Two Cases of Eosinophilic Granulomatosis With Polyangiitis Presenting With Rhinologic Manifestations

Article information

J Rhinol. 2026;33(2):120-125

Publication date (electronic) : 2026 July 31

doi : https://doi.org/10.18787/jr.2026.00009

Sae Hwan Park, MD, Chan Ho Yoon, MD, Dong Won Jung, MD, Woo Yong Bae, MD, PhD

Department of Otolaryngology-Head and Neck Surgery, Dong-A University College of Medicine, Busan, Republic of Korea

Address for correspondence: Dong Won Jung, MD, Department of Otolaryngology-Head and Neck Surgery, Dong-A University College of Medicine, 26 Daesingongwon-ro, Seo-gu, Busan 49201, Republic of Korea, Tel: +82-51-240-5423, Fax: +82-51-253-0712, E-mail: superbeam01@naver.com

Received 2026 February 23; Revised 2026 April 9; Accepted 2026 May 13.

Abstract

INTRODUCTION

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg–Strauss syndrome, is a rare necrotizing vasculitis affecting small- and medium-sized blood vessels throughout the body. The annual incidence of EGPA is extremely low, with approximately 1–3 cases per million individuals reported each year [1]. Bronchial asthma precedes disease onset in most patients, and otorhinolaryngologic manifestations, including allergic rhinitis, nasal polyps, and sinusitis, occur in more than 70% of cases [2]. Although symptoms such as nasal obstruction, rhinorrhea, and intranasal crusting substantially impair quality of life, they are often initially misdiagnosed as allergic diseases or chronic sinusitis because of their nonspecific clinical presentation.

In Korea, the first case of EGPA was reported in the internal medicine literature in 1986, and intermittent case reports have subsequently been published. However, cases diagnosed in the otorhinolaryngology setting based primarily on rhinologic symptoms remain extremely rare; one such case was reported by Jang et al. in 2014 [3]. We encountered two cases of EGPA presenting with nasal polyps and nasal septal perforation, respectively. Both patients were diagnosed through systemic evaluation and responded well to treatment. Herein, we report these cases and review the relevant literature.

CASE REPORT

Case 1

A 52-year-old woman presented with a multi-year history of recurrent nasal obstruction and nasal polyps that had been identified on nasal endoscopy at another hospital. In addition to nasal obstruction, she complained of postnasal drip, cough, and hyposmia. She had been diagnosed with asthma 25 years earlier and had been using an inhaled corticosteroid since that time.

Nasal endoscopy revealed multiple polyps in the nasal cavity along with nasal septal deviation (Fig. 1). Preoperative blood tests demonstrated an elevated peripheral eosinophil count of 700/μL (reference range: 35–300/μL) and a total immunoglobulin E (IgE) level of 76 kU/L (reference range: 0–200 kU/L). Paranasal sinus computed tomography (CT) revealed opacification of all paranasal sinuses (Fig. 2).

Fig. 1

Preoperative endoscopic findings. Multiple polyps are observed in both nasal cavities, with septal deviation.

Fig. 2

Computed tomography findings of the paranasal sinuses. Axial (A) and coronal images show pansinusitis (B).

The patient subsequently underwent nasal polypectomy and endoscopic sinus surgery. Histopathological examination of the surgical specimen demonstrated findings consistent with a simple nasal polyp, without evidence of vasculitis in the sinonasal tissue. However, on postoperative day 26, the patient developed hypoesthesia, pain, and weakness in both lower extremities, accompanied by an erythematous rash on the lower limbs (Fig. 3). Although cranial nerve examination findings were normal, nerve conduction studies revealed abnormalities suggestive of sciatic neuropathy involving both the motor and sensory nerves of the left lower extremity. Peripheral blood testing demonstrated leukocytosis (17,890/mm³) and a rapid increase in the eosinophil fraction to 41.0%. Autoimmune testing was positive for myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA). Biopsy of the skin lesion demonstrated ulcerative inflammation with perivascular eosinophilic infiltration. Although definitive necrotizing vasculitis was not identified in the specimen, histological examination confirmed the presence of extravascular eosinophilic infiltration. Chest CT demonstrated peripheral ground-glass opacities and pneumonic infiltrates in both lungs (Fig. 4).

