Granulomatosis with Polyangiitis (GPA)

Table of Contents

1. Overview and History
2. Pathogenesis and ANCA Immunology
3. Classic Triad: Upper Respiratory Tract
4. Classic Triad: Lower Respiratory Tract and Kidneys
5. Additional Organ Involvement
6. Diagnosis
7. Treatment: Induction and Remission
8. Prognosis and Monitoring

Overview and History

Granulomatosis with Polyangiitis (GPA) was formerly called Wegener's granulomatosis. In 2011 the American College of Rheumatology (ACR), the American Society of Nephrology and the European League Against Rheumatism (EULAR) jointly recommended the descriptive name “granulomatosis with polyangiitis (Wegener’s)” in place of the eponym (Falk 2011). The change followed a wider move away from eponyms and research that had documented Friedrich Wegener’s Nazi Party membership and his wartime past (Woywodt and Matteson 2006).

GPA is one of the three major ANCA-associated vasculitides (AAV), alongside Microscopic Polyangiitis (MPA) and Eosinophilic Granulomatosis with Polyangiitis (EGPA). Its defining hallmarks are granulomatous inflammation and necrotizing vasculitis that primarily targets the upper respiratory tract, lower respiratory tract, and kidneys — the classic clinical triad.

• How common: a 2022 meta-analysis of 25 studies estimated a worldwide pooled GPA prevalence of 96.8 per million people and an incidence of 9.0 per million person-years, with ANCA-associated vasculitis more frequent in the northern hemisphere and rising over time (Redondo-Rodriguez 2022)
• Age: GPA can occur at any age; in the US National Institutes of Health (NIH) series of 158 patients, 85% were older than 19 (Hoffman 1992)
• Sex: men and women were equally represented in the NIH series (Hoffman 1992)
• Ancestry: 97% of the NIH series were white (Hoffman 1992)
• Prognosis: without therapy the prognosis is poor (Kitching 2020); in the NIH series treated mainly with daily cyclophosphamide and glucocorticoids, 13% died of the disease, its treatment or both over a mean follow-up of 8 years (Hoffman 1992). In a European Vasculitis Study Group cohort of 535 patients with ANCA-associated vasculitis, mortality was 2.6 times that of an age- and sex-matched general population (Flossmann 2011)

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Pathogenesis and ANCA Immunology

The disease is driven by anti-neutrophil cytoplasmic antibodies targeting proteinase 3 (PR3), producing a characteristic c-ANCA pattern on indirect immunofluorescence (IIF). Anti-PR3 ANCA is the dominant autoantibody in GPA and is found in most patients with active generalized disease; anti-MPO ANCA is found in a minority (Kitching 2020).

The two-hit model of ANCA-mediated vasculitis explains how these antibodies damage blood vessels:

1. Priming (first hit): Pro-inflammatory cytokines (IL-8, TNF-α) — released during infections or other stimuli — cause neutrophils to translocate PR3 and MPO from intracellular granules to the cell surface, making them accessible to circulating ANCA.
2. ANCA activation (second hit): ANCA binds surface-expressed PR3, activating primed neutrophils. Activated neutrophils adhere to vascular endothelium, degranulate, and release reactive oxygen species (ROS) and pro-inflammatory cytokines. This cascade produces vessel wall necrosis and tissue destruction characteristic of vasculitis.

The C5a–C5aR axis amplifies neutrophil priming through complement activation, providing the mechanistic rationale for avacopan, a selective C5a receptor (C5aR) antagonist; its approval rested mainly on a trial whose published report was retracted in 2026 (see Avacopan below).

Granuloma formation is mechanistically distinct from ANCA-mediated vasculitis. CD4+ Th1 and Th17 cells aggregate around areas of necrosis in the airways, forming the granulomas that give the disease its name. Effector T cells, not only ANCA, take part in the disease (Kitching 2020); this T-cell component is prominent in the upper respiratory tract.

