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ObjectiveTo develop and validate revised classification criteria for eosinophilic granulomatosis with polyangiitis (EGPA).MethodsPatients with vasculitis or comparator diseases were recruited into an international cohort. The study proceeded in five phases: (1) identification of candidate criteria items using consensus methodology, (2) prospective collection of candidate items present at the time of diagnosis, (3) data-driven reduction of the number of candidate items, (4) expert panel review of cases to define the reference diagnosis and (5) derivation of a points-based risk score for disease classification in a development set using least absolute shrinkage and selection operator logistic regression, with subsequent validation of performance characteristics in an independent set of cases and comparators.ResultsThe development set for EGPA consisted of 107 cases of EGPA and 450 comparators. The validation set consisted of an additional 119 cases of EGPA and 437 comparators. From 91 candidate items, regression analysis identified 11 items for EPGA, 7 of which were retained. The final criteria and their weights were as follows: maximum eosinophil count ≥1×109/L (+5), obstructive airway disease (+3), nasal polyps (+3), cytoplasmic antineutrophil cytoplasmic antibody (ANCA) or anti-proteinase 3–ANCA positivity (−3), extravascular eosinophilic predominant inflammation (+2), mononeuritis multiplex/motor neuropathy not due to radiculopathy (+1) and haematuria (−1). After excluding mimics of vasculitis, a patient with a diagnosis of small- or medium-vessel vasculitis could be classified as having EGPA if the cumulative score was ≥6 points. When these criteria were tested in the validation data set, the sensitivity was 85% (95% CI 77% to 91%) and the specificity was 99% (95% CI 98% to 100%).ConclusionThe 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis demonstrate strong performance characteristics and are validated for use in research.

ObjectiveTo develop and validate revised classification criteria for granulomatosis with polyangiitis (GPA).MethodsPatients with vasculitis or comparator diseases were recruited into an international cohort. The study proceeded in five phases: (1) identification of candidate criteria items using consensus methodology, (2) prospective collection of candidate items present at the time of diagnosis, (3) data-driven reduction of the number of candidate items, (4) expert panel review of cases to define the reference diagnosis and (5) derivation of a points-based risk score for disease classification in a development set using least absolute shrinkage and selection operator logistic regression, with subsequent validation of performance characteristics in an independent set of cases and comparators.ResultsThe development set for GPA consisted of 578 cases of GPA and 652 comparators. The validation set consisted of an additional 146 cases of GPA and 161 comparators. From 91 candidate items, regression analysis identified 26 items for GPA, 10 of which were retained. The final criteria and their weights were as follows: bloody nasal discharge, nasal crusting or sino-nasal congestion (+3); cartilaginous involvement (+2); conductive or sensorineural hearing loss (+1); cytoplasmic antineutrophil cytoplasmic antibody (ANCA) or anti-proteinase 3 ANCA positivity (+5); pulmonary nodules, mass or cavitation on chest imaging (+2); granuloma or giant cells on biopsy (+2); inflammation or consolidation of the nasal/paranasal sinuses on imaging (+1); pauci-immune glomerulonephritis (+1); perinuclear ANCA or antimyeloperoxidase ANCA positivity (−1); and eosinophil count ≥1×109 /L (−4). After excluding mimics of vasculitis, a patient with a diagnosis of small- or medium-vessel vasculitis could be classified as having GPA if the cumulative score was ≥5 points. When these criteria were tested in the validation data set, the sensitivity was 93% (95% CI 87% to 96%) and the specificity was 94% (95% CI 89% to 97%).ConclusionThe 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for GPA demonstrate strong performance characteristics and are validated for use in research.

