Granulomatosis with Polyangiitis (Wegener's)

Granulomatosis with polyangiitis (GPA), formerly Wegener's granulomatosis, is an ANCA-associated vasculitis defined by necrotizing granulomatous inflammation of the upper and lower respiratory tract combined with pauci-immune crescentic glomerulonephritis; c-ANCA targeting proteinase-3 (PR3) is positive in 75–90% of cases, and without treatment, historical median survival was just 5 months.

Table of Contents

  1. Overview and Classification
  2. Pathophysiology
  3. ENT and Upper Airway Manifestations
  4. Pulmonary Manifestations
  5. Renal Manifestations
  6. Other Organ Involvement
  7. Diagnosis and ANCA Testing
  8. Induction Therapy
  9. Maintenance Therapy and Relapse
  10. Prognosis and Special Situations
  11. References & Research
  12. Featured Videos

Overview and Classification

Granulomatosis with polyangiitis (GPA) was historically known as Wegener's granulomatosis, named after the German pathologist Friedrich Wegener (1907–1990), who first described the triad in the 1930s. In 2011, the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR) reached consensus to rename the disease GPA, in part due to later evidence of Wegener's ties to the Nazi party. The renamed term is now standard across major medical journals and clinical guidelines.

GPA belongs to the family of ANCA-associated vasculitides (AAV), which also includes microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome). GPA is predominantly associated with c-ANCA targeting proteinase-3 (PR3-ANCA), whereas MPA more often carries p-ANCA targeting myeloperoxidase (MPO-ANCA), and EGPA may carry either. The distinction matters because PR3-ANCA GPA has a higher relapse rate than MPO-ANCA disease.

The classic GPA triad is: necrotizing granulomatous inflammation of the upper respiratory tract, necrotizing granulomatous inflammation of the lower respiratory tract, and necrotizing (pauci-immune) glomerulonephritis. Not all three must be present at diagnosis — limited GPA refers to disease confined to the upper or lower airways without renal involvement. Incidence is approximately 3 per 100,000 persons per year. GPA occurs at any age but peaks between ages 65 and 74, with a slight male predominance; it disproportionately affects White populations. Before the era of cyclophosphamide, median survival was approximately 5 months. With modern therapy, complete remission is achievable in over 90% of patients, though the relapse rate remains approximately 50% at 5 years.

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Pathophysiology

The pathogenesis of GPA centers on anti-neutrophil cytoplasmic antibodies (ANCA). In GPA, approximately 75–90% of patients are positive for c-ANCA directed against proteinase-3 (PR3), a serine protease stored in neutrophil azurophilic granules. The remaining 10–25% are positive for p-ANCA targeting myeloperoxidase (MPO). ANCA titers correlate loosely with disease activity: a rising PR3-ANCA titer suggests an impending flare in many patients, but the relationship is not one-to-one, and approximately 50% of patients with a rising titer do not relapse — titers should inform, not dictate, clinical decisions.

The ANCA disease mechanism proceeds through a sequence of events. Pro-inflammatory cytokines including TNF-α, IL-1, and IL-18 prime circulating neutrophils, causing PR3 and MPO to translocate from intracellular granules to the neutrophil surface membrane. ANCA then binds these surface-expressed antigens, triggering full neutrophil activation. Activated neutrophils degranulate, releasing proteases and reactive oxygen species that cause direct endothelial injury, thrombosis, and inflammatory infiltration. Neutrophil extracellular traps (NETs) also contribute by presenting ANCA autoantigens in a highly immunogenic form.

Granuloma formation is the histological hallmark of GPA. CD4+ Th1 lymphocytes and macrophages accumulate around foci of necrotic tissue, forming multinucleated giant cell granulomas with characteristic "geographic necrosis" — irregular, map-like areas of tissue destruction surrounded by palisading histiocytes. These granulomas differ from the caseating granulomas of tuberculosis and the non-caseating granulomas of sarcoidosis, though all three can affect the lungs and may be confused radiographically.

A widely accepted two-hit model proposes: (1) an environmental trigger — most convincingly, nasal colonization by Staphylococcus aureus, which carries an 8-fold increased relapse risk in colonized GPA patients — disrupts immune tolerance and initiates ANCA production; (2) upon re-exposure or intercurrent inflammatory stress, circulating ANCA triggers the systemic vasculitic cascade. This model explains why trimethoprim-sulfamethoxazole (TMP-SMX), which suppresses nasal Staph carriage, reduces relapse rates in limited GPA.

