammation of vessels

Signs and symptoms

VASCULITIS: FEVER – MALAISE – WEIGHT LOSS – ARTHRALGIA/MYALGIA

• Recurrent respiratory infection in adults
• Sinusitis
• Epistaxis
• Otitis media
• Mastoiditis
• Rhinitis
• Nasal ulcers
• Pulmonary nodules
• Pulmonary infiltrates
• Hemoptysis
• Hematuria
• Glomerulonephritis
• Renal failure

Other: Joint, eye, skin lesions

Saddle nose deformity

Goodpasture syndrome is similar to GPA, however nasal lesions and sinusitis could not be found in Goodpasture syndrome.

Dx

The best initial test: C-ANCA (+ve) Cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA)

Accurate test: Biopsy

CXR: pneumonitis, pulmonary nodules ( “coin lesions”) and infiltrations

Tx

Prednisolone

Cyclophosphamide

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Read more at:

1. http://www.merckmanuals.com/en-ca/professional/musculoskeletal-and-connective-tissue-disorders/vasculitis/granulomatosis-with-polyangiitis-gpa
2. https://en.wikipedia.org/wiki/Goodpasture_syndrome
3. http://emedicine.medscape.com/article/332622-overview