BackgroundRecently published classification criteria for eosinophilic granulomatosis with polyangiitis (EGPA) [1] require, to be applied, a diagnosis of small- or medium-vessel vasculitis, without specifying how that diagnosis should be made. Histologically, EGPA is characterized by eosinophil-rich and necrotizing granulomatous inflammation together with necrotizing vasculitis, predominantly affecting small to medium vessels. Some of these features are not specific for EGPA and may be seen in other forms of ANCA associated vasculitis (AAV). Current guidelines on the management of AAV recommend biopsies to assist in establishing a new diagnosis and for further evaluation of patients with suspected relapse [2]. Therefore, biopsies are essential tools for the diagnosis, classification and management of these diseases.ObjectivesTo describe the histopathological findings, diagnostic yield and the most common localizations of biopsies performed to assist in the diagnosis and follow-up of patients with EGPA.MethodsMedical charts of patients with EGPA regularly followed at our department were reviewed to retrieve the number and localization of biopsies, as well as biopsy findings including proportion of a) eosinophil-rich extravascular inflammation, b) granulomas and/or c) necrotizing vasculitis. Biopsies were performed if patients presented symptoms in any given and most accessible localization (at diagnosis) or if they were necessary to confirm active disease (during follow-up).ResultsAmong 59 EGPA patients regularly controlled at our department, a total of 163 biopsies were performed on 54 of them. In 44 patients (81.5%), 89 biopsies were performed at diagnosis and in 27 (50%), 74 biopsies were performed during follow-up. A total of 37 biopsies in 26 patients (48.1%) had proven vasculitis. A total of 7 biopsies in 5 patients (9.3%) had proven granuloma. A total of 87 biopsies in 42 patients (77.8%) had proven eosinophil-rich extravascular inflammation. The main localizations were: ear, nose and throat (ENT, 49 biopsies), respiratory (31), skin (25), gastrointestinal (GI, 18), muscular (14), nerve (12), bone marrow (8), renal (4) and temporal artery biopsy (TAB, 2). Among them, at least 1 typical histological feature detailed above was found in: ENT 38/49 biopsies (sensitivity of 77.6%), respiratory 15/31 (48.4%), skin 23/25 (92%), GI 10/18 (55.6%), muscular 4/14 (28.6%), nerve 5/12 (41.7%), bone marrow 6/8 (75%), renal 4/4 (100%) and TAB 1/2 (50%). A total of 57 biopsies performed in 29 patients (53.7%) showed none of the typical findings described above, although only 6 patients had all their biopsies without abnormal findings. These results are summarized in Table 1.ConclusionMultiple tissues are biopsied in clinical practice to assist in the diagnosis and evaluation of patients with EGPA, according to the multi-organ nature of this disease. Biopsies are more frequently obtained from accessible sites such as ENT or skin and usually dictated by clinical manifestations. In our cohort, the highest sensitivity was obtained from kidney, skin, ENT, bone marrow? and GI tract.References[1]Grayson et al, Ann Rheum Dis 2022[2]Yates et al, Ann Rheum Dis 2016Table 1.Biopsies in the EGPA cohort (N=59)Patients with biopsy: 54 (91.5%)At diagnosis: 44 (81.5%); 89 biopsies During follow-up: 27 (50%); 74 biopsiesHistological findingsVasculitisPatients with vasculitis: 26 (48.1%)At diagnosis: 22 (40.7%)During follow-up: 5 (9.3%)GranulomaPatients with granuloma: 5 (9.3%)At diagnosis: 3 (5.6%)During follow-up: 2 (3.7%)Eosinophil-rich inflammationPatients with eosinophil-rich inflammation: 42 (77.8%)At diagnosis: 31 (57.4%)During follow-up: 23 (42.6%)Main localizations of biopsies, n (with at least 1 histological finding, n)At diagnosisDuring follow-upTotalENT21 (16)28 (22)49 (38)Respiratory13 (6)18 (9)31 (15)Skin15 (14)10 (9)25 (23)Gastrointestinal3 (3)15 (7)18 (10)Muscular13 (4)1 (0)14 (4)Nerve12 (5)0 (0)12 (5)Bone marrow6 (5)2 (1)8 (6)Renal4 (4)0 (0)4 (4)Vascular (temporal artery biopsy)2 (1)0 (0)2 (1)AcknowledgementsThis study was supported by Instituto de Salud Carlos III (ISCIII) through the project PI18/00461, part of Plan Estatal de Investigación Científica y Técnica y de Innovación 2013–2016, and co-funded by the European Union. Roberto Ríos-Garcés is a recipient of a Río Hortega grant from Instituto de Salud Carlos III, Spain (CM19/00032).Disclosure of InterestsRoberto Ríos-Garcés: None declared, José Hernández-Rodríguez: None declared, Sergio Prieto-González: None declared, Maria C. Cid Paid instructor for: Vifor, GSK, Consultant of: GSK, Abbvie and Janssen, Grant/research support from: Kiniksa, Georgina Espígol-Frigolé Consultant of: Janssen.

