EGPA — Eosinophilic Granulomatosis with Polyangiitis

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a rare systemic necrotizing vasculitis of small and medium-sized blood vessels, classified within the ANCA-associated vasculitides (AAV) alongside granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). EGPA is distinguished by its nearly universal association with asthma and allergic disease, peripheral blood eosinophilia, and granulomatous inflammation. First described by pathologists Jacob Churg and Lotte Strauss in 1951 based on postmortem findings, EGPA typically evolves through three overlapping phases — prodromal (asthma/rhinitis), eosinophilic (organ eosinophilia), and vasculitic — though these phases are not always sequential or distinct. Cardiac involvement is the leading cause of mortality; mononeuritis multiplex the most common presenting neuropathy.

Table of Contents

  1. Overview
  2. Historical Recognition
  3. Pathophysiology and Immunology
  4. Three Clinical Phases
  5. Clinical Manifestations
  6. Diagnostic Criteria and Workup
  7. Treatment
  8. Differential Diagnosis
  9. Prognosis and Prognostic Scoring
  10. Recent Research and Advances
  11. References
  12. Connections
  13. Featured Videos

Overview

EGPA is the rarest of the three ANCA-associated vasculitides, with an estimated prevalence of 10–14 per million. It is strongly linked to allergic disease: virtually all patients have asthma, often severe and late-onset, and many have allergic rhinitis, nasal polyposis, and elevated serum IgE. The hallmark peripheral eosinophilia — greater than 10% of the white blood cell differential, or >1.5 × 10⁹/L absolute count — distinguishes EGPA from GPA and MPA. ANCA, specifically perinuclear ANCA (p-ANCA) targeting myeloperoxidase (MPO), is positive in approximately 30–40% of patients; ANCA-negative patients have a distinct clinical phenotype with more cardiac and gastrointestinal disease.

The annual incidence is approximately 1–3 per million; it is slightly more common in women. Mean age of diagnosis is in the fourth to fifth decade. Because EGPA is rare and its early phases mimic common allergic and asthmatic conditions, years may pass between symptom onset and systemic diagnosis. The average interval from asthma onset to vasculitis is 3–9 years, during which patients are often treated solely by allergists or pulmonologists.

ANCA status divides EGPA into two partially distinct phenotypes. ANCA-positive patients (predominantly MPO-ANCA, ~40%) resemble classical AAV with glomerulonephritis, alveolar hemorrhage, and purpura. ANCA-negative patients (~60%) have predominant eosinophilic organ damage, particularly cardiomyopathy and GI eosinophilia. These phenotypes likely reflect different pathophysiological mechanisms and have different responses to treatment, particularly to the anti-IL-5 agent mepolizumab, which is most effective in the eosinophilic-dominant subset.

Cardiovascular disease — especially eosinophilic myocarditis and endomyocardial fibrosis — is the leading cause of death in EGPA. Troponin elevation during active disease is a serious warning sign requiring immediate cardiac evaluation. The Five Factor Score (FFS) is a validated prognostic tool that stratifies patients by mortality risk and guides the intensity of initial immunosuppressive therapy.

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Historical Recognition

Jacob Churg (1910–2005) and Lotte Strauss (1913–1985) were pathologists at Mount Sinai Hospital in New York who published their landmark case series in 1951, describing 13 patients who died of a syndrome combining asthma, fever, eosinophilia, and systemic vasculitis at autopsy. They named the condition "allergic granulomatosis," recognizing the triad of necrotizing vasculitis, tissue eosinophilia, and extravascular granulomas as a distinct pathological entity. Both Churg and Strauss spent decades in New York, contributing significantly to cardiovascular and renal pathology. Their original paper, published in the American Journal of Pathology, remains a foundational reference in vasculitis medicine.

