Interdisciplinary guidelines for the diagnosis and treatment of eosinophilic granulomatosis with polyangiitis

Czech version

Authors: L. Šenolt ¹; M. E. Bonatto ¹; L. Heribanová ²; M. Doubková ³; E. Lišková ⁴; I. Krčmová ⁵; T. Paleček ⁶; P. Kuchynka ⁶; M. Kubánek ⁷; J. Bouček ⁸; V. Koucky ⁸; M. Chovanec ⁹; M. Doubek ¹⁰; O. Kodet ¹¹; P. Nytrová ¹²; M. Elišák ¹³; J. Heissigerová ¹⁴; P. Svozílková ¹⁴; M. Brichová ¹⁴; K. Grusová ¹⁵; E. Müllerová ¹⁶; D. Kříž ¹⁶; Z. Hrušková ¹⁷; V. Tesař ¹⁷; P. Němec ¹⁸; T. Soukup ¹⁹; D. Suchý ²⁰; J. Hartinger ²¹; M. Kollár ²²; P. Horák ²³; R. Bečvář ¹
Authors‘ workplace: Revmatologická klinika 1. lékařské fakulty UK a Revmatologický ústav, Praha ¹; Pneumologická klinika 1. lékařské fakulty UK a Fakultní Thomayerova nemocnice, Praha ²; Klinika nemocí plicních a tuberkulózy Lékařské fakulty MU a Fakultní nemocnice, Brno ³; 1. klinika tuberkulózy a respiračních nemocí 1. lékařské fakulty UK a Všeobecné fakultní nemocnice v Praze ⁴; Ústav klinické imunologie a alergologie Lékařské fakulty UK a Fakultní nemocnice, Hradec Králové ⁵; II. interní klinika – klinika kardiologie a angiologie 1. lékařské fakulty a Všeobecná fakultní nemocnice v Praze ⁶; Klinika kardiologie, Institut klinické a experimentální medicíny, Praha ⁷; Klinika otorinolarynoglogie a chirurgie hlavy a krku 1. lékařské fakulty UK a Fakultní nemocnice v Motole, Praha ⁸; Otorinolaryngologická klinika 3. lékařské fakulty UK a Fakultní nemocnice Královské Vinohrady, Praha ⁹; Interní hematologická a onkologická klinika Lékařské fakulty MU a Fakultní nemocnice, Brno ¹⁰; Dermatovenerologická klinika 1. lékařské fakulty UK a Všeobecná fakultní nemocnice v Praze ¹¹; Neurologická klinika 1. lékařské fakulty UK a Všeobecná fakultní nemocnice v Praze ¹²; Neurologická klinika 2. lékařské fakulty UK a Fakultní nemocnice Motol, Praha ¹³; Oční klinika 1. lékařské fakulty UK a Všeobecná fakultní nemocnice v Praze ¹⁴; Zástupce pacientů, člen pracovní skupiny ČRS ¹⁵; Zástupce pacientské organizace Revma Liga, Česká republika, z. s. ¹⁶; Klinika nefrologie 1. lékařské fakulty UK a Všeobecná fakultní nemocnice v Praze ¹⁷; II. interní klinika Lékařské fakulty MU a Fakultní nemocnice U sv. Anny v Brně ¹⁸; II. interní gastroenterologická klinika Lékařské fakulty UK a Fakultní nemocnice, Hradec Králové ¹⁹; Oddělení klinické farmakologie, Fakultní nemocnice, Plzeň ²⁰; Farmakologický ústav 1. lékařské fakulty UK a Všeobecná fakultní nemocnice v Praze ²¹; Pracoviště klinické a transplantační patologie, Institut klinické a experimentální medicíny, Praha ²²; III. interní klinika – nefrologická, revmatologická a endokrinologická, Lékařská fakulta Univerzity Palackého a Fakultní nemocnice, Olomouc ²³
Published in: Čes. Revmatol., 33, 2025, No. 2, p. 57-79.
Category: Recomendation

Overview

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is the rarest ANCA-associated vasculitis, characterized by asthma, blood and tissue eosinophilia, and necrotizing vasculitis of small -⁠ to medium-sized vessels. The clinical course of EGPA is variable, ranging from a mild form dominated by eosinophilic manifestations to severe vasculitic involvement of vital organs. Diagnosis is complex and requires clinical experience and a multidisciplinary approach. The Czech Society of Rheumatology initiated the development of interdisciplinary guidelines for the diagnosis and treatment of EGPA, based on several international recommendations and the most recent evidence. The diagnosis of EGPA is established based on the presence of vasculitis and relies on classification criteria that include evaluation of clinical signs, laboratory markers, imaging methods, and histopathological findings. Disease activity monitoring is an essential part of management. Treatment of EGPA is guided by stratification of patients according to disease severity.
In severe cases with involvement of life-threatening organs, induction therapy with high-dose glucocorticoids
in combination with cyclophosphamide or rituximab is recommended. In milder forms, remission can be achieved with glucocorticoid monotherapy. Maintenance therapy includes conventional immunosuppressants (methotrexate, azathioprine, mycophenolate mofetil, or leflunomide), biologic agents (mepolizumab, benralizumab, or rituximab), and gradual tapering of glucocorticoids. Relapses are treated based on severity, with biologic therapy playing
a key role in prevention. The guidelines also outline an approach for the treatment of refractory disease. Emphasis is placed on the prevention of complications such as infections, osteoporosis, and cardiovascular and oncologic risks. These recommendations provide a comprehensive framework for optimizing the care of patients with EGPA
and highlight the importance of interdisciplinary collaboration to ensure an individualized approach for each
patient.

Keywords:

vasculitis – Eosinophilia – systemic inflammation – eosinophilic granulomatosis with polyangiitis

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2025 Issue 2