Churg-Strauss Syndrome or Eosinophilic Granulomatosis with Polyangiitis

Abstract

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss, EGPA) is a systemic small-to-medium-sized vasculitis associated with asthma and eosinophilia. Histologically EGPA presents tissue eosinophilia, necrotizing vasculitis, and granulomatous inflammation with eosinophil tissue infiltration. EGPA commonly involves the upper airway and lung parenchyma, peripheral neuropathy, cardiac disorders, and skin lesions. The anti-neutrophil cytoplasmic antibodies (ANCA) are positive in 40% of cases, especially in those patients with clinical signs of vasculitis. The pathogenesis of EGPA is multifactorial. The disease can be triggered by exposure to a variety of allergens and drugs, but a genetic background has also been described, particularly an association with HLA-DRB4. Th2 response is of special importance in the upregulation of different interleukins such as IL-4, IL-13, and IL-5. Th1 and Th17 responses are also of significance. Activated eosinophils have a prolonged survival and probably cause tissue damage by releasing eosinophil granule proteins, while their tissue recruitment can be regulated by chemokines such as eotaxin-3 and CCL17. Humoral immunity is also OPEN ACCESSSinusitis 2016, 1 25 abnormally regulated, as demonstrated by excessive responses of IgG4 and IgE. EGPA has a good respond to glucocorticoids, although the combination of glucocorticoids and immunosuppressants (e.g., cyclophosphamide, azathioprine) is needed in most of cases. Newer treatment options include anti-IL-5 antibodies (mepolizumab), whose efficacy has been described in clinical trials, and anti-CD-20, a B cell-depleting agent (rituximab), reported in several case series.

Eosinophilic Granulomatosis with Polyangiitis in an 8-year-old Girl Manifesting as Hypereosinophilic Syndrome with Myocarditis, Stroke, and Subsequent Orbital Involvement

“…Histologically, EGPA presents tissue eosinophilia, necrotizing vasculitis, and granulomatous inflammation with eosinophil tissue infiltration and commonly involves the upper airway and lung parenchyma, peripheral neuropathy, cardiac disorders, and skin lesions. Anti-neutrophil cytoplasmic antibodies (ANCA) are positive in only up to 40% of paediatric cases [5,7,10,11]. The pattern of organ involvement and clinical outcomes of patients with EGPA can differ depending on their ANCA status, which may reflect the different pathogenic mechanisms underlying ANCApositive vs ANCA-negative EGPA [12].…”

“…Histologically, EGPA presents tissue eosinophilia, necrotizing vasculitis, and granulomatous inflammation with eosinophil tissue infiltration and commonly involves the upper airway and lung parenchyma, peripheral neuropathy, cardiac disorders, and skin lesions. Anti-neutrophil cytoplasmic antibodies (ANCA) are positive in 40% of cases [ 5]. The 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis are a maximum eosinophil count ≥1×10 9 /L (+5), obstructive airway disease (+3), nasal polyps (+3), a 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) [ 6].…”

The Utility of Nasal Challenges to Phenotype Asthma Patients

“…The main indication for the nasal challenge with lysine-acetylsalicylate (NC-L-ASA) is the investigation of AERD in an asthma/CRSwNP patient who experienced airway symptoms (nasal and/or bronchial) after the intake of NSAIDs (especially strong COX-1 blockers) [52]. If the patient had ≥2 reactions with NSAIDs belonging to different pharmacological groups (e.g., aril-propionic, and aril-acetic acids), the diagnosis of NSAID intolerance is considered proven, with no need for additional tests [53]. However, in patients with a single airway reaction, further investigation is required [52].…”

“…However, in patients with a single airway reaction, further investigation is required [52]. The NC-L-ASA is especially indicated in those individuals where an oral or bronchial challenge cannot be performed (e.g., those with uncontrolled asthma or FEV1 < 70%) [53]. Nevertheless, it might represent the safest and most rapid alternative for AERD screening in the heterogeneous population of asthmatics seen in the clinic [40].…”

Eosinophilic granulomatosis with polyangiitis: case report of three patients

“…Laboratóriumi vizsgálat során eosinophilia (>10%), ANCA pozitivitás (átlagosan az esetek 40%-ban, perinucleáris festő-dés), emelkedett CRP, IgE és IgG4 szint mutatható ki. A legfrissebb tanulmányok alapján új biomarkereket is azonosí-tottak, melyek korrelálnak a betegség súlyosságával, mint az eotaxin, illetve eosinophil kationos fehérje, de klinikai felhasználásukhoz további vizsgálatokra van szükség (15). Differenciáldiagnosztikai szempontból ajánlott lépések az egyéb ANCA asszociált vasculitisek, egyéb reaktív eosinophiliák (Toxocara, Strongyloides stercoralis, HIV, Aspergillus fertőzés), paraneoplasztikus hypereosinophila kizárása (16).…”