ReviewRevisiting the systemic vasculitis in eosinophilic granulomatosis with polyangiitis (Churg-Strauss): A study of 157 patients by the Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires and the European Respiratory Society Taskforce on eosinophilic granulomatosis with polyangiitis (Churg-Strauss)☆
Introduction
Eosinophilic granulomatosis with polyangiitis (EGPA) (formerly Churg-Strauss syndrome) is defined as an eosinophil-rich and necrotizing granulomatous inflammation often involving the respiratory tract, with necrotizing vasculitis predominantly affecting small to medium-sized vessels, occurring in patients with asthma and eosinophilia [1]. Although early studies [2] reported on cases of late stage disease with florid systemic manifestations, the diagnosis is now more frequently made before multiple severe organ involvement [3].
Although the terminology of Churg-Strauss syndrome was replaced by EGPA, ANCA are present in only about 40% of patients in recent series [4], [5], [6], [7], [8]. Although nonspecific and not validated as diagnostic biomarkers, ANCA when present are usually considered contributive to EGPA diagnosis. Since many patients lack confident criteria of documented systemic vasculitis and/or are ANCA negative [9], the current terminology and classification require further refinement.
Recently, several studies have described phenotypes of clinical manifestations of EGPA patients according to their ANCA status [4], [5], [6], [8], [10], [11]. Patients with ANCA more frequently have a “vasculitic” phenotype (i.e. more frequent glomerulonephritis, alveolar hemorrhage, and/or biopsy-proven vasculitis), whereas those without ANCA more frequently have an eosinophilic “tissue” phenotype, with more frequent cardiomyopathy. The phenotype of EGPA is influenced by genetic predisposition [12], suggesting that different manifestations are not only explained by the disease stage. However, whether phenotype distinction should be based on ANCA status and/or on organ manifestations, is unknown. In a recent workshop [13], it was emphasized that future efforts should aim at determining whether EGPA represents a single or multiple nosologic entities, and at validating the diagnostic utility of ANCA in eosinophilic lung diseases.
Although EGPA clinical manifestations are highly heterogeneous, no study has yet described EGPA patients' manifestations according to the presence or not of genuine vasculitis features. Here, we aimed to describe organ manifestations and ANCA status in a large series of EGPA patients based on the presence or not of vasculitis features, with the additional aim of guiding future recommendations for nosology and classification.
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Study design and data collection
For this retrospective study, a detailed standardized form was sent to physicians who previously had prospectively reported cases of EGPA to our registry, and to members of the Task Force who had prospectively collected data in their own site database. The study was approved by the Institutional Review Board of the Société de Pneumologie de Langue Française. The database was anonymous and complied with the requirements of the Commission Nationale Informatique et Libertés. Patients received
Patient characteristics, diagnostic criteria
Out of 168 case report forms received, 11 were excluded because of absence of extra-respiratory organ manifestation in 10 cases, and because of absence of eosinophilia in one case. A total of 157 cases were included, with a mean age of 49.4 ± 14.1 (range, 17.2–80.9), and 51% of women.
All patients had a history of asthma, eosinophilia, and ≥ 1 system organ manifestation (other than asthma, pulmonary or ENT involvement) directly attributable to EGPA. The mean maximal peripheral blood eosinophil
Discussion
Because systemic manifestations in EGPA are heterogeneous, we studied organ manifestations and outcome of patients with EGPA based on presence or absence of definite vasculitis features and/or ANCA status. Using a stepwise approach of categorization using predefined criteria of vasculitis or surrogates of vasculitis, we found that not all patients with EGPA had definite vasculitis features.
Patients with ANCA were more likely to have definite features or surrogates of vasculitis. Interestingly,
Funding source
European Respiratory Society, grant number ERS TF 2008-08.
Conflict of interest
None for all authors.
Acknowledgements
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We are grateful to Mrs. Anne Beghoul, Raphaele Guelminger, Marion Durand, Chantal Silarakis, Sabrina Zeghmar who contributed to data acquisition and entry.
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Members of the European Respiratory Society Taskforce on eosinophilic granulomatosis with polyangiitis (Churg-Strauss) were:
CHAIRS | L. Guillevin (Paris, France), J.F. Cordier (Lyon, France), D. Jayne (Cambridge, UK) |
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SECRETARY | C. Pagnoux (Toronto, Canada) |
ASSOCIATED SECRETARIES | V. Cottin (Lyon, France) – Respiratory panel M. Groh (Paris, |
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Other Contributors (to be listed on PubMed): Contributed to the study : Guillaume Beltramo (Dijon, France), Pascal Beynel (Bourg en Bresse, France), Isabelle Court-Fortune (Saint-Etienne, France), Luca Di Toma, Legnano (Milan, Italy), Emmanuel Gomez (Nancy, France), Lize Kiakouama (Lyon, France), Christophe Leroyer (Brest, France), Marianne Levesque (Montréal, Canada), Anita Mollard (Strasbourg, France), Christophe Rogé (Morlaix, France). Other members of the core panel : Wolfgang Gross, P. A. Merkel, Ulrich Specks, Jean Bousquet, Eric Marchand, Ian Pavord, Johan-Christian Virchow.