The eponym Churg-Strauss syndrome came into widespread clinical and research use and persisted for more than six decades. In 2012, the European Medicines Agency and leading vasculitis researchers proposed renaming the condition "eosinophilic granulomatosis with polyangiitis" (EGPA) to align with the descriptive naming convention of the other AAVs — granulomatosis with polyangiitis (GPA, replacing Wegener's granulomatosis) and microscopic polyangiitis (MPA). This was part of a broader initiative by the European Vasculitis Society and American College of Rheumatology (ACR) to move away from eponymous names for diseases associated with physicians whose historical conduct had been questioned, and to use names that convey pathological features rather than honor individuals.

Understanding of EGPA's immunopathology advanced substantially through the 1990s and 2000s, with the recognition of two distinct ANCA-associated and non-ANCA phenotypes, the central role of IL-5 in eosinophil-driven tissue damage, and the utility of the Five Factor Score for prognosis. The pivotal MIRRA trial (2017) demonstrated that mepolizumab, an anti-IL-5 monoclonal antibody, significantly reduced EGPA relapse rates and steroid burden, representing the first randomized controlled trial evidence for a biologic in EGPA and leading to FDA approval that year.

The ACR and European Alliance of Associations for Rheumatology (EULAR) published updated classification criteria for EGPA in 2022, replacing the 1990 ACR criteria and providing a modern, points-based system validated in large international cohorts. The 2022 criteria better reflect the two-phenotype model and include quantitative eosinophil thresholds, biopsy findings, and ANCA serology.

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Pathophysiology and Immunology

EGPA involves dysregulation of both innate (eosinophil-driven) and adaptive (T helper 2, Th2) immunity, combined with small-vessel vasculitis. The Th2 immune bias — characterized by elevated IL-4, IL-5, and IL-13 — drives eosinophil production (via IL-5) and IgE synthesis (via IL-4). Eosinophils recruited to tissues release major basic protein, eosinophil cationic protein, and reactive oxygen species, causing direct tissue damage — particularly to cardiac myocytes, peripheral nerves, and vascular endothelium. The coexistence of systemic vasculitis with this eosinophilic pattern is what makes EGPA unique among the AAVs.

The two-phenotype model separates ANCA-positive EGPA (MPO-ANCA, ~40%) from ANCA-negative EGPA (~60%). ANCA-positive patients have features more typical of classical AAV: glomerulonephritis, alveolar hemorrhage, mononeuritis multiplex, and purpura — injury driven primarily by ANCA-mediated neutrophil activation against vascular endothelium. ANCA-negative patients have predominant eosinophilic organ damage: cardiomyopathy, pulmonary infiltrates, and GI eosinophilia — reflecting direct eosinophil toxicity rather than neutrophil-driven small-vessel destruction. This molecular and phenotypic split may eventually justify classifying EGPA as two related but distinct diseases.

IL-5 is the central cytokine in EGPA pathogenesis — it is the primary eosinophil survival and maturation factor, promoting bone marrow eosinophilopoiesis and prolonging tissue eosinophil survival. This makes the IL-5/IL-5 receptor alpha (IL-5Rα) axis the most important therapeutic target in EGPA. Eosinophil-derived Charcot-Leyden crystals (composed of galectin-10 protein) are found in tissues. Granuloma formation — palisading histiocytes surrounding an eosinophilic necrotic core — distinguishes EGPA histologically from other eosinophilic disorders and is one of the cardinal diagnostic features on biopsy.

The trigger for EGPA remains incompletely understood. Molecular mimicry, chronic allergen stimulation, and environmental exposures have all been proposed. An early epidemiological observation linked leukotriene receptor antagonists (specifically montelukast) to EGPA, but this association is now thought to represent unmasking of subclinical EGPA rather than drug causation — steroid reduction with the introduction of montelukast exposed an underlying vasculitis that had been suppressed. Genetic associations include HLA-DRB1 alleles, and genome-wide association studies are beginning to identify susceptibility loci.