BackgroundIn 2022, the ACR and the EULAR proposed new classification criteria for granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) based on numerical item scoring for different manifestations in ANCA-associated vasculitis (AAV). Recently, a low concordance rate of only 73% in comparison with the 2012 revised International Chapel Hill Consensus Conference Nomenclature and the 2007 European Medicines Agency (EMA) algorithm [1] was reported in a Korean cohort of 65 GPA patients.ObjectivesWe performed a retrospective classification according to the new 2022 classification criteria of patients from our multi-centric AAV cohort, using a computational algorithm that involved existing clinical, laboratory and histological entries. These results were compared to prior clinical diagnoses.MethodsData of AAV patients from four tertiary referral centers (Germany and Switzerland) were collected between 2000 and 2021. The 2022 ACR/EULAR criteria for GPA and MPA were applied by algorithmically analyzing (1) BVAS entries for organ manifestation, (2) laboratory results for ANCA/ ELISA testing and (3) histological data for detection of granuloma and/or pauci-immune glomerulo-nephritis. Twenty cases with inconclusive ANCA status were excluded from computed analysis. Results were compared to previously reported diagnoses; whereby renal limited disease was considered a distinct entity.ResultsThe final dataset included 305 cases, 294 (96.4%) of Caucasian ethnicity, 161 males (52.8%), median age 61 years (IQR 50-70). Based on the 2022 ACR/EULAR criteria, 299 (98%) cases could be unambiguously categorized, 180 patients were classified as GPA and 119 as MPA (Table 1). Positive concordance, i.e. patients matching the novel classification criteria and clinical diagnosis, was higher in MPA than in GPA patients (98.8 vs. 90.8%). By contrast, negative concordance, i.e. proportion of cases negative for both clinical and novel classification-based diagnosis, was higher in GPA (97.3 vs. 84.1%). Twenty-seven cases of renal limited disease were mostly reclassified as MPA (92%). Taken together, alignment of matching both, positive and negative cases in relation to all cases was 93.1% in GPA and 88.2% in MPA. Six cases (2%) that were clinically diagnosed as GPA could not be classified due to competing findings of increased eosinophil cell count and anti-proteinase-3 positivity.ConclusionIn the present multi-centric cohort, our computational algorithm applying the novel 2022 ACR/EULAR criteria based on existing clinical, laboratory and histological data performed well to categorize 98% of all cases. Our findings demonstrated overall positive and negative concordance rates above 88% and suggest a higher specificity for GPA, but higher sensitivity for MPA classification criteria.Reference[1]Pyo JY, Ahn SS, Song JJ, et al. Reclassification of previously diagnosed GPA patients using the 2022 ACR/EULAR classification criteria. Rheumatology (Oxford) Published Online First: 4 May 2022. doi:10.1093/RHEUMATOLOGY/KEAC267Table 1.EntityClinical Diagnosisn (%)2022 criteria-basedn (%)CasesnConcordance(%)GPA195 (63.9)180 (59)++17790.8+–18–+3––10797.3both positive and negative matching cases for GPA (284/305):93.1MPA85 (27.9)119 (32.8)++8498.8+–1–+35––18584.1both positive and negative matching cases for MPA (269/305):88.2Not classified06 (2)GPAMPARenal limited disease25 (8.2)2 (8)23 (92)Acknowledgements:NIL.Disclosure of InterestsStefan Krämer: None declared, Thomas Rauen Consultant of: Vifor, Kristian Pruin: None declared, Teresa Anslinger: None declared, Martin Busch: None declared, Tobias Schmitt: None declared, Raoul Bergner Speakers bureau: Abbvie, Bristol Myers Squibb, Chugai, Glaxo Smith Kline, Galapagos, MSD, Novartis, Consultant of: Galapagos, Vifor, Sebastian Mosberger: None declared, Thomas Neumann Speakers bureau: Roche Pharma (Suisse) AG, GlaxoSmithKline AG, Celgene Switzerland, UCB-Pharma AG, Janssen-Cilag AG, Vifor Pharma Switzerland, Novartis Switzerland, Consultant of: Roche Pharma (Suisse) AG, GlaxoSmithKline AG, Celgene Switzerland, AstraZeneca GmbH, Janssen-Cilag AG, Amgen Switzerland AG, Vifor Pharma Switzerland, Grant/research support from: Vifor Pharma Switzerland.