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ENT and Upper Airway Manifestations

The upper respiratory tract is involved in approximately 90% of GPA patients at presentation and is often the initial site of disease. These manifestations can precede systemic vasculitis by months to years in limited GPA.

Nasal and Sinus Disease

Nasal involvement presents as chronic, bloody nasal crusting, recurrent epistaxis, nasal obstruction, and mucosal ulceration. Sinusitis — most commonly maxillary — is refractory to repeated courses of antibiotics and does not respond to standard treatments, a clinical clue that prompts further workup. CT imaging of the sinuses in GPA shows mucosal thickening, destruction of the nasal septum, and erosion of sinus walls, distinguishing it from simple chronic sinusitis. Progressive cartilage and bone destruction leads to septal perforation and ultimately the classic saddle nose deformity — a depressed nasal bridge from collapse of the nasal cartilage framework. This deformity, while cosmetically distressing, develops over months to years and is not present at initial diagnosis. Surgical reconstruction (rhinoplasty with cartilage grafts) is cosmetic and must be deferred until sustained remission of at least 12 months, as active inflammation will destroy any graft.

Subglottic Stenosis

Subglottic stenosis (SGS) develops in approximately one-third of GPA patients and is one of the most clinically dangerous upper airway manifestations. Circumferential granulomatous inflammation of the subglottis produces fibrous scarring that narrows the airway below the vocal cords. Symptoms include stridor, progressive dyspnea on exertion, exercise intolerance, and hoarseness. The critical point about subglottic stenosis is that it can progress — and even cause life-threatening airway compromise — even when systemic GPA is otherwise in remission. The fibrotic scar continues to contract independently of active vasculitis, meaning standard immunosuppressive therapy does not reliably prevent progression. Management is surgical: intralesional injection of triamcinolone followed by endoscopic dilation is the preferred approach. Tracheostomy is required in cases of critical stenosis. Regular laryngoscopy surveillance is essential in all GPA patients.

Ear Involvement

Serous otitis media from Eustachian tube dysfunction is common, producing conductive hearing loss. Tympanic membrane perforation and mastoiditis occur in more severe cases. Sensorineural hearing loss from vasculitis of the internal auditory artery is a less common but recognized complication. Cranial nerve VIII involvement should prompt consideration of GPA in any patient with unexplained mixed sensorineural and conductive hearing loss.

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Pulmonary Manifestations

The lungs are involved in 70–85% of GPA patients over the course of disease, though many pulmonary lesions are initially asymptomatic and discovered only on imaging performed for other reasons. When symptomatic, pulmonary GPA presents with cough (dry or productive), hemoptysis, dyspnea, and occasionally pleuritis with pleural effusion.

Pulmonary Nodules and Cavitation

The most characteristic pulmonary finding in GPA is the presence of bilateral cavitating nodules. These appear on chest CT as multiple, variably-sized nodules distributed throughout both lungs, often in the lower lobes. As nodules enlarge, their centers undergo geographic necrosis and eventually cavitate, producing a "crater" appearance sometimes described as a "craters of the moon" pattern. Cavitary lesions can be mistaken for fungal infection (aspergillosis, cryptococcosis), septic emboli, or malignancy. Tissue biopsy — often via bronchoscopy or CT-guided approach — is needed for definitive diagnosis when clinical context is insufficient.

Diffuse Alveolar Hemorrhage

Diffuse alveolar hemorrhage (DAH) is the most life-threatening pulmonary complication of GPA. It results from capillaritis — neutrophilic destruction of alveolar capillary walls — leading to flooding of alveolar spaces with red blood cells. The clinical triad is hemoptysis, progressive anemia, and bilateral infiltrates on chest imaging. However, hemoptysis may be absent in up to one-third of DAH cases because blood is trapped in the alveoli. Bronchoalveolar lavage (BAL) confirms the diagnosis: serial lavage aliquots return progressively bloodier fluid rather than clearing. Pulmonary function testing in DAH reveals an elevated DLCO because free hemoglobin in the alveoli avidly binds carbon monoxide — the paradoxical finding of high DLCO amid respiratory failure is a diagnostic clue. Management requires emergent high-dose intravenous glucocorticoids combined with rituximab or cyclophosphamide, and plasmapheresis is considered on a case-by-case basis.

