Childhood-onset Eosinophilic Granulomatosis with Polyangiitis (formerly Churg-Strauss Syndrome): A Contemporary Single-center Cohort

Gendelman, Samantha R.; Zeft, Andrew; Spalding, Steven J.

doi:10.3899/jrheum.120808

Cited by 65 publications

(57 citation statements)

References 15 publications

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“…This review of cEGPA cases also reveals the presence of cardiac involvement in 45% of patients (cardiomyopathy, 34%; pericarditis, 26%); gastrointestinal involvement in 47%, peripheral neuropathy (mononeuritis multiplex) in 40%, arthralgia in 27%, myalgia in 22% and kidney disease in 13% of patients, respectively 6 7…”

Section: Discussionmentioning

confidence: 80%

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Childhood-onset eosinophilic granulomatosis with polyangiitis: a rare childhood vasculitis mimicking anthrax and eosinophilic leukaemia

et al. 2016

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A 14-year-old boy previously misdiagnosed as having cutaneous anthrax was referred with a 2-month history of multiple wide and deep ulceronecrotic lesions in the lower extremities, which occurred after contact with animals. Skin biopsy was compatible with vasculitis. Further examination at our hospital elicited eosinophilia and a history of asthma. On the second day of hospitalisation, he developed deep vein thrombosis. A diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA) was established and intravenous methylprednisolone was administered. The patient showed remarkable improvement of the cutaneous lesions. Diagnosis of EGPA is challenging in the vasculitic phase and necessitates a detailed history that specifically questions the patient for an asthma background. This case illustrates a severe cutaneous presentation of EGPA and emphasises the difficulty of diagnosis as a result of overlapped signs and symptoms with cutaneous anthrax and leukaemia. EGPA should be kept in mind in the differential diagnosis of cutaneous lesions associated with eosinophilia.

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Section: Discussionmentioning

confidence: 80%

“…In the literature, anti-myeloperoxidase-ANCA is not present in 75% of cEGPA cases 6 7. Cardiac involvement is more commonly seen in ANCA-negative cases while renal disease, pulmonary haemorrhage, ear-nose-throat and peripheral nerve involvement, and biopsy-proven vasculitis, are more frequent in ANCA-positive cases 2 8–10.…”

Section: Discussionmentioning

confidence: 99%

Childhood-onset eosinophilic granulomatosis with polyangiitis: a rare childhood vasculitis mimicking anthrax and eosinophilic leukaemia

et al. 2016

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“…Die eosinophile Granulomatose mit Polyangiitis tritt im Kindesalter selten auf [8,31] bei Erwachsenen und teilweise auch pä-diatrischen Patienten existieren zu Therapieplänen mit i.v.-applizierten Immunglobulinen, Plasmapherese, Interferon-α, Mepolizumab, Rituximab, Imatinib und Omalizumab. Eine gute Übersicht findet sich in der aktuellen Literatur [9].…”

Section: Eosinophile Granulomatose Mit Polyangiitis (Churg-strauss-syunclassified

Pulmonale Manifestationen von Autoimmunerkrankungen und neue Therapieoptionen

Nagel

Burmester

Feilcke

et al. 2015

Monatsschr Kinderheilkd

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“…Further studies are needed to determine the prognostic role of ANCA and whether treatment should be tailored to the presence or absence of ANCA (Table 1). A retrospective review of pediatric patients evaluated from 2003 to 2011, with a mean diagnosis age of 15 years, showed eosinophilia, involvement of upper and lower airways and, ANCA negativity in all patients [60].…”

Section: Anca Phenotypesmentioning

confidence: 99%

Churg-Strauss Syndrome or Eosinophilic Granulomatosis with Polyangiitis

et al. 2015

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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.

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Childhood-onset Eosinophilic Granulomatosis with Polyangiitis (formerly Churg-Strauss Syndrome): A Contemporary Single-center Cohort

Abstract: Eosinophilia, in combination with upper airway, pulmonary, musculoskeletal, neurologic, and cardiac manifestations, is frequently observed in cEGPA. ANCA titers are often negative. Steroids are the mainstay of initial therapy but steroid-related side effects occur regularly.

Cited by 65 publications

References 15 publications

Childhood-onset eosinophilic granulomatosis with polyangiitis: a rare childhood vasculitis mimicking anthrax and eosinophilic leukaemia

Childhood-onset eosinophilic granulomatosis with polyangiitis: a rare childhood vasculitis mimicking anthrax and eosinophilic leukaemia

Pulmonale Manifestationen von Autoimmunerkrankungen und neue Therapieoptionen

Churg-Strauss Syndrome or Eosinophilic Granulomatosis with Polyangiitis

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