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Three Clinical Phases

EGPA classically evolves through three overlapping phases, though these phases are not rigidly sequential and patients may present at any stage or skip phases entirely. The three-phase model is a useful clinical framework but should not be applied too strictly in practice — particularly because the eosinophilic and vasculitic phases often coexist.

Prodromal phase spans an average of 3–9 years before vasculitis onset. It is dominated by atopic disease: asthma (often requiring oral steroids), allergic rhinitis, nasal polyposis, and sinusitis. The asthma in EGPA is characteristically adult-onset, appearing relatively late in life (30s–50s), and worsens in severity as the systemic phase approaches. Patients in the prodromal phase are typically managed by pulmonologists or allergists, and the eventual vasculitic diagnosis is usually not anticipated. Any adult with new-onset severe asthma — especially if accompanied by nasal polyposis and peripheral eosinophilia — warrants monitoring for systemic vasculitis.

Eosinophilic phase is characterized by peripheral blood eosinophilia exceeding 1,500 cells/μL, and often >5,000 or even >10,000/μL, along with eosinophilic tissue infiltration in multiple organs. Pulmonary eosinophilia — presenting as Löffler syndrome-like infiltrates, which are fleeting and migratory on chest X-ray — is characteristic. GI eosinophilia (eosinophilic gastroenteritis with abdominal pain, diarrhea, GI bleeding, or bowel perforation) is a serious complication. Eosinophilic myocarditis can develop silently during this phase, making cardiac monitoring with echocardiography and troponin essential even before overt vasculitis is present.

Vasculitic phase is defined by small-to-medium vessel inflammation causing characteristic end-organ damage. Constitutional symptoms — fever, profound fatigue, weight loss — are prominent. This phase is when mononeuritis multiplex, palpable purpura, renal disease, and the full spectrum of organ involvement becomes clinically apparent. The Five Factor Score should be calculated at vasculitic-phase diagnosis to stratify patients for treatment intensity. The eosinophilic and vasculitic phases often overlap, and many patients have evidence of eosinophilic tissue injury simultaneously with active vasculitic lesions.

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Clinical Manifestations

Constitutional: Fever, weight loss, and profound fatigue occur during active vasculitic disease and parallel disease activity. Night sweats and malaise are common.

Respiratory and ENT: Asthma is virtually universal — present in more than 95% of patients — and is typically severe, adult-onset, and often steroid-dependent. Allergic rhinitis, nasal polyposis, and recurrent sinusitis are common prodromal features. Pulmonary infiltrates on chest X-ray are characteristically fleeting and migratory (non-fixed, Löffler-type), distinguishing them from the fixed cavitary lesions of GPA. Alveolar hemorrhage is less common than in GPA or MPA but occurs in the ANCA-positive subset. Pleuritis and pleural effusions occur.

Nervous system: Mononeuritis multiplex is the most common neuropathy pattern in EGPA, affecting 50–75% of patients. This means simultaneous or sequential involvement of multiple, non-contiguous peripheral nerves — presenting as foot drop (peroneal nerve), wrist drop (radial nerve), or stepwise asymmetric sensorimotor deficits. The asymmetric pattern and acute onset distinguish it from the symmetric stocking-glove neuropathy of diabetic or nutritional neuropathies. Electromyography and nerve conduction studies confirm the diagnosis. Cranial neuropathies and CNS vasculitis are less common.

Cardiac (leading cause of mortality): Eosinophilic myocarditis — eosinophil infiltration of the myocardium — causes heart failure, arrhythmias, and sudden death. Late-stage endomyocardial fibrosis produces a restrictive cardiomyopathy. Constrictive pericarditis and coronary arteritis also occur. Cardiac involvement affects 16–92% of patients in autopsy series; clinical echocardiographic involvement is 14–26% in modern cohorts. Cardiac MRI is superior to echocardiography for detecting early myocardial inflammation and fibrosis. Troponin elevation during active disease is an ominous finding requiring urgent cardiac assessment.