Pulmonary Function Testing

Spirometry in GPA varies with disease location. Upper airway involvement produces a characteristic obstructive pattern on flow-volume loops with truncated inspiratory or expiratory flow (fixed obstruction from subglottic stenosis). Parenchymal disease with alveolar involvement reduces DLCO. The combination of obstructive spirometry (subglottic disease) and reduced DLCO (parenchymal disease) in the same patient is strongly suggestive of GPA.

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Renal Manifestations

Renal involvement is the most serious organ manifestation of GPA and is the primary determinant of long-term prognosis. The pathological lesion is necrotizing crescentic glomerulonephritis, characterized by fibrinoid necrosis of glomerular capillary loops, cellular crescents (proliferating parietal epithelial cells filling Bowman's space), and glomerular collapse. On immunofluorescence, the pattern is pauci-immune — little to no immunoglobulin or complement deposition — a key histological distinction from Goodpasture syndrome (linear IgG along the glomerular basement membrane) and lupus nephritis or IgA nephropathy (granular immune complex deposition).

Renal GPA can progress rapidly. Creatinine may double within days to weeks without treatment, and patients can progress from normal renal function to dialysis dependence within weeks if therapy is not initiated. Conversely, patients whose kidneys are already severely scarred at diagnosis are less likely to recover renal function regardless of treatment.

Clinical Findings

The urinalysis is the critical bedside test for detecting renal involvement. An active urinary sediment — the presence of dysmorphic red blood cells and red cell casts on urine microscopy — is the hallmark of active glomerulonephritis in GPA and demands urgent evaluation and treatment. Dipstick proteinuria and hematuria should trigger microscopic examination of fresh urine. Rising serum creatinine, even without dramatic urinary findings, warrants renal biopsy. Renal biopsy provides the definitive histological diagnosis, characterizes severity (percentage of crescents and sclerotic glomeruli), and informs prognosis: patients with more than 50% normal glomeruli at biopsy are substantially more likely to recover renal function than those with predominantly crescentic or sclerotic glomeruli.

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Other Organ Involvement

GPA is a systemic vasculitis capable of affecting virtually any organ. Recognition of these extrapulmonary, extrarenal manifestations is critical to avoid misattributing them to unrelated conditions and delaying treatment.

Ocular Involvement

Orbital pseudotumor (retro-orbital granulomatous inflammation) is the most common serious ocular manifestation, producing proptosis, diplopia, and periorbital pain from a retro-orbital inflammatory mass. Without treatment, compression of the optic nerve leads to irreversible blindness. Other ocular manifestations include scleritis (deep, painful inflammation of the scleral coat), episcleritis (milder superficial redness), uveitis, corneal ulceration, and dacryocystitis (lacrimal duct inflammation).

Cutaneous Involvement

Skin manifestations in GPA include palpable purpura of the lower extremities (leukocytoclastic vasculitis), subcutaneous nodules (extravascular granulomas histologically similar to rheumatoid nodules), and necrotic ulcers. Skin biopsy can provide diagnostic tissue in patients who present predominantly with cutaneous disease.

Neurological Involvement

Peripheral neuropathy — particularly mononeuritis multiplex, the simultaneous or sequential infarction of multiple individual peripheral nerves — produces painful, asymmetric sensory and motor deficits. Cranial nerve palsies involving cranial nerves VI (abducens, causing lateral gaze palsy), VII (facial), and VIII (vestibulocochlear, causing hearing loss and vertigo) are the most common. Pachymeningitis (granulomatous inflammation of the dura mater) is a rare but recognized cause of headache, cranial neuropathy, and cerebellar dysfunction in GPA.

Joint and Constitutional Features

Arthralgias or frank non-erosive arthritis occurs in approximately 60% of GPA patients, typically affecting large joints in a migratory pattern that parallels systemic disease activity. Constitutional features — fever, weight loss, and profound fatigue — are present in the majority of patients at diagnosis and often precede the recognition of organ-specific disease by weeks to months.

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Diagnosis and ANCA Testing

GPA diagnosis requires the integration of clinical features, serology, and histology. No single test is pathognomonic, and the 2022 revised ACR/EULAR classification criteria reflect this complexity. In a patient with a compatible clinical presentation — upper airway disease, pulmonary nodules, active urinary sediment, and positive PR3-ANCA — the diagnosis can be made with high confidence without biopsy. Conversely, in atypical presentations or seronegative disease, biopsy is essential.