Chronic nasal carriage of Staphylococcus aureus is linked to relapse: in a Dutch cohort of 57 patients, chronic carriers had a higher relapse risk (adjusted relative risk 7.16) (Stegeman 1994). The authors suggested a role for S. aureus in the disease’s pathophysiology. In a placebo-controlled trial in patients in remission, trimethoprim-sulfamethoxazole (TMP-SMX) reduced relapses (see TMP-SMX below) (Stegeman 1996).

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Classic Triad: Upper Respiratory Tract

Upper respiratory tract involvement is the presenting feature in the majority of GPA patients and is often mistaken for recurrent sinusitis or chronic rhinitis before the diagnosis is established. The destructive, treatment-resistant nature of these symptoms distinguishes GPA from common infections.

• Chronic sinusitis: persistent, antibiotic-resistant pansinusitis; CT imaging typically shows bony erosion rather than simple mucosal thickening, reflecting the underlying destructive granulomatous process
• Nasal manifestations: saddle-nose deformity (destruction of nasal cartilage causes collapse of the nasal bridge), nasal septal perforation, epistaxis, bloody nasal discharge, and crusting
• Otitis media: conductive hearing loss from Eustachian tube obstruction and middle ear effusion; facial nerve (cranial nerve VII) palsy can result from granulomatous involvement of the temporal bone
• Subglottic stenosis: narrowing of the trachea immediately below the vocal cords; one of the most feared upper airway complications; can cause progressive dyspnea, stridor, and respiratory failure; treatments reported include intralesional corticosteroid injection with dilation, and tracheostomy in severe cases; subglottic stenosis reflects granulomatous scarring rather than active vasculitis and can occur — or progress — even when systemic disease is in remission and ANCA titers are negative

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Classic Triad: Lower Respiratory Tract and Kidneys

Pulmonary and renal involvement define the severity spectrum of GPA. Together with upper respiratory disease, they form the classic triad and account for the bulk of early mortality when untreated.

Pulmonary Manifestations

• Pulmonary nodules: bilateral, multiple nodules that frequently cavitate; typically asymptomatic but may cause cough or hemoptysis
• Pulmonary infiltrates: ground-glass or consolidative opacities on CT; may represent active inflammation or hemorrhage
• Hemoptysis: ranges from blood-streaked sputum to massive hemoptysis
• Diffuse Alveolar Hemorrhage (DAH): pulmonary capillaritis causing widespread bleeding into alveoli; presents with hemoptysis, progressive hypoxemic respiratory failure, and a falling hematocrit; bronchoalveolar lavage (BAL) returns increasingly bloody fluid from all segments; DAH is a medical emergency with high early mortality, treated with urgent immunosuppression

Renal Manifestations: Rapidly Progressive Glomerulonephritis (RPGN)

Renal disease in GPA takes the form of pauci-immune crescentic glomerulonephritis — so named because immunofluorescence of kidney biopsy specimens shows few or no immune complex deposits (in contrast to the full-house immunofluorescence of lupus nephritis or the linear IgG of anti-GBM disease). Despite the absence of deposited immune complexes, ANCA-mediated neutrophil activation produces severe fibrinoid necrosis and crescent formation.

• Urinary findings: hematuria, red blood cell (RBC) casts (pathognomonic for glomerulonephritis), proteinuria
• Renal function: creatinine can rise over days to weeks, progressing to dialysis-requiring kidney failure if untreated — the hallmark of RPGN
• Without treatment: RPGN can progress to irreversible end-stage kidney disease (ESRD) within weeks, which is why the speed of diagnosis matters for kidney function

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Additional Organ Involvement

While the upper respiratory tract, lungs, and kidneys define the classic triad, GPA can involve virtually any organ system. Recognizing these additional manifestations is critical for diagnosis, particularly in atypical or limited presentations.