Fig. 3

Multiple erythematous patches with bullae on both lower extremities.

Fig. 4

Chest radiographic and computed tomographic findings. A: Chest radiograph showing diffuse patchy infiltrates in both lower lung fields (black arrows) and blunting of the costophrenic angles, suggesting pleural effusion (white arrowheads). B: High-resolution computed tomography scan showing bilateral peripheral ground-glass opacities (black arrowheads) and pneumonic infiltrates in the lower lobes.

The patient fulfilled the 2022 American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) classification criteria for EGPA (a score of ≥6 is required for diagnosis) based on asthma, nasal polyps, eosinophilia (≥1×10⁹/L), mononeuritis multiplex, and extravascular eosinophilic infiltration [4]. Accordingly, the patient was diagnosed with EGPA. Systemic corticosteroid therapy with methylprednisolone (62.5 mg/day) was initiated as first-line treatment. Remission induction therapy consisting of oral corticosteroids and six cycles of cyclophosphamide (800 mg every 3 weeks) was subsequently administered. For maintenance therapy, the patient received weekly oral methotrexate (12.5 mg). Following treatment, the lower extremity symptoms and skin lesions improved markedly. The patient remains under outpatient follow-up at 8 months postoperatively without evidence of recurrence.

Case 2

A 39-year-old woman presented to the otorhinolaryngology outpatient clinic with worsening nasal obstruction, epistaxis, and nasal septal perforation that had been identified at another hospital 1 month earlier. Twenty-three years previously, she had presented to the Department of Pulmonology with cough and nocturnal wheezing and was subsequently diagnosed with bronchial asthma. One year after treatment initiation, she developed hemoptysis. Blood tests revealed marked eosinophilia, with an eosinophil fraction of 51.6%, and chest CT demonstrated focal nodular consolidation consistent with eosinophilic infiltration. Bronchoscopy with bronchoalveolar lavage cytology confirmed an eosinophil fraction of 61.0%, leading to the diagnosis of EGPA.

The patient was initially treated with low-dose oral corticosteroids. Following diagnosis, she experienced recurrent exacerbations of cutaneous symptoms, including rash and pruritus, as well as respiratory symptoms such as cough and sputum production. Each exacerbation was managed conservatively with high-dose systemic corticosteroids. Her most recent flare occurred 2 years before presentation to the otorhinolaryngology outpatient clinic.

Nasal endoscopy revealed a 1-cm perforation in the anterior nasal septum (Fig. 5). There was no history of nasal trauma or previous septal surgery. The characteristics of the perforation differed substantially from those typically observed in mechanical or traumatic perforations. Whereas traumatic perforations generally exhibit smooth, thin, and well-epithelialized margins, this lesion demonstrated irregular, thickened, and friable edges. The margins were covered with adherent hemorrhagic crusts, and the surrounding mucosa showed evidence of necrotizing inflammation rather than simple scarring. A biopsy was performed to differentiate malignancy from other granulomatous diseases and demonstrated chronic inflammation with subepithelial eosinophilic infiltration and necrosis. Despite conservative management, the patient was hospitalized 2 weeks later because of worsening cough, dyspnea, hemoptysis, and fever.

Fig. 5

Endoscopic findings of nasal cavity. A: Initial endoscopic view showing a septal perforation with thick hemorrhagic crusts and necrotic margins. B: Follow-up endoscopic view at 1 week showing persistent crusting and inflammation at the perforation site.