ANCA Testing

Two complementary methods are used:

Current consensus recommends performing both IIF and antigen-specific ELISA simultaneously — dual testing improves sensitivity without unacceptably compromising specificity. A positive PR3-ANCA in a patient with sinusitis, pulmonary nodules, and an active urinary sediment is sufficient to diagnose GPA and begin treatment.

Biopsy

When tissue is needed, site selection follows a risk-benefit hierarchy. Nasal or sinus biopsy is the most accessible option but carries the lowest diagnostic yield — it often shows only nonspecific inflammation or ulceration without diagnostic granulomas. Open lung biopsy provides the highest diagnostic yield and demonstrates the full histological triad (necrotizing granulomas, vasculitis, and geographic necrosis), but requires general anesthesia and thoracotomy or VATS. Renal biopsy confirms pauci-immune crescentic GN and establishes the extent of renal involvement, but it cannot show pulmonary granulomas. Bronchoscopic transbronchial biopsy is often attempted but has limited yield for GPA-specific findings.

Laboratory and Imaging

Supporting laboratory findings include elevated ESR and CRP, normocytic anemia of chronic disease, leukocytosis, thrombocytosis, and elevated creatinine. Urinalysis with microscopy for casts must be performed at diagnosis and at every follow-up visit. Chest CT (preferred over plain radiography) demonstrates nodules, cavities, consolidation, and ground-glass opacities with greater sensitivity. PET-CT can map disease extent and guide biopsy site selection in difficult cases.

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Induction Therapy

The goal of induction therapy is complete remission — elimination of all detectable disease activity, ideally with ANCA negativity. Treatment intensity is calibrated to disease severity.

Rituximab Plus Glucocorticoids (Preferred for Generalized Disease)

Rituximab (RTX) combined with high-dose glucocorticoids is the preferred induction regimen for generalized or severe GPA, including patients with renal involvement, diffuse alveolar hemorrhage, or subglottic stenosis. The landmark RAVE trial (Stone et al., NEJM 2010, PMID 20647199) randomized 197 patients with active AAV to rituximab (375 mg/m² weekly ×4) versus oral cyclophosphamide; rituximab was non-inferior to cyclophosphamide for complete remission (64% vs. 53%), with a superior outcome in relapsing disease. Rituximab has become the preferred first-line agent over cyclophosphamide in most centers, particularly in younger patients and those wishing to preserve fertility.

Cyclophosphamide

Cyclophosphamide (CYC) remains an important option, particularly when rituximab is unavailable or contraindicated. It can be administered as intravenous pulse therapy or as daily oral dosing. The CYCLOPS trial (de Groot et al., Ann Intern Med 2009, PMID 19451574) demonstrated equivalent remission rates for IV pulse versus daily oral CYC, with lower cumulative dose and less bladder toxicity in the IV arm — making IV CYC the preferred formulation. Oral CYC carries a significant risk of hemorrhagic cystitis (from the urotoxic metabolite acrolein) and bladder cancer with cumulative use; mesna (2-mercaptoethane sulfonate sodium) must always be co-administered to protect the bladder.

Glucocorticoids

High-dose glucocorticoids are essential to the induction regimen. For severe disease, 3 days of IV methylprednisolone pulse (500–1000 mg/day) precedes transition to oral prednisone at approximately 1 mg/kg/day (maximum 80 mg/day). The PEXIVAS trial (Walsh et al., NEJM 2020, PMID 32053298) showed that a reduced-dose glucocorticoid regimen was non-inferior in efficacy to standard dosing while significantly reducing serious infections — an important finding that has shifted many centers toward lower initial steroid doses.

Plasma Exchange

Plasma exchange (PLEX) removes circulating ANCA and inflammatory mediators from the blood and was previously recommended for patients with severe renal failure (creatinine >5.7 mg/dL). However, the PEXIVAS trial found that adding PLEX to standard immunosuppression did not reduce the rate of end-stage kidney disease or death at 28 months. Current EULAR recommendations do not routinely recommend PLEX for renal GPA, though it may be considered case-by-case for dialysis-dependent patients or those with concurrent anti-GBM antibody disease.

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Maintenance Therapy and Relapse

Achieving remission is only the first challenge; maintaining it over the long term without intolerable toxicity is equally demanding. GPA carries a relapse rate of approximately 50% at 5 years, substantially higher than MPA, and relapses can cause permanent organ damage.