Eosinophilic Granulomatosis with Polyangiitis (EGPA) is a rare multisystemic disease classified both amongst hypereosinophilic disorders and ANCA-associated vasculitis. Vessel inflammation and eosinophilic proliferation are the hallmarks of the disease and main effectors of organ damage. Two distinct disease phenotypes have classically been described according to ANCA-status: the ANCA-negative subset with eosinophil-driven manifestation and the ANCA-positive one with vasculitic manifestations. An analogous dichotomization has also been backed by histological findings and a distinct genetic background. EGPA is typically consider a Th2-mediated disease and blood and tissue eosinophilia represent the cornerstone of diagnosis. Besides, ANCA are known for inducing endothelial injury and vascular inflammation by activating the circulating neutrophils. Thus, the pathogenesis of EGPA seems to be mediated by two coexisting mechanisms. However, the verbatim application of this strict dualism cannot always be translated into routine clinical practice. In the present review we describe the current knowledge on the eosinophilic and ANCA-mediated aspects of EGPA pathogenesis. Finally, we review the rationale of the currently proposed EGPA dichotomy and future research perspectives.

CD30-positive T-cell lymphoproliferative disorders are classified as cutaneous (primary cutaneous anaplastic large cell lymphoma and lymphomatoid papulosis) or systemic. As extent of disease dictates prognosis and treatment, patients with skin involvement need clinical staging to determine whether systemic lymphoma also is present. Similar processes may involve mucosal sites of the head and neck, constituting a spectrum that includes both neoplasms and reactive conditions (eg, traumatic ulcerative granuloma with stromal eosinophilia). However, no standard classification exists for mucosal CD30-positive T-cell lymphoproliferations. To improve our understanding of these processes, we identified 15 such patients and examined clinical presentation, treatment and outcome, morphology, phenotype using immunohistochemistry, and genetics using gene rearrangement studies and fluorescence in situ hybridization. The 15 patients (11 M, 4 F; mean age, 57 years) had disease involving the oral cavity/lip/tongue (9), orbit/conjunctiva (3) or nasal cavity/sinuses (3). Of 14 patients with staging data, 7 had mucosal disease only; 2 had mucocutaneous disease; and 5 had systemic anaplastic large cell lymphoma. Patients with mucosal or mucocutaneous disease only had a favorable prognosis and none developed systemic spread (follow-up, 4–93 months). Three of five patients with systemic disease died of lymphoma after 1–48 months. Morphologic and phenotypic features were similar regardless of extent of disease. One anaplastic lymphoma kinase-positive case was associated with systemic disease. Two cases had rearrangements of the DUSP22-IRF4 locus on chromosome 6p25.3, seen most frequently in primary cutaneous anaplastic large cell lymphoma. Our findings suggest mucosal CD30-positive T-cell lymphoproliferations share features with cutaneous CD30-positive T-cell lymphoproliferative disorders, and require clinical staging for stratification into primary and secondary types. Primary cases have clinicopathologic features closer to primary cutaneous disease than to systemic anaplastic large cell lymphoma, including indolent clinical behavior. Understanding the spectrum of mucosal CD30-positive T-cell lymphoproliferations is important to avoid possible overtreatment resulting from a diagnosis of overt T-cell lymphoma.