Eyes

• Orbital pseudotumor/proptosis: granulomatous mass behind the globe causing eye protrusion; can compress the optic nerve and cause irreversible visual loss
• Scleritis: painful, deep red eye with scleral inflammation; highly specific for systemic autoimmune disease
• Episcleritis, uveitis, dacryocystitis: variable inflammatory involvement of ocular structures
• Nasolacrimal duct obstruction: caused by granulomatous inflammation, resulting in chronic tearing and recurrent eye infections

Nervous System

• Mononeuritis multiplex: ischemia of individual peripheral nerves from vasculitis (e.g., foot drop from common peroneal nerve involvement, wrist drop from radial nerve)
• Cranial nerve palsies: variable cranial nerve involvement from granulomatous or vasculitic infiltration
• Pachymeningitis: granulomatous thickening of the dura mater causing persistent headache and multiple cranial nerve palsies

Skin

• Palpable purpura: leukocytoclastic vasculitis of dermal capillaries, typically on dependent areas (legs)
• Ulcers and papulonecrotic nodules: cutaneous manifestations of vasculitis

Joints

Joint pain (arthralgia) and non-erosive, migratory arthritis are common, often as an early feature before the diagnosis is established.

Disease Severity Classification

• Limited GPA: upper respiratory and/or pulmonary disease without significant renal involvement; generally lower immediate mortality
• Generalized GPA: includes active renal disease
• Severe GPA: organ- or life-threatening manifestations (DAH, RPGN, orbital compression with visual loss)

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Diagnosis

Diagnosis requires integrating clinical features, ANCA serology, imaging, urinalysis, and — whenever possible — tissue biopsy. No single test is sufficient.

ANCA Serology

• c-ANCA/anti-PR3: positive in most patients with active generalized disease, less often in limited disease or remission. A 2017 international consensus states that high-quality PR3- and MPO-ANCA immunoassays can be used as the primary screening test for GPA and MPA, without the categorical need for indirect immunofluorescence (Bossuyt 2017)
• Anti-MPO ANCA (p-ANCA pattern) is present in a minority of GPA cases and is more characteristic of MPA

Biopsy (Gold Standard)

• Lung biopsy (surgical/VATS): demonstrates necrotizing granulomatous vasculitis; in a review of 87 open lung biopsies, vascular changes were seen in 94% of specimens and parenchymal necrosis in 84% (Travis 1991)
• Kidney biopsy: shows pauci-immune crescentic glomerulonephritis with fibrinoid necrosis; confirms renal involvement and guides treatment urgency
• Upper airway/nasal biopsy: easy to obtain but often non-diagnostic; in 126 head and neck biopsies, vasculitis, necrosis and granulomatous inflammation were seen together in only 16% (Devaney 1990)

Urinalysis

Urinalysis with microscopy is a standard part of assessment at diagnosis and during follow-up. Hematuria with RBC casts indicates active glomerulonephritis, even when creatinine is still normal.

Imaging

• Sinus CT: bony erosion, pansinusitis (distinguishes GPA from simple bacterial sinusitis)
• Chest CT: nodules (with or without cavitation), ground-glass opacity (DAH), consolidation
• PET-CT: may delineate extent of inflammatory activity in atypical or widespread presentations

Classification Criteria

The 2022 ACR/EULAR classification criteria for GPA use a points-based score: bloody nasal discharge, crusting or sino-nasal congestion (+3); cartilaginous involvement (+2); conductive or sensorineural hearing loss (+1); c-ANCA or anti-PR3 positivity (+5); pulmonary nodules, mass or cavitation (+2); granuloma or giant cells on biopsy (+2); sinus inflammation or consolidation on imaging (+1); pauci-immune glomerulonephritis (+1); p-ANCA or anti-MPO positivity (−1); and an eosinophil count of at least 1 × 10⁹/L (−4). A patient already diagnosed with small- or medium-vessel vasculitis is classified as GPA at 5 points or more; in validation the criteria had 93% sensitivity and 94% specificity (Robson 2022). They are validated for research classification, not as diagnostic criteria for individual patients.