Rituximab Maintenance (Preferred)

The MAINRITSAN trial (Guillevin et al., NEJM 2014, PMID 25372085) established rituximab as the preferred maintenance agent over azathioprine. Patients with AAV in remission were randomized to rituximab 500 mg at months 0, 6, 12, and 18 versus azathioprine 2 mg/kg/day; rituximab was markedly superior (relapse rate 5% vs. 29% at 28 months). Most centers now use a fixed-interval rituximab schedule (500 mg or 1 g every 6 months) for 18–24 months after remission, with decisions guided by serial ANCA titers and clinical assessment.

Azathioprine

Azathioprine (AZA, 2 mg/kg/day) is a reasonable alternative maintenance agent when rituximab is unavailable or contraindicated, or when cost is prohibitive. Thiopurine methyltransferase (TPMT) genotyping or enzyme activity testing before initiation is essential: patients with absent TPMT activity are at risk for life-threatening myelosuppression at standard doses. The WEGENT trial (Pagnoux et al., NEJM 2008, PMID 19109574) demonstrated comparable maintenance efficacy between azathioprine and methotrexate in patients achieving remission with cyclophosphamide.

Methotrexate

Methotrexate (MTX, 20–25 mg/week orally or subcutaneously) is appropriate for maintenance in limited or non-severe GPA and can also be used for induction in non-severe disease (without significant renal involvement). It is contraindicated in patients with renal impairment due to the risk of methotrexate accumulation and toxicity.

Avacopan

Avacopan (Tavneos), an oral C5a receptor inhibitor, was FDA-approved in 2021 for AAV as a steroid-sparing adjunct to rituximab or cyclophosphamide. The ADVOCATE trial (Jayne et al., NEJM 2021, PMID 33596356) demonstrated that avacopan was non-inferior to prednisone at 26 weeks and superior at 52 weeks for sustained remission, with significantly fewer glucocorticoid-related adverse effects. Avacopan does not replace induction therapy but substantially reduces steroid exposure during induction and early maintenance.

TMP-SMX for Relapse Prevention and PCP Prophylaxis

Trimethoprim-sulfamethoxazole (TMP-SMX, one double-strength tablet three times weekly) serves two roles in GPA management. First, in patients with limited upper airway disease, it reduces relapse rates — likely by suppressing nasal S. aureus colonization (Stegeman et al., NEJM 1996, PMID 8637536). Second, all patients receiving cyclophosphamide or rituximab plus glucocorticoids should receive TMP-SMX as prophylaxis against Pneumocystis jirovecii pneumonia (PCP), a potentially fatal opportunistic infection in immunosuppressed AAV patients.

Monitoring for Relapse

Serial PR3-ANCA titers, urinalysis, serum creatinine, and chest imaging as clinically indicated are the mainstays of relapse monitoring. A rising PR3-ANCA predicts relapse in roughly 50% of cases — the other 50% of patients with rising ANCA do not relapse, meaning a rising titer alone should not trigger preemptive therapy without clinical correlates. Urinary sediment abnormalities or worsening pulmonary symptoms in a previously quiescent patient warrant urgent evaluation and possible re-biopsy.

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Prognosis and Special Situations

The prognosis of GPA has been transformed since the introduction of cyclophosphamide in the 1970s and rituximab in the 2000s. Five-year survival in the modern era exceeds 80–85%, and complete remission is achievable in more than 90% of patients with appropriate therapy. Nevertheless, the disease remains lifelong and carries significant burdens of relapse, infection, treatment toxicity, and permanent organ damage.

Causes of Death

Infection is the leading cause of early mortality in GPA — a direct consequence of profound immunosuppression with cyclophosphamide and high-dose glucocorticoids. Opportunistic infections including PCP, invasive aspergillosis, and disseminated herpes zoster account for a substantial proportion of early deaths. Antimicrobial prophylaxis and vaccination (pneumococcal, influenza, herpes zoster) are essential in all GPA patients receiving immunosuppression. Renal failure requiring long-term dialysis occurs in 10–20% of GPA patients despite best available therapy. Malignancy — bladder cancer and lymphoma — is an important late cause of death in patients who have received substantial cumulative cyclophosphamide exposure.

Permanent Organ Damage

Even in patients who achieve and sustain remission, permanent organ damage accumulates. Subglottic stenosis may require repeated endoscopic procedures or permanent tracheostomy. Saddle nose deformity is permanent absent surgical correction. Sensorineural hearing loss, visual impairment from orbital or ocular disease, and peripheral neuropathy may persist despite remission. The Vasculitis Damage Index (VDI) quantifies this cumulative burden and is used in clinical research and practice.