Laboratory testing demonstrated marked elevations in inflammatory markers and eosinophil counts, including a white blood cell count of 24,110/μL, eosinophil fraction of 56.0%, C-reactive protein level of 9.25 mg/dL (reference range: 0–0.5 mg/dL), and erythrocyte sedimentation rate of 41 mm/h (reference range: 0–30 mm/h). Antineutrophil cytoplasmic antibody (ANCA) testing was negative. Chest CT demonstrated multiple ground-glass opacities and interstitial consolidations in both lungs, whereas chest radiography revealed patchy consolidations and ground-glass opacities bilaterally (Fig. 6). Quantitative acid-fast bacilli staining and sputum culture results were negative.

Fig. 6

Chest radiographic and computed tomographic findings. A: Chest radiograph showing patchy consolidation and ground-glass opacities (black arrows) in the left lung and right lower lobe. B: High-resolution computed tomography scan showing multifocal ground-glass opacities (black arrowheads) and consolidations with interlobular septal thickening (white arrow), mainly in the left upper lobe.

Collectively, the patient fulfilled the 2022 ACR/EULAR classification criteria, with a total score of 10 based on underlying asthma (obstructive airway disease, +3 points), blood eosinophil count ≥1×10⁹/L (+5 points), and extravascular eosinophilic inflammation on biopsy (+2 points). She was diagnosed with an acute exacerbation of EGPA. Treatment with high-dose methylprednisolone (125 mg/day) and ceftriaxone (2 g/day) was initiated. Hemoptysis resolved on the third hospital day, and both respiratory symptoms and chest imaging findings improved by hospital day 16, permitting discharge. At 3 weeks after discharge, most symptoms, including nasal obstruction, epistaxis, and rhinorrhea, had improved, although postnasal drip persisted. Follow-up nasal endoscopy demonstrated improvement in crusting and mucosal inflammation, whereas the size of the septal perforation remained unchanged. The patient remains under outpatient follow-up 1 year later, with stable septal perforation and no evidence of crusting or active inflammation (Fig. 7).

Fig. 7

Follow-up endoscopic view at 1 year showing a nasal septal perforation without crusting or mucosal inflammation.

DISCUSSION

EGPA is a necrotizing vasculitis affecting small- and medium-sized vessels and was first described by Churg and Strauss in 1951 [1]. Although EGPA is rare, with an incidence of approximately 1–3 cases per million individuals annually, untreated disease may result in fatal multiorgan damage.

Lanham et al. [5] classified the clinical course of EGPA into three phases. The first, or prodromal, phase is characterized by allergic manifestations such as allergic rhinitis, nasal polyps, and asthma that may persist for several years or decades. The second, referred to as the eosinophilic phase, is characterized by eosinophilic infiltration of peripheral blood and tissues. The final phase, the vasculitic phase, manifests as systemic vasculitic involvement.

The 1990 ACR classification criteria were widely used previously [6]. Diagnosis required the presence of four or more of the following six criteria: 1) asthma, 2) eosinophilia (>10%), 3) neuropathy, 4) pulmonary infiltrates, 5) paranasal sinus abnormalities, and 6) extravascular eosinophils. However, in 2022, the ACR and EULAR proposed revised weighted classification criteria to improve diagnostic accuracy [4]. These updated criteria assign weighted scores to clinical findings, including obstructive airway disease (+3), nasal polyps (+3), and mononeuritis multiplex (+1), as well as laboratory findings such as blood eosinophil count ≥1×10⁹/L (+5) and cytoplasmic ANCA positivity (−3). A total score of ≥6 supports the classification of EGPA. Notably, the revised criteria assign substantial weight to nasal polyps, emphasizing their diagnostic significance, and reflect the observation that only 30%–40% of patients with EGPA are ANCA-positive, predominantly with MPO-ANCA positivity. Both patients in the present report exhibited asthma, nasal manifestations, and marked eosinophilia, allowing for straightforward classification according to the revised criteria.