Differential Diagnosis

• Microscopic Polyangiitis (MPA): also pauci-immune GN but lacks granulomas and upper respiratory tract involvement; predominantly anti-MPO ANCA
• Anti-GBM disease (Goodpasture syndrome): linear IgG on kidney immunofluorescence; anti-GBM antibodies positive; can co-occur with ANCA
• Lymphomatoid granulomatosis: EBV-driven B-cell lymphoproliferative disorder involving lung and CNS; can mimic GPA on imaging
• Cocaine-induced midline destructive lesion (CIMDL): nasal septal and palate destruction from cocaine use. ANCA can be positive: in 25 patients with CIMDL, ANCA against human neutrophil elastase was found in 84% by at least one assay, and 57% of these were also PR3-ANCA positive, while patients with GPA were uniformly negative for elastase ANCA (Wiesner 2004); clinical history is key

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Treatment: Induction and Remission

Treatment is divided into two phases: induction (achieving remission rapidly to prevent organ damage) and maintenance (sustaining remission while minimizing cumulative drug toxicity). The approach depends on disease severity. The doses below are those used in the trials named, not instructions; decisions about treatment belong with a clinician.

Induction of Remission — Severe/Organ-Threatening Disease

• Rituximab + glucocorticoids: in the RAVE trial (Stone 2010) of 197 ANCA-positive patients with GPA or MPA, rituximab (375 mg/m² weekly for 4 weeks) was non-inferior to daily oral cyclophosphamide (2 mg/kg/day): 64% versus 53% reached remission without prednisone at 6 months. In relapsing disease rituximab did better (67% versus 42%), and adverse-event rates did not differ significantly. The 2021 ACR/Vasculitis Foundation guideline recommends rituximab for remission induction and maintenance in severe GPA and MPA (all its recommendations are conditional) (Chung 2021); the 2022 EULAR update recommends high-dose glucocorticoids with either rituximab or cyclophosphamide for organ- or life-threatening disease (Hellmich 2024). Cyclophosphamide’s toxicity to the ovaries and testes is one reason rituximab is often discussed for people who may want children.
• Cyclophosphamide + glucocorticoids: in the CYCLOPS trial (de Groot 2009) of 149 patients with generalized ANCA-associated vasculitis and kidney involvement, pulse cyclophosphamide (15 mg/kg every 2–3 weeks) and daily oral cyclophosphamide (2 mg/kg/day) produced similar remission rates at 9 months (88.1% versus 87.7%), with a lower cumulative dose in the pulse group.
• Glucocorticoid dose: in PEXIVAS, a reduced-dose oral glucocorticoid regimen was non-inferior to a standard-dose regimen for death or end-stage kidney disease, with fewer serious infections at 1 year (Walsh 2020).

Avacopan (Adjunct to Induction)

Avacopan, an oral selective complement 5a receptor (C5aR) antagonist, was approved by the US FDA in October 2021 as an adjunctive treatment for adults with severe active ANCA-associated vasculitis (GPA and MPA), in combination with standard therapy including glucocorticoids; the US label states that it does not eliminate glucocorticoid use, and the label dose is 30 mg orally twice daily. The approval rested mainly on the ADVOCATE trial (Jayne et al., NEJM 2021, PubMed PMID: 33596356), whose published report was retracted by the journal on 29 June 2026 (retraction notice PMID 42377355). The European Medicines Agency opened a review in January 2026 over the integrity of the ADVOCATE data and concluded in June 2026 that the trial was conducted in breach of good clinical practice and that the study data provided at the time of assessment were “incorrect and misleading” and could no longer be relied upon to show the medicine’s effectiveness; the EU marketing authorisation was revoked by European Commission decision on 4 August 2026 (EMA announcement, 26 June 2026). In the United States, the FDA’s Drugs@FDA record still listed the 2021 approval with prescription marketing status as of October 2026; whether the FDA has taken any action since the retraction could not be confirmed. The EMA’s review also states that avacopan is associated with an increased risk of drug-induced liver injury and vanishing bile duct syndrome, including fatal cases, particularly in the first three months of treatment (EMA referral record). The 2022 EULAR update, published before the retraction, had stated that avacopan may be considered as part of a strategy to reduce glucocorticoid exposure (Hellmich 2024).

Maintenance of Remission

• Rituximab: in the MAINRITSAN trial (Guillevin 2014) of 115 patients in remission after cyclophosphamide, rituximab 500 mg on days 0 and 14 and at months 6, 12 and 18 was compared with daily azathioprine; major relapse by month 28 occurred in 5% on rituximab versus 29% on azathioprine, with similar rates of severe adverse events. The 2021 ACR and 2022 EULAR guidance both recommend rituximab for maintenance (Chung 2021; Hellmich 2024).
• Azathioprine: in the CYCAZAREM trial (Jayne 2003), switching to azathioprine (2 mg/kg/day) after remission did not increase relapse compared with continued cyclophosphamide (15.5% versus 13.7% over 18 months). EULAR lists azathioprine and methotrexate as alternatives to biologics for maintenance (Hellmich 2024).