Pregnancy in GPA

Pregnancy in GPA carries high risk. Disease flares occur in approximately 50% of pregnancies, and the physiological immunomodulation of pregnancy may paradoxically worsen vasculitis in some patients. Rituximab is generally avoided in the third trimester due to neonatal B-cell depletion, though it has been used in the first and second trimesters when benefit clearly outweighs risk. Azathioprine is the preferred maintenance immunosuppressive during pregnancy, as it has the best safety profile among available agents. Cyclophosphamide is teratogenic and absolutely contraindicated in pregnancy. Methotrexate is teratogenic and must be discontinued at least 3 months before conception in both female and male patients.

Subglottic Stenosis as an Independent Entity

Subglottic stenosis deserves special emphasis because it behaves differently from other GPA manifestations. The fibrous scar tissue that narrows the subglottis is not driven by ongoing vasculitis; instead, it is a mechanical consequence of prior inflammation that contracts and progresses even when systemic GPA is fully quiescent. Systemic immunosuppression does not reliably prevent progression. Surgical dilation — with or without intralesional triamcinolone injection — provides the primary treatment, and patients often require multiple procedures over their lifetime. Tracheostomy provides definitive airway protection when dilation fails or when the stenosis is too severe for endoscopic management.

Saddle Nose Reconstruction

Correction of saddle nose deformity is purely cosmetic and does not affect disease prognosis. It must be deferred until the patient has been in stable, sustained remission for at least 12 months with no active nasal inflammation, as even subclinical disease activity will destroy cartilage grafts. When performed in the appropriate setting, rhinoplasty with rib or auricular cartilage grafts can provide excellent cosmetic outcomes.

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References & Research

Key Research Papers

  1. Stone JH, Merkel PA, Spiera R, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis (RAVE trial). N Engl J Med. 2010;363(3):221-232. PMID 20647199
  2. Guillevin L, Pagnoux C, Karras A, et al. Rituximab versus azathioprine for maintenance in ANCA-associated vasculitis (MAINRITSAN). N Engl J Med. 2014;371(19):1771-1780. PMID 25372085
  3. Walsh M, Merkel PA, Peh CA, et al. Plasma exchange and glucocorticoids in severe ANCA-associated vasculitis (PEXIVAS). N Engl J Med. 2020;382(7):622-631. PMID 32053298
  4. 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. PMID 33596356
  5. Fauci AS, Haynes BF, Katz P, Wolff SM. Wegener's granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med. 1983;98(1):76-85. PMID 6336643
  6. Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1-11. PMID 23045170
  7. Hoffman GS, Kerr GS, Leavitt RY, et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med. 1992;116(6):488-498. PMID 1739240
  8. de Groot K, Harper L, Jayne DR, et al. Pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody-associated vasculitis (CYCLOPS). Ann Intern Med. 2009;150(10):670-680. PMID 19451574
  9. Pagnoux C, Mahr A, Hamidou MA, et al. Azathioprine or methotrexate maintenance for ANCA-associated vasculitis (WEGENT). N Engl J Med. 2008;359(26):2790-2803. PMID 19109574
  10. Holle JU, Voigt C, Both M, et al. Orbital masses in granulomatosis with polyangiitis are associated with a refractory course and a high burden of local damage. Rheumatology. 2013;52(5):875-882. PMID 23329742
  11. 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. PMID 8637536
  12. Ntatsaki E, Carruthers D, Chakravarty K, et al. BSR and BHPR guideline for the management of adults with ANCA-associated vasculitis. Rheumatology. 2014;53(12):2306-2309. PMID 25099993

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Research Papers

The following PubMed topic searches retrieve current peer-reviewed literature on Granulomatosis with Polyangiitis. Each link opens a live PubMed query.

  1. Granulomatosis with polyangiitis treatment rituximab
  2. ANCA vasculitis cyclophosphamide induction
  3. Wegener granulomatosis diagnosis c-ANCA PR3
  4. GPA renal involvement glomerulonephritis
  5. Avacopan ANCA vasculitis steroid sparing
  6. Granulomatosis polyangiitis subglottic stenosis
  7. ANCA vasculitis relapse rituximab maintenance
  8. GPA saddle nose orbital pseudotumor
  9. Granulomatosis polyangiitis pulmonary manifestations
  10. PEXIVAS plasma exchange ANCA vasculitis

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Connections

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