Recent studies have suggested that EGPA demonstrates two clinically distinct phenotypes according to ANCA status [7]. In general, ANCA-positive patients, most commonly those with MPO-ANCA positivity, demonstrate more prominent small-vessel vasculitic manifestations, including peripheral neuropathy, glomerulonephritis, and purpura. In contrast, ANCA-negative patients more frequently exhibit cardiac involvement, pulmonary parenchymal infiltrates, and pleural effusions, likely driven by direct eosinophilic tissue infiltration. Our cases were consistent with these observations. Case 1, who was MPO-ANCA-positive, presented with mononeuritis multiplex accompanied by weakness and hypoesthesia of the lower extremities. In contrast, case 2, who was ANCA-negative, presented with severe respiratory manifestations, including extensive pulmonary infiltrates and bilateral pleural effusions, without neurological deficits.

Another noteworthy otorhinolaryngologic feature of EGPA is the appearance of the nasal mucosa. Unlike typical inflammatory nasal polyps, polyps associated with EGPA frequently exhibit crusting and are histologically characterized by marked eosinophilic infiltration and necrosis [8]. In case 2, given the absence of trauma or prior nasal surgery, the septal perforation was presumed to result from microvascular ischemia involving the septal mucosa and perichondrium secondary to vasculitis, ultimately leading to tissue necrosis. Septal perforation in EGPA is therefore considered a vasculitic process rather than the result of simple inflammatory extension. Necrotizing vasculitis affecting the microvasculature supplying the septal cartilage and mucosa may induce localized ischemia, resulting in necrosis and septal perforation. Accordingly, when septal perforation is identified in a patient without a history of trauma or surgery, particularly in the presence of crusting or necrotic mucosal changes, biopsy should be performed to differentiate EGPA from granulomatosis with polyangiitis or NK/T-cell lymphoma.

The cornerstone of treatment is high-dose systemic corticosteroid therapy. Although the initial response is generally favorable, relapse commonly occurs during corticosteroid tapering. The Five-Factor Score is widely used to assess prognosis, and poor prognostic factors include: 1) age >65 years, 2) cardiac involvement, 3) gastrointestinal involvement, 4) renal insufficiency (creatinine >1.5 mg/dL), and 5) absence of specific ear, nose, and throat manifestations [9]. In patients with poor prognostic factors or inadequate response to corticosteroid monotherapy, combination therapy with immunosuppressive agents such as cyclophosphamide or rituximab is recommended. Mortality among untreated patients was previously reported to reach 50% within 3 months; however, with appropriate treatment, the 5-year survival rate now exceeds 90% [10].

EGPA typically progresses through a prodromal phase lasting several years to decades before advancing to systemic vasculitis with potentially fatal multiorgan involvement. During this early phase, upper airway manifestations such as allergic rhinitis, chronic sinusitis, and nasal polyps are among the earliest and most common clinical findings. Otorhinolaryngologists frequently encounter patients during the prodromal or eosinophilic phases because approximately 70% of patients with EGPA present with sinonasal symptoms. Therefore, atypical intranasal findings, including refractory nasal polyps that are unresponsive to standard medical or surgical treatment, adult-onset asthma, or unexplained nasal septal perforation, should not be regarded solely as localized nasal disease. In such patients, clinicians should carefully evaluate for systemic manifestations, including generalized weakness, paresthesia, and skin rash, and should actively perform screening investigations such as peripheral eosinophil count, ANCA testing, and IgE measurement. If EGPA is suspected, systemic corticosteroids and immunosuppressive therapy should be initiated in consultation with rheumatology or pulmonology specialists.

In conclusion, otorhinolaryngologists play a critical role in the early diagnosis of EGPA because they are often the first clinicians to encounter prodromal upper airway manifestations. An active diagnostic approach that considers the possibility of underlying systemic vasculitis in patients presenting with early upper airway symptoms is essential for preventing irreversible multiorgan damage and improving long-term outcomes.

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