Plasma Exchange: The PEXIVAS Trial

The PEXIVAS trial (Walsh 2020) randomized patients with severe ANCA-associated vasculitis (low kidney function or diffuse pulmonary hemorrhage). Death or ESKD occurred in 100 of 352 patients (28.4%) with plasma exchange and 109 of 352 (31.0%) without it (hazard ratio 0.86, not statistically significant): plasma exchange did not reduce death or ESKD. The 2022 EULAR update states that plasma exchange may be considered in patients with rapidly progressive glomerulonephritis (Hellmich 2024).

TMP-SMX

Trimethoprim-sulfamethoxazole (TMP-SMX) has two roles. (1) Relapse prevention: in a placebo-controlled trial of patients with GPA in remission (41 on TMP-SMX 800/160 mg twice daily for 24 months, 40 on placebo), 82% stayed in remission at 24 months versus 60% on placebo (relative risk of relapse 0.40), with fewer respiratory infections; 20% stopped the drug because of side effects (Stegeman 1996). (2) Prophylaxis against Pneumocystis jirovecii pneumonia (PCP) during intensive immunosuppression.

Subglottic Stenosis

Subglottic stenosis is often treated by ENT surgeons separately from systemic immunosuppression, because the stenosis reflects fibrotic scarring rather than active vasculitis. In a series of 21 patients (64 procedures), intralesional injection of a long-acting corticosteroid (methylprednisolone acetate) followed by dilation kept the airway open, and none of the 21 needed a new tracheostomy; the authors describe it as their preferred therapy (Hoffman 2003). Tracheostomy is used for critical airway narrowing.

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Prognosis and Monitoring

Immunosuppression has turned a once rapidly fatal disease into a manageable, though relapsing, chronic illness. In the NIH series, 91% of patients improved markedly and 75% achieved complete remission (Hoffman 1992); in CYCAZAREM, 93% of patients with generalized disease entered remission (Jayne 2003).

Relapse

Relapse is common. In the NIH series, 50% of remissions were followed by one or more relapses (Hoffman 1992). A 2021 meta-analysis of patients with ANCA-associated vasculitis given cyclophosphamide induction estimated cumulative relapse at about 12% at 1 year, 33% at 3 years and 47% at 5 years, with higher 5-year relapse where more patients had GPA; PR3-ANCA positivity at diagnosis and a rise in ANCA were among the risk factors for relapse (He 2021). In a Dutch cohort, 22 of 33 patients who were persistently or intermittently ANCA-positive relapsed, against 1 of 21 who stayed negative (Stegeman 1994).

Damage Accrual

Even with effective treatment, GPA causes cumulative organ damage: chronic sinusitis and nasal deformity, conductive or sensorineural hearing loss, renal insufficiency from prior RPGN, peripheral neuropathy, and subglottic scarring. The VDI (Vasculitis Damage Index) is used in clinical research to quantify this accrued damage.

Infection

Infection is the leading cause of early death: in the same cohort of 535 patients, infection caused 48% of deaths in the first year, and active vasculitis 19%; after the first year the main causes were cardiovascular disease (26%), cancer (22%) and infection (20%) (Flossmann 2011). Intensive immunosuppression during induction raises the risk of PCP, invasive fungal infection and bacterial sepsis, which is why PCP prophylaxis with TMP-SMX (or atovaquone or dapsone in people allergic to sulfonamides) is used during cyclophosphamide- or rituximab-based induction.

