Eosinophilic Granulomatosis with Polyangiitis: An Overview

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystemic disorder, belonging to the small vessel anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, defined as an eosinophil-rich and necrotizing granulomatous inflammation often involving the respiratory tract, and necrotizing vasculitis predominantly affecting small to medium-sized vessels, associated with asthma and eosinophilia. EGPA pathogenesis is not well known: HLA-DRB1*04 and *07, HLA-DRB4 and IL10.2 haplotype of the IL-10 promoter gene are the most studied genetic determinants. Among the acquired pathogenetic factors, the exposure to different allergens, infections, vaccinations, drugs, and silica exposure have been involved. Eosinophils are the most characteristic cells in EGPA and different studies have demonstrated their role as effector and immunoregulatory cells. EGPA is considered as a disease with a prevalent activation of the Th-2 cellular-mediated inflammatory response and also humoral immunity plays an important role. A link between B and T inflammatory responses may explain different disease features. EGPA typically develops into three sequential phases: the allergic phase, distinguished by the occurrence of asthma, allergic rhinitis, and sinusitis, the eosinophilic phase, in which the main pathological finding is the eosinophilic organ infiltrations (e.g., lungs, heart, and gastrointestinal system), and the vasculitic phase, characterized by purpura, peripheral neuropathy, and constitutional symptoms. ANCA (especially pANCA anti-myeloperoxidase) are present in 40–60% of the patients. An elevation of IgG4 is frequently found. Corticosteroids and cyclophosphamide are classically used for remission induction, while azathioprine and methotrexate are the therapeutic options for remission maintenance. B-cell depletion with rituximab has shown promising results for remission induction.

Familial Eosinophilic Granulomatosis with Polyangiitis

“…The only review article which presents the association of genetic determinants in EPGA with HLA is by Andrea Gioffredi (2014), where the author cited many references which prove the association of HLA-DRB1 * 04, *07 and HLA-DRB4 with EPGA [25]. …”

Severe alveolar bone loss and internal root resorption in linear IgA disease and eosinophilic granulomatosis with polyangiitis: A rare case report

“…This disease most commonly affects subjects between 40-60 year-old, with a mean age at diagnosis of 48 years. 19 Other manifestations of EGPA include major salivary gland involvement with the development of a focal mass or enlargement and neurolaryngeal involvement with dysphonia. [27][28][29][30] Treatment of patients with EGPA must be performed by experienced physicians who are specialists in rare autoimmune diseases.…”

“…18 Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multisystemic disorder that affects small-to-medium-sized vessels and is associated with severe asthma and eosinophilia. 19 The pathogenesis of EGPA is still unknown but eosinophils are the most characteristic cells in this disease. Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis of the small vessels, especially glomerulonephritis, is another feature of EGPA.…”

Recurrent myocardial infarction in a 50-year-old woman

“…These four factors meet the diagnostic criteria for eosinophilic granulomatosis with polyangiitis (EGPA)1 which was formerly known as Churg Strauss vasculitis.…”

“…Cardiac involvement is the major cause of mortality and can manifest as: coronary vasculitis, myocardial granuloma, eosinophilic endomyocarditis and pericarditis 1. ANCA was negative but this is frequently encountered in EGPA with cardiac involvement 2–4…”