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Granulomatosis with polyangiitis

Last updated: October 16, 2020

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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.

Epidemiological data refers to the US, unless otherwise specified.

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.

A biopsy is necessary to confirm the diagnosis.

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

The differential diagnoses listed here are not exhaustive.

  • Without adequate treatment, the 1-year survival rate is < 20%. [16]
  • 5-year survival with adequate treatment is approx. 80%. [17]
  1. Pace C, Presicce M, Lamacchia F, Ferrari D, Sergiacomi G. Onset of granulomatosis with polyangiitis obscured by heart disease in an elderly man.. Radiology case reports. 2020; 15 (1): p.54-58. doi: 10.1016/j.radcr.2019.09.037 . | Open in Read by QxMD
  2. Panupattanapong S, Stwalley DL, White AJ, Olsen MA, French AR, Hartman ME. Epidemiology and Outcomes of Granulomatosis With Polyangiitis in Pediatric and Working-Age Adult Populations In the United States: Analysis of a Large National Claims Database.. Arthritis & rheumatology (Hoboken, N.J.). 2018; 70 (12): p.2067-2076. doi: 10.1002/art.40577 . | Open in Read by QxMD
  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.
  5. Heera R, Choudhary K, Beena VT, Simon R. Strawberry gingivitis: A diagnostic feature of gingival Wegener's granulomatosis!. Dental research journal. 2012; 9 (Suppl 1): p.S123-6.
  6. Sung IY, Kim YM, Cho YC, Son JH. Role of gingival manifestation in diagnosis of granulomatosis with polyangiitis (Wegener's granulomatosis).. Journal of periodontal & implant science. 2015; 45 (6): p.247-51. doi: 10.5051/jpis.2015.45.6.247 . | Open in Read by QxMD
  7. Qasim A, Patel J. ANCA Positive Vasculitis (ANCA Positive Angitis). StatPearls. 2020 .
  8. Walsh M, Merkel PA, Peh CA, et al. Plasma Exchange and Glucocorticoids in Severe ANCA-Associated Vasculitis.. N Engl J Med. 2020; 382 (7): p.622-631. doi: 10.1056/NEJMoa1803537 . | Open in Read by QxMD
  9. Miloslavsky EM, Niles JL, Wallace ZS, et al. Reducing glucocorticoid duration in ANCA-associated vasculitis: A pilot trial.. Semin Arthritis Rheum. 2018; 48 (2): p.288-292. doi: 10.1016/j.semarthrit.2018.01.013 . | Open in Read by QxMD
  10. Charles P, Perrodeau É, Samson M, et al. Long-Term Rituximab Use to Maintain Remission of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: A Randomized Trial.. Ann Intern Med. 2020; 173 (3): p.179-187. doi: 10.7326/M19-3827 . | Open in Read by QxMD
  11. Pagnoux C, Mahr A, Hamidou MA, et al. Azathioprine or methotrexate maintenance for ANCA-associated vasculitis.. N Engl J Med. 2008; 359 (26): p.2790-803. doi: 10.1056/NEJMoa0802311 . | Open in Read by QxMD
  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
  14. Xiao H, Hu P, Falk RJ, Jennette JC. Overview of the Pathogenesis of ANCA-Associated Vasculitis. Kidney Diseases. 2015; 1 (4): p.205-215. doi: 10.1159/000442323 . | Open in Read by QxMD
  15. Jennette JC, Falk RJ, Gasim AH. Pathogenesis of antineutrophil cytoplasmic autoantibody vasculitis.. Curr Opin Nephrol Hypertens. 2011; 20 (3): p.263-70. doi: 10.1097/MNH.0b013e3283456731 . | Open in Read by QxMD
  16. Nakazawa D, Masuda S, Tomaru U, Ishizu A. Pathogenesis and therapeutic interventions for ANCA-associated vasculitis.. Nature reviews. Rheumatology. 2019; 15 (2): p.91-101. doi: 10.1038/s41584-018-0145-y . | Open in Read by QxMD
  17. Falk RJ, Jennette JC. ANCA disease: where is this field heading?. J Am Soc Nephrol. 2010; 21 (5): p.745-52. doi: 10.1681/ASN.2009121238 . | Open in Read by QxMD
  18. Yuen J, Pluthero FG, Douda DN, et al. NETosing Neutrophils Activate Complement Both on Their Own NETs and Bacteria via Alternative and Non-alternative Pathways.. Frontiers in immunology. 2016; 7 : p.137. doi: 10.3389/fimmu.2016.00137 . | Open in Read by QxMD