Skin: Palpable purpura of the lower extremities is the hallmark cutaneous manifestation of small-vessel vasculitis. Subcutaneous nodules on extensor surfaces histologically show granulomas and are relatively specific for EGPA. Livedo reticularis and urticaria also occur.

Renal: Glomerulonephritis — typically segmental necrotizing GN with crescents on biopsy — occurs mainly in ANCA-positive patients. Renal involvement is generally less severe than in GPA or MPA; dialysis-requiring renal failure is uncommon. Microscopic hematuria and proteinuria on urinalysis are the first indicators.

Gastrointestinal: Eosinophilic gastroenteritis causes abdominal pain, diarrhea, and GI bleeding. Bowel perforation or ischemia from mesenteric vasculitis is rare but life-threatening and is a component of the Five Factor Score when severe.

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Diagnostic Criteria and Workup

ACR 1990 criteria (historical, for comparison): 4 of 6 criteria — asthma, eosinophilia >10%, neuropathy, pulmonary infiltrates, paranasal sinus abnormality, extravascular eosinophils on biopsy. Sensitivity 85%, specificity 99.7%. These criteria were designed for classification in clinical trials, not for clinical diagnosis, and are now superseded by the 2022 ACR/EULAR criteria.

ACR/EULAR 2022 classification criteria: A points-based system applied after excluding alternative diagnoses. Positive criteria: +3 for obstructive airway disease (asthma or reduced FEV1/FVC ratio); +3 for nasal polyps; +3 for mononeuritis multiplex; +2 for blood eosinophils >1 × 10⁹/L; +2 for extravascular eosinophilic-predominant inflammation on biopsy; +1 for blood eosinophils >5 × 10⁹/L. A score ≥5 classifies the patient as EGPA with high sensitivity and specificity. ANCA positivity for MPO subtracts points in some versions of the algorithm, reflecting that MPO-ANCA positivity points toward a GPA/MPA-like phenotype. These criteria are validated for research classification but serve as a useful clinical framework.

Laboratory workup: Complete blood count (peripheral eosinophilia — mark eosinophils >1,500/μL as diagnostically significant); ANCA panel (p-ANCA/MPO-ANCA by ELISA); urinalysis with microscopy and urine protein/creatinine ratio; serum creatinine; ESR and CRP; serum IgE (elevated); serum troponin (cardiac); chest X-ray and high-resolution CT chest; pulmonary function tests (obstructive pattern); nerve conduction studies/EMG (mononeuritis multiplex); echocardiography and cardiac MRI (myocardial inflammation and fibrosis); bronchoalveolar lavage (BAL eosinophilia >25% strongly supports diagnosis).

Tissue biopsy: Skin (purpuric lesions showing small-vessel vasculitis with eosinophilic infiltrate); kidney (segmental necrotizing GN with crescents in ANCA-positive disease); sural nerve (vasculitis of vasa nervorum with eosinophilia); lung (wedge biopsy for extravascular granulomas and eosinophilic infiltration). Finding extravascular necrotizing granulomas with eosinophilic necrosis is pathognomonic. Not all biopsies show all three hallmarks simultaneously.

Five Factor Score (FFS): Validated prognostic scoring system assigning one point each for: proteinuria >1 g/24h; creatinine >140 μmol/L; severe GI involvement (bleeding, perforation, infarction); cardiomyopathy; central nervous system involvement. FFS 0 = low risk (5-year mortality ~12%); FFS 1 = intermediate risk (~26%); FFS ≥2 = high risk (~46%). Guides intensity of initial immunosuppressive regimen — particularly the decision to add cyclophosphamide to glucocorticoids.

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Treatment

Glucocorticoids (first line): High-dose prednisone 1 mg/kg/day (up to 60–80 mg/day) for 4–6 weeks, followed by a slow taper over 12–18 months. Patients with mild disease (FFS 0) and no organ-threatening features may be managed with glucocorticoids alone. Many patients require ongoing low-dose steroids to control asthma and systemic inflammation; complete steroid discontinuation is achieved in only a minority. IV methylprednisolone pulses (500–1000 mg/day × 3 days) are used for severe, life-threatening presentations.