Malignancy

Long-term cyclophosphamide use carries significant risks: hemorrhagic cystitis and bladder cancer (linked to its metabolite acrolein), myelodysplasia, and lymphoma. In 145 NIH patients treated with cyclophosphamide, half developed non-glomerular hematuria, 5% developed bladder cancer, and the estimated bladder-cancer incidence was 5% at 10 years and 16% at 15 years after first exposure; every patient who developed bladder cancer had earlier had non-glomerular hematuria (Talar-Williams 1996). That finding is the basis for continued urine testing after cyclophosphamide. Mesna and hydration are used to reduce bladder toxicity. Rituximab-based regimens and shorter cyclophosphamide courses have reduced cumulative cyclophosphamide exposure.

Laboratory Monitoring

Monitoring commonly described during and after treatment includes:

• Blood counts, serum creatinine, and urinalysis at regular intervals during active therapy
• ANCA (anti-PR3) levels during follow-up, since a rise is one of the risk factors for relapse (He 2021)
• Hepatitis B testing before rituximab, because rituximab can reactivate hepatitis B
• Immunoglobulin levels when infections recur on rituximab (hypogammaglobulinemia)
• Vaccinations: inactivated vaccines (pneumococcal, influenza) are usually brought up to date before rituximab; live vaccines are not given during rituximab therapy

