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Granulomatosis with polyangiitis

Last updated: October 16, 2020

Summary

Granulomatosis with polyangiitis (GPA, previously known as Wegener 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

Epidemiological data refers to the US, unless otherwise specified.

Etiology

Pathophysiology

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.

Diagnostics

A biopsy is necessary to confirm the diagnosis.

Differential diagnoses

Granulomatosis with polyangiitis (GPA) Microscopic polyangiitis
Clinical presentation
Laboratory tests

The differential diagnoses listed here are not exhaustive.

Treatment

Prognosis

  • Without adequate treatment, the 1-year survival rate is < 20%. [16]
  • 5-year survival with adequate treatment is approx. 80%. [17]

References

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  3. de Lind van Wijngaarden RA, van Rijn L, Hagen EC, et al. Hypotheses on the etiology of antineutrophil cytoplasmic autoantibody associated vasculitis: the cause is hidden, but the result is known.. Clin J Am Soc Nephrol. 2008; 3 (1): p.237-52. doi: 10.2215/CJN.03550807 . | Open in Read by QxMD
  4. Granulomatosis with polyangiitis. https://radiopaedia.org/articles/granulomatosis-with-polyangiitis. Updated: April 13, 2017. Accessed: April 13, 2017.
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  12. Kubaisi B, Abu Samra K, Foster CS. Granulomatosis with polyangiitis (Wegener's disease): An updated review of ocular disease manifestations.. Intractable & rare diseases research. 2016; 5 (2): p.61-9. doi: 10.5582/irdr.2016.01014 . | Open in Read by QxMD
  13. Flossmann O, Berden A, de Groot K, et al. Long-term patient survival in ANCA-associated vasculitis. Ann Rheum Dis. 2010; 70 (3): p.488-494. doi: 10.1136/ard.2010.137778 . | Open in Read by QxMD
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