Cyclophosphamide: Added to glucocorticoids for patients with FFS ≥1 or organ-threatening disease (cardiac, renal, severe neuropathy). IV pulse cyclophosphamide (15 mg/kg every 2–3 weeks for 6–9 pulses) is preferred over daily oral dosing for equivalent efficacy with lower cumulative toxicity. After cyclophosphamide induction (typically 6 months), transition to a less toxic maintenance agent — azathioprine or methotrexate — for 12–24 months.

Mepolizumab (Nucala): Anti-IL-5 monoclonal antibody — FDA approved in 2017 for relapsing or refractory EGPA based on the MIRRA trial (Wechsler et al., N Engl J Med 2017). Mepolizumab 300 mg subcutaneously every 4 weeks significantly reduced relapse rates compared with placebo and allowed steroid tapering. It is most effective in ANCA-negative, eosinophil-predominant phenotype. Mechanism: blocks IL-5, preventing eosinophil maturation and tissue survival. Mepolizumab does not replace glucocorticoids entirely and is best used as a steroid-sparing maintenance agent in relapsing disease.

Rituximab (anti-CD20): An emerging alternative to cyclophosphamide for induction in ANCA-positive EGPA, and a proven option for relapsing or refractory vasculitic disease. Rituximab depletes B cells, reducing ANCA-producing plasmablasts and downstream T-cell activation. Standard dosing: 375 mg/m² IV weekly × 4, or 1000 mg IV × 2 doses 2 weeks apart. Evidence in EGPA is largely observational and from case series; randomized trial data are less robust than for GPA/MPA.

Maintenance immunosuppression: Azathioprine (2 mg/kg/day) or methotrexate (15–25 mg/week, with folic acid supplementation) are used after cyclophosphamide induction. Mycophenolate mofetil is an alternative for patients intolerant of azathioprine. Duration of maintenance is typically 18–24 months, but many patients require longer-term treatment due to relapse on tapering.

Benralizumab (anti-IL-5Rα): Under active investigation in clinical trials for EGPA. Benralizumab binds IL-5 receptor alpha and additionally induces antibody-dependent cytotoxicity against eosinophils — a mechanism distinct from mepolizumab's ligand blockade. The MANDARA trial is comparing benralizumab vs mepolizumab head-to-head in EGPA.

Cardiac management: Aggressive monitoring with troponin, echocardiography, and cardiac MRI. Standard heart failure therapy for cardiomyopathy. Anticoagulation for intracardiac thrombus (a complication of eosinophilic endocardial involvement). Corticosteroid therapy is the primary treatment for eosinophilic myocarditis; immunosuppression must be sustained until cardiac markers normalize.

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Differential Diagnosis

Granulomatosis with polyangiitis (GPA): More prominent upper airway destruction (saddle nose deformity, nasal septal perforation), PR3-ANCA rather than MPO-ANCA, cavitary pulmonary nodules, and severe glomerulonephritis. Absence of asthma and peripheral eosinophilia. Biopsy shows necrotizing granulomatous inflammation without eosinophilic predominance.

Microscopic polyangiitis (MPA): MPO-ANCA positive, pauci-immune rapidly progressive glomerulonephritis, alveolar hemorrhage. No asthma, no eosinophilia, no granulomas on biopsy. The distinction from ANCA-positive EGPA can be difficult and may hinge on the presence of asthma and the degree of eosinophilia.

Hypereosinophilic syndrome (HES): Chronic eosinophilia (>1,500/μL for >6 months) causing end-organ damage — particularly cardiac (identical Löffler endocarditis pattern) and neurological. No asthma, no ANCA, no vasculitis histologically. FIP1L1-PDGFRA fusion gene-positive HES (myeloproliferative variant) responds to imatinib.

