Eosinophilic granulomatosis with polyangitis: A new target for biologicals

Objective & Hypothesis:Eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome) is a rare systemic necrotizing granulomatous vasculitis in the spectrum of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).

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Key Findings & Data:Conventional treatment of EGPA relies mainly on agents that decrease inflammation.

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Conclusion & Impact:This review provides an overview of eosinophilic granulomatosis with polyangiitis in the light of new targeted biological therapies.

🔬Design:Review & Systematic Survey

📍Scope:Comprehensive Sample

⚙️Variables:Domain Parameters

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome) is a rare systemic necrotizing granulomatous vasculitis in the spectrum of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Nevertheless, EGPA has specific clinical, biological and histological properties different from other AAVs [microscopic polyangiitis (MPA) and granulomatous polyangiitis (GPA)]. Recently, thanks to the studies conducted to understand the pathophysiology of EGPA, unlike neutrophils in other AAVs, the main cells involved in EGPA have been observed to be eosinophils. The key role of eosinophils in EGPA and recent development of targeted agents to treat other eosinophil-related diseases have created new therapeutic opportunities for EGPA. Conventional treatment of EGPA relies mainly on agents that decrease inflammation. Cornerstone therapy is systemic glucocorticoids, used as monotherapy or in combination with immunosuppressive agents. However, new therapeutic approaches are needed especially for persistent asthma symptoms, refractory disease, relapses and problems associated with corticosteroid dependence. Recently, the first large-scale randomized controlled clinical trial on polyangiitis and eosinophilic granulomatosis has demonstrated the efficacy of eosinophil-targeted biotherapy anti-interleukin-5 (IL-5) mepolizumab, and is approved for the management of EGPA. This finding opens a new era for EGPA management. This review provides an overview of eosinophilic granulomatosis with polyangiitis in the light of new targeted biological therapies.

Özet

Eozinofilik granülomatöz polianjitis (EGPA, Churg-Strauss sendromu), antinötrofil sitoplazmik antikor (ANCA) ile ilişkili vaskülit (AAV) spektrumunda nadir görülen sistemik nekrotizan granülomatöz vaskülittir. Bununla birlikte, EGPA’nın diğer AAV’lerden [mikroskopik polianjiitis (MPA) ve granülomatöz polianjit (GPA)] farklı olarak spesifik klinik, biyolojik ve histolojik özellikleri vardır. Son zamanlarda; EGPA’nın patofizyolojisini anlamak için yapılan çalışmalar sayesinde EGPA’da görev alan ana hücrelerin diğer AAV’lerdeki görev alan nötrofillerden farklı olarak eozinofiller olduğu gözlemlendi. Eozinofillerin EGPA’daki kilit rolü ve diğer eozinofil ile ilişkili hastalıkları tedavi etmek için hedeflenen ajanların son zamanlardaki gelişimi, EGPA için yeni terapötik fırsatlar yaratmıştır. EGPA’nın geleneksel tedavisi, esas olarak antiinflamatuvar ajanlardır. Başlıca ana tedavisi, monoterapi olarak veya immünosupresif ajanlarla kombinasyon halinde kullanılan sistemik glukokortikoidlerdir. Ancak özellikle persistan astım semptomları, refrakter hastalık, relapslar ve kortikosteroid bağımlılığı ile ilişkili problemler için yeni tedavi yaklaşımlarına ihtiyaç duyulmaktadır. Yakın zamanlı; eozinofilik granülomatöz polianjitis üzerine ilk büyük ölçekli randomize kontrollü klinik çalışma, eozinofil hedefli biyoterapi anti-interlökin- 5 (IL-5) mepolizumabın etkinliğini göstermiştir ve EGPA tedavisi için onaylanmıştır. Bu çalışma sonuçları EGPA tedavisi için yeni bir çağ açmaktadır. Bu derleme, yeni hedefe yönelik biyolojik tedaviler ışığında eozinofilik granülomatozis polianjitise genel bir bakış sunmaktadır.

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Cite This Article

BAVBEK, S., SÖZENER, Z. Ç., DURMAZ, M. S. B. (2022). Eosinophilic granulomatosis with polyangitis: A new target for biologicals. Tüberküloz ve Toraks, Cilt: 70 - Sayı: 1, 93–101. https://ajindex.com/articles/618262

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