Key Research Papers

1. Stone JH et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med. 2010;363(3):221-232. — PubMed PMID: 20647199 (RAVE trial)
2. Guillevin L et al. Rituximab versus azathioprine for maintenance in ANCA-associated vasculitis. N Engl J Med. 2014;371(19):1771-1780. — PubMed PMID: 25372085 (MAINRITSAN trial)
3. Jayne DRW, Merkel PA, Schall TJ, et al. Avacopan for the treatment of ANCA-associated vasculitis (ADVOCATE). N Engl J Med. 2021;384(7):599-609. — PubMed PMID: 33596356 — Retracted (retraction notice: PubMed PMID: 42377355)
4. Walsh M et al. Plasma exchange and glucocorticoids in severe ANCA-associated vasculitis. N Engl J Med. 2020;382(7):622-631. — PubMed PMID: 32053298 (PEXIVAS trial)
5. Jayne D et al. A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med. 2003;349(1):36-44. — PubMed PMID: 12840090 (CYCAZAREM)
6. Kitching AR, Anders HJ, Basu N, et al. ANCA-associated vasculitis. Nat Rev Dis Primers. 2020;6(1):71. — PubMed PMID: 32855422 (replaces an entry, “Guilpain P et al., Granulomatosis with polyangiitis, Presse Med 2013;42(4 Pt 2):651-659”, that could not be found in PubMed or Crossref)
7. Seo P, Stone JH. The antineutrophil cytoplasmic antibody-associated vasculitides. Am J Med. 2004;117(1):39-50. — PubMed PMID: 15210387
8. Lutalo PM, D'Cruz DP. Diagnosis and classification of granulomatosis with polyangiitis (aka Wegener's granulomatosis). J Autoimmun. 2014;48-49:94-98. — PubMed PMID: 24485158
9. Robson JC et al. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis. Arthritis Rheumatol. 2022;74(3):393-399. — PubMed PMID: 35106964
10. Hoffman GS et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med. 1992;116(6):488-498. — PubMed PMID: 1739240
11. Chung SA, Langford CA, Maz M, et al. 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheumatol. 2021;73(8):1366-1383. — PubMed PMID: 34235894 (replaces an entry, “Kronbichler A et al., Rituximab for granulomatosis with polyangiitis: clinical evidence and mechanisms of action, Ther Adv Musculoskel Dis 2015;7(3):79-90”, that could not be found in PubMed or Crossref)
12. McAdoo SP, Pusey CD. Anti-glomerular basement membrane disease. Clin J Am Soc Nephrol. 2017;12(7):1162-1172. — PubMed PMID: 28515156
13. Hellmich B, Sanchez-Alamo B, Schirmer JH, et al. EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update. Ann Rheum Dis. 2024;83(1):30-47. — PubMed PMID: 36927642
14. de Groot K, Harper L, Jayne DR, et al. Pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized trial. Ann Intern Med. 2009;150(10):670-680. — PubMed PMID: 19451574 (CYCLOPS trial)
15. Stegeman CA, Tervaert JW, de Jong PE, Kallenberg CG. Trimethoprim-sulfamethoxazole (co-trimoxazole) for the prevention of relapses of Wegener’s granulomatosis. N Engl J Med. 1996;335(1):16-20. — PubMed PMID: 8637536
16. Stegeman CA, Tervaert JW, Sluiter WJ, et al. Association of chronic nasal carriage of Staphylococcus aureus and higher relapse rates in Wegener granulomatosis. Ann Intern Med. 1994;120(1):12-17. — PubMed PMID: 8250451
17. Redondo-Rodriguez R, Mena-Vázquez N, Cabezas-Lucena AM, et al. Systematic review and metaanalysis of worldwide incidence and prevalence of antineutrophil cytoplasmic antibody (ANCA) associated vasculitis. J Clin Med. 2022;11(9):2573. — PubMed PMID: 35566698
18. He P, Hu JP, Tian XJ, et al. Prevalence and risk factors of relapse in patients with ANCA-associated vasculitis receiving cyclophosphamide induction: a systematic review and meta-analysis of large observational studies. Rheumatology (Oxford). 2021;60(3):1067-1079. — PubMed PMID: 33677596
19. Flossmann O, Berden A, de Groot K, et al. Long-term patient survival in ANCA-associated vasculitis. Ann Rheum Dis. 2011;70(3):488-494. — PubMed PMID: 21109517
20. Devaney KO, Travis WD, Hoffman G, et al. Interpretation of head and neck biopsies in Wegener’s granulomatosis. A pathologic study of 126 biopsies in 70 patients. Am J Surg Pathol. 1990;14(6):555-564. — PubMed PMID: 2337204
21. Travis WD, Hoffman GS, Leavitt RY, et al. Surgical pathology of the lung in Wegener’s granulomatosis. Review of 87 open lung biopsies from 67 patients. Am J Surg Pathol. 1991;15(4):315-333. — PubMed PMID: 2006712
22. Bossuyt X, Cohen Tervaert JW, Arimura Y, et al. Position paper: Revised 2017 international consensus on testing of ANCAs in granulomatosis with polyangiitis and microscopic polyangiitis. Nat Rev Rheumatol. 2017;13(11):683-692. — PubMed PMID: 28905856
23. Hoffman GS, Thomas-Golbanov CK, Chan J, et al. Treatment of subglottic stenosis, due to Wegener’s granulomatosis, with intralesional corticosteroids and dilation. J Rheumatol. 2003;30(5):1017-1021. — PubMed PMID: 12734898
24. Talar-Williams C, Hijazi YM, Walther MM, et al. Cyclophosphamide-induced cystitis and bladder cancer in patients with Wegener granulomatosis. Ann Intern Med. 1996;124(5):477-484. — PubMed PMID: 8602705
25. Wiesner O, Russell KA, Lee AS, et al. Antineutrophil cytoplasmic antibodies reacting with human neutrophil elastase as a diagnostic marker for cocaine-induced midline destructive lesions but not autoimmune vasculitis. Arthritis Rheum. 2004;50(9):2954-2965. — PubMed PMID: 15457464
26. Falk RJ, Gross WL, Guillevin L, et al. Granulomatosis with polyangiitis (Wegener’s): an alternative name for Wegener’s granulomatosis. Arthritis Rheum. 2011;63(4):863-864. — PubMed PMID: 21374588
27. Woywodt A, Matteson EL. Wegener’s granulomatosis—probing the untold past of the man behind the eponym. Rheumatology (Oxford). 2006;45(10):1303-1306. — PubMed PMID: 16887845

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Connections

• Rheumatology
• Microscopic Polyangiitis (MPA)
• Eosinophilic Granulomatosis with Polyangiitis (EGPA)
• Takayasu Arteritis
• Granulomatosis with Polyangiitis (Pulmonology) — the same disease from the respiratory side, with fuller detail on subglottic stenosis, diffuse alveolar hemorrhage, and the rituximab-versus-cyclophosphamide induction and maintenance choice.
• Lupus Nephritis
• Rapidly Progressive Glomerulonephritis
• Pulmonary Hemorrhage
• Vasculitis — the wider family of blood-vessel inflammation that GPA belongs to.

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