Eosinophilic pneumonia (Löffler syndrome, chronic eosinophilic pneumonia): Pulmonary eosinophilia without systemic vasculitis. Fleeting infiltrates on imaging, peripheral blood eosinophilia, and bronchoalveolar lavage eosinophilia are shared features, but there is no mononeuritis multiplex, purpura, cardiac vasculitis, or ANCA seropositivity. Responds rapidly to steroids.

Drug-induced eosinophilia and vasculitis: Leukotriene receptor antagonists (montelukast), macrolide antibiotics, and hydralazine are reported triggers. The current consensus is that montelukast unmasks subclinical EGPA by allowing steroid reduction rather than causing de novo disease.

Parasitic infections: Strongyloides stercoralis, Toxocara canis, and Ascaris lumbricoides cause peripheral eosinophilia that can reach very high levels. Serology (anti-Toxocara IgG, Strongyloides ELISA), stool examination, and travel history distinguish these from EGPA. Strongyloides must always be excluded before starting high-dose immunosuppression to avoid hyperinfection syndrome.

Allergic bronchopulmonary aspergillosis (ABPA): Asthma, eosinophilia, pulmonary infiltrates, and elevated IgE overlap with EGPA prodromal phase. Aspergillus-specific IgE/IgG serology, bronchiectasis, and mucus plugging on CT distinguish ABPA.

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Prognosis and Prognostic Scoring

With modern treatment, five-year survival in EGPA exceeds 90%, a dramatic improvement from the ~50% five-year survival reported in early pre-biologic series. The Five Factor Score remains the most clinically validated prognostic tool. Patients with FFS 0 at diagnosis have a low early mortality risk and may be managed with glucocorticoids alone, whereas FFS ≥2 mandates aggressive combination immunosuppression from the outset.

Cardiac involvement at diagnosis is the single most important adverse prognostic factor. Eosinophilic myocarditis — if recognized and treated early — can show substantial recovery; endomyocardial fibrosis established late in disease is largely irreversible. Sudden death from arrhythmia is a recognized presentation of EGPA cardiac involvement, and patients with troponin elevation or wall motion abnormalities on echocardiography require urgent cardiac MRI and intensified treatment.

Relapse is common in EGPA — approximately 35–50% of patients relapse during or after glucocorticoid taper. Relapse risk is highest in ANCA-positive patients and in those who required oral steroids to control asthma even before systemic disease developed. Predictors of relapse include persistently elevated eosinophils during remission, proximal airway involvement, and inability to achieve complete steroid discontinuation. Mepolizumab has meaningfully reduced relapse rates in the eosinophilic phenotype.

Mononeuritis multiplex frequently improves with aggressive early treatment, but residual neurological deficits — particularly distal sensory loss and mild motor weakness — are common. Foot drop and wrist drop may not fully recover. Physiotherapy and occupational therapy are important components of long-term management for neuropathy. Asthma frequently persists even during complete vasculitic remission and requires ongoing pulmonary management independent of systemic immunosuppression.

Long-term steroid toxicity — osteoporosis, diabetes, cataracts, infection risk, and adrenal insufficiency — is a major management burden, particularly in older patients. Bone protection (calcium, vitamin D, bisphosphonate) should be started with high-dose corticosteroids. Steroid-sparing strategies and early transition to mepolizumab or rituximab maintenance are increasingly favored to reduce cumulative glucocorticoid exposure.

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Recent Research and Advances

MIRRA trial (2017): The pivotal randomized, double-blind, placebo-controlled trial demonstrating that mepolizumab 300 mg SQ every 4 weeks significantly reduced relapse rates (28% vs 56% placebo), increased the proportion of patients achieving remission, and allowed steroid tapering in EGPA. Published by Wechsler et al. in the New England Journal of Medicine in 2017. This trial led to FDA approval of mepolizumab for EGPA and established IL-5 blockade as a disease-modifying treatment.