BackgroundThe diagnostic workup of ANCA-associated vasculitis (AAV) is a challenge due to the possible multi-organ involvement and the wide range of differential diagnosis. Before classification, vasculitis needs to be proofed by clinical or histopathologic signs.ObjectivesWe aimed to evaluate specific histopathologic features of organ biopsies and their contribution to the diagnosis of vasculitis and to the classification of specific AAV subgroups.MethodsRetrospective, single-centre cohort study in patients with granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA), classified by ACR-criteria, who have received at least one organ biopsy. Characteristic histopathologic features were analysed and compared between AAV subgroups, organ systems and ANCA-status (Chi-square-test).Results306 patients (GPA n=154, MPA n=58, EGPA n=94) diagnosed between 1990–2017 were included. All biopsies were taken at active stage of GPA, MPA or EGPA at initial diagnosis (n=415) or during flair of the disease (n=36). 168 patients had a renal biopsy, 185 patients had 283 non-renal biopsies (1 biopsy in 102 pts, 2 different organ biopsies in 68 pts,>2 in 15 pts). In kidney biopsies glomerulonephritis was described in 78.6%, unspecific inflammation in 26.8% and normal tissue in 1.2%. In non-renal biopsies vasculitis, granuloma, tissue eosinophilia, unspecific inflammation or normal tissue were reported in patients with GPA 32.9/29.4/21.2/71.8/9.4%, MPA 27.3/9.1/27.3/90.9/9.1% and EGPA 20.2/10.1/67.4/73.0/20.2%; p<0.0001 (lung n=103, 13.6/13.6/37.9/71.8/3.9%; skin n=35, 42.8/11.4/40.0/51.4/1.4%; upper respiratory tract n=98, 19.3/16.3/37.7/72.4/8.2%; peripheral nerves n=7, 14.3/14.3/0/14.3/42.8%; p<0.0001). According to the ANCA status the distribution was 31.1/25.2/28.3/68.9/10.7% in ANCA+patients and 20.8/11.1/62.5/79.2/19.4% in ANCA- patients (p<0.0001). Diagnosis of vasculitis was based on biopsy result in 2% of GPA, none of MPA and 8% of EGPA patients, but ACR criteria were only fulfilled including a characteristic biopsy in 6.6% of GPA and 35.5% of EGPA patients. Assignment to GPA, MPA and EGPA by application of the EMA algorithm were only possible in consideration of a characteristic non-renal biopsy in 2.8/0/21.9% and of a characteristic non-renal and/or renal biopsy in 4.7/14.6/28.1% of all patients.ConclusionsHistologic proof of vasculitis remains the gold standard of AAV diagnostic, however the diagnostic value is most prominent for renal biopsies. Distribution of various histopathologic features is different among AAV subgroups (GPA, MPA, EGPA) and varies between different organ biopsies. While classification of GPA and MPA is only in a few cases based on histopathology, in EGPA a characteristic histopathology is necessary for classification in almost one third of patients.Disclosure of InterestNone declared

Eosinophilic angiocentric fibrosis is a rare indolent lesion of the head and neck region that has characteristic histologic findings of onionskin fibrosis and prominent eosinophils. Its pathogenesis has been poorly understood and has been most commonly attributed to hypersensitivity or previous trauma. Recently, the lesion has been included in the spectrum of immunoglobulin G4 (IgG4)–related disease. However, few of the existing cases of eosinophilic angiocentric fibrosis have been evaluated for IgG4+ and IgG+ plasma cells. Therefore, we provide an update on the clinical and histologic features of eosinophilic angiocentric fibrosis to increase awareness of the entity and encourage its further characterization as an IgG4-related disease.

