• Clinical science

Granulomatosis with polyangiitis (Wegener granulomatosis)

Abstract

Granulomatosis with polyangiitis (GPA, previously known as Wegener's granulomatosis) is a systemic vasculitis that affects both small and medium-sized vessels. Patients typically initially suffer from a limited form that may consist of constitutional symptoms and localized manifestations such as chronic sinusitis, rhinitis, otitis media, ocular conditions, and/or skin lesions. In later stages, more serious manifestations may arise, including pulmonary complications and glomerulonephritis, although the skin, eyes, and heart may also be involved. Diagnosis is based on laboratory testing (positive for PR3-ANCA/c-ANCA), imaging, and biopsy of affected organs, which demonstrate necrotizing granulomatous inflammation. GPA is treated with immunosuppressive drugs, typically consisting of glucocorticoids combined with methotrexate, cyclophosphamide, or rituximab. Relapses are common.

Epidemiology

  • Sex: =
  • Peak incidence: 65–74 years
  • Prevalence: ∼ 3/100,000 in the United States (rare condition)
  • Affects white patients disproportionately; African-American patients are rarely affected.

References:[1][2][3]

Epidemiological data refers to the US, unless otherwise specified.

Etiology

Idiopathic

References:[4][5]

Clinical features

Upper respiratory manifestations (i.e., purulent, sometimes bloody discharge; chronic nasopharyngeal infections; saddle nose deformity) are the most common chief complaints!

GPA triad: necrotizing vasculitis of small arteries, upper/lower respiratory tract manifestations, and glomerulonephritis!

References:[3][6][7]

Diagnostics

A biopsy is necessary to confirm the diagnosis!

References:[3][6]

Differential diagnoses

Granulomatosis with polyangiitis (GPA) Microscopic polyangiitis
Clinical presentation
  • Potentially palpable purpura
  • Nasopharyngeal involvement less common
  • No granulomatous inflammation
Laboratory tests

References:[8]

The differential diagnoses listed here are not exhaustive.

Treatment

References:[1][3][9][10]

Prognosis

  • Without adequate treatment, the mortality rate is approx. 90% within 2 years.
  • 5-year survival with adequate treatment is approx. 80%.

References:[11][12]