ACR/EULAR 2022 classification criteria: Updated and validated criteria replacing the 1990 ACR criteria, incorporating the two-phenotype model, quantitative eosinophil thresholds, biopsy findings, and ANCA serology into a points-based system. These criteria were developed using a large international cohort and are now the standard for clinical trial inclusion and case classification.

MANDARA trial: An active or recently reported head-to-head randomized controlled trial comparing benralizumab (anti-IL-5Rα, which depletes eosinophils directly via ADCC) against mepolizumab (anti-IL-5 ligand) in EGPA. Results are emerging and will clarify whether receptor blockade with direct eosinophil depletion is superior to ligand blockade in the EGPA eosinophilic phenotype.

Two-phenotype model confirmation: Large registry studies — including the French Vasculitis Study Group cohort (383 patients, Comarmond et al. 2013) and subsequent multi-center registries — have confirmed the clinical, serological, and prognostic differences between ANCA-positive and ANCA-negative EGPA. These data increasingly support treating the two phenotypes as distinct disease entities with different therapeutic strategies.

Cardiac MRI: Cardiac MRI with late gadolinium enhancement (LGE) demonstrates subclinical myocardial involvement — including non-ischemic fibrosis and active myocarditis patterns — in a substantial proportion of EGPA patients at diagnosis, even in the absence of symptoms or echocardiographic abnormalities. This finding underscores the importance of routine cardiac MRI at diagnosis for all EGPA patients, not only those with clinical cardiac symptoms.

FDG-PET/CT for disease mapping: Positron emission tomography with CT is increasingly used in EGPA to map active eosinophilic inflammation and vasculitis across the body and to monitor treatment response. FDG-PET may detect occult cardiac or vascular involvement and identify biopsy targets in patients with cryptic disease activity.

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References

  1. Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol. 1951;27(2):277–301. PMID 14819645
  2. Masi AT, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome. Arthritis Rheum. 1990;33(8):1094–1100. PMID 2202307
  3. Wechsler ME, et al. Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis. N Engl J Med. 2017;376(20):1921–1932. PMID 28514601
  4. Grayson PC, et al. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for eosinophilic granulomatosis with polyangiitis. Ann Rheum Dis. 2022;81(3):309–314. PMID 35110333
  5. Guillevin L, et al. A prospective, multicenter, randomized trial comparing steroids and pulse cyclophosphamide versus steroids and oral cyclophosphamide in the treatment of generalized Wegener's granulomatosis. Arthritis Rheum. 1997;40(12):2187–2198. PMID 9416855
  6. Groh M, et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) consensus task force recommendations for evaluation and management. Eur J Intern Med. 2015;26(7):545–553. PMID 26166009
  7. Comarmond C, et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): clinical characteristics and long-term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort. Arthritis Rheum. 2013;65(1):270–281. PMID 23044708
  8. Guillevin L, et al. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients. Medicine (Baltimore). 1996;75(1):17–28. PMID 8569467
  9. Dunogué B, et al. Churg-Strauss Syndrome: Clinical Symptoms, Complementary Investigations, Prognosis and Outcome, and Treatment. Semin Respir Crit Care Med. 2011;32(3):298–309. PMID 21674411
  10. Zwerina J, et al. Churg-Strauss syndrome in childhood: a systematic literature review and clinical comparison with adult patients. Semin Arthritis Rheum. 2009;39(2):108–115. PMID 18701138
  11. Schiavon F, et al. The role of echocardiography in Churg-Strauss syndrome. Reumatismo. 2012;63(4):178–185. PMID 22303604
  12. Vaglio A, et al. Eosinophilic granulomatosis with polyangiitis: mimicking diseases, mimicked diseases, and overlap syndromes. Rheumatology (Oxford). 2015;54(1):62–70. PMID 24489064

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Connections

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