BackgroundClassification criteria for the ANCA-associated vasculitides (AAVs) were developed in the 1980s prior to the use of ANCA testing and newer imaging techniques. The Diagnostic and Classification of the Systemic Vasculitides (DCVAS) study is an international project to update classification criteria for the systemic vasculitides.ObjectivesDevelopment of draft classification criteria for Granulomatosis with Polyangiitis (GPA), Microscopic Polyangiitis (MPA) and Eosinophilic Granulomatosis with Polyangiitis (EGPA).MethodsThree phases: 1) Expert panel review of cases to identify gold standard set of new cases of small vessel vasculitis; 2) Item reduction of >8000 individual DCVAS items using data-driven and consensus methodology; 3) Lasso logistic regression models within each development set comparing each of the AAV types to other small and medium vessel vasculitides. Final criteria derived through clinical consensus, tested in validation set. The classification project has received financial support from the ACR and EULAR.ResultsThe expert review process approved 2072/2871 (72%) of physician diagnosed DCVAS cases, including [724 GPA, 291 MPA, 226 EGPA, 51 polyarteritis nodose (PAN), 220 other small vessel disease (SVV)]. Data driven and expert consensus resulted in 91 items retained. Draft criteria, and sensitivity and specificity in table 1.Abstract OP0021 – Table 1Draft classification criteria for the ANCA-associated vasculitides. *Cartilagenous involvement: Inflamed ear or nose cartilage or hoarse voice/stridor, endobronchial involvement or saddle nose deformityGranulomatosis with polyangiitis (GPA)Microscopic polyangiitis (MPA)Eosinophilic granulomatosis with polyangiitis (EGPA) Blood nasal discharge, ulcers, crusting, congestion or blockage, or septal defect/perforation+3Pauci-immune glomerulonephritis+3Obstructive airways diseases+3Cartilagenous involvement*+2Bloody nasal discharge, ulcers, crusting, congestion or blockage, septal defect/perforation −3Nasal polyps+3Conductive or sensorineural hearing loss+1pANCA or MPO-antibody positive+6Mononeuritis multiplex or motor neuropathy+1Pauci-immune glomerulonephritis+1Fibrosis or ILD on chest imaging+3Eosinophil count≥1×109/L+5cANCA or PR3-antibody+5cANCA or PR3-antibody −1Extravascular eosinophilic predominant inflammation/eosinophils in bone marrow+2pANCA or MPO-antibody −1Eosinophil count≥1×109/L −4cANCA or PR3-antibody −3Eosinophil count≥1×109/L −4Microscopic haematuria −1Granuloma, extravascular granulomatous inflammation, or giant cells on biopsy+2Nodules, mass, cavitation on chest imaging+2Inflammation, consolidation, or effusion of the nasal/paranasal sinuses on imaging+1Total score of≥5 is needed for classification Sensitivity 93%, Specificity 94%Total score of≥6 is needed for classificationSensitivity 87%, Specificity 96%)Total score of≥5 is needed for classificationSensitivity 88%, Specificity 98%ConclusionsDraft classification criteria for GPA, MPA and EGPA have been created which reflect current practice and have good sensitivity and specificity.AcknowledgementsDCVAS sites and expert panel membersDisclosure of InterestNone declared

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of systemic disease characterized by inflammation and necrotizing effects of the small and medium blood vessels. It is a vasculitis found in all age groups and both genders, although its etiology is unknown. The mean age at diagnosis is 40 years, consisting of an uncommon cause of vasculitis in people older than 65 years. It is the least common of the three antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (EGPA, granulomatosis with polyangiitis (GPA), and microscopic polyangiitis). The characteristic features of EGPA include extravascular eosinophilic granulomas, peripheral eosinophilia, and asthma, usually responsive to steroid treatment. In this article, we discuss a case of an 83-year-old male with a history of undetermined etiology of chronic kidney disease, chronic obstructive pulmonary disease (COPD), and severe chronic rhinosinusitis with nasal polyposis. First hospitalized with the suspicion of community-acquired pneumonia (CAP), based on worsening blood eosinophilia and unresolving respiratory symptoms, a suspicion for EGPA was raised. The development of an eosinophilic pleural effusion, later upon admission, was a predominant factor for its confirmation, as it constitutes a rare finding, only present in about 30% of patients. Laboratory tests showed elevated IgE, the presence of antineutrophil cytoplasmic antibodies directed against myeloperoxidase with a perinuclear staining pattern (ANCA-MPO), and the absence of antiproteinase 3 (anti-PR3) ANCA, which were consistent with the diagnosis. A pleural biopsy was then made, revealing fibrosis with the presence of eosinophils, although with no evidence of granulomas. According to the most recent and accepted classification criteria, the \"2022 American College of Rheumatology and European Alliance of Associations for Rheumatology (ACR/EULAR) for EGPA,\" this patient presented with a score of 13 (a score greater than or equal to 6 is needed for the classification of EGPA). Hence, a diagnosis of EGPA was assumed, and the patient was initiated on corticosteroid therapy, with a favorable response. The aim of this article is to present a rare case of EGPA diagnosis made at the age of 83 years old, although there was evidence that could point to this disease years before the diagnosis was made. In the present case, it is important to point out the long diagnostic delay in a geriatric patient, much older than the median age of diagnosis for EGPA, culminating in a curious case of uncommon pleuroparenchymal involvement.