A vasculitis centre based management strategy leads to improved outcome in eosinophilic granulomatosis and polyangiitis (Churg–Strauss, EGPA): monocentric experiences in 150 patients

Moosig, Frank; Bremer, Jan Phillip; Hellmich, Bernhard; Holle, Julia; Holl‐Ulrich, Konstanze; Laudien, Martin; Matthis, Christine; Metzler, Claudia; Nölle, Bernhard; Richardt, Gert; Gross, Wolfgang L.

doi:10.1136/annrheumdis-2012-201531

Cited by 233 publications

(145 citation statements)

References 27 publications

Supporting

Mentioning

114

Contrasting

Unclassified

Order By: Relevance

“…Endomyocardial involvement and lung infiltrates are more common in the ANCAnegative subset [20]. ANCA, with an immunofluorescence pattern usually consisting of P-ANCA and anti-MPO specificity, is present in up to 40% of EGPA patients, but only a minority of patients have cytoplasmic ANCA with antibodies to proteinase 3 [21][22][23]. In our study, only one of the 15 patients (6.6%) was positive for ANCA, and this patient was in the vasculitic phase.…”

Section: Discussionmentioning

confidence: 45%

Clinical and Serological Features of Eosinophilic and Vasculitic Phases of Eosinophilic Granulomatosis with Poliangiitis: a Case Series of 15 Patients

Yılmaz¹,

Tutar²,

Şimşek³

et al. 2017

Turk Thorac J

View full text Add to dashboard Cite

OBJECTIVES:Eosinophilic granulomatosis with poliangiitis (EGPA) which was previously called Churg-Strauss Syndrome, is classified into eosinophilic and vasculitic phases. To characterize the eosinophilic and vasculitic phases of the disease in terms of clinical findings, serology, and treatment. MATERIALS AND METHODS:We included 15 EGPA patients in the study. The clinical, serological, and therapeutic characteristics and the treatment responses of the patients were recorded. RESULTS:Thirteen patients were classified as being in the eosinophilic phase and two were classified as being in the vasculitic phase of EGPA. Initial symptoms were worsening asthma in all patients (n=15; 100%). All patients had rhinosinusitis, and 66.6% had hypersensitivity to nonsteroidal anti-inflammatory drugs. The two patients in the vasculitic phase did not have nasal polyposis. Pulmonary and nervous system involvement were the most common symptoms. The erythrocyte sedimentation rates (ESRs) of the two patients in the vasculitic phase were 65 mm/h and 55 mm/h, while ESR was normal in eosinophilic-phase patients. Antineutrophil cytoplasmic antibodies (ANCA) was detected in one patient (6.6%) who was in the vasculitic phase (Case 15). The disease was under control with higher doses of methylprednisolone in the vasculitic phase (Case 14: 12 mg/day, Case 15: 10 mg/day) than in the eosinophilic phase. Relapse was detected in the two patients in the vasculitic phase. Oral corticosteroid was not discontinued in any case, and no mortality was reported. CONCLUSION:Patients with eosinophilic phase or vasculitic phase EGPA had similar clinical onset. However, higher ESR, ANCA positivity, and extrapulmonary organ involvement were only found in patients in the vasculitic phase. Corticosteroid responsiveness was very good in all patients in the eosinophilic phase, and the disease could be controlled with a very low maintenance dose of a corticosteroid.

show abstract

Section: Discussionmentioning

confidence: 45%

Clinical and Serological Features of Eosinophilic and Vasculitic Phases of Eosinophilic Granulomatosis with Poliangiitis: a Case Series of 15 Patients

Yılmaz¹,

Tutar²,

Şimşek³

et al. 2017

Turk Thorac J

View full text Add to dashboard Cite

show abstract

“…Mono-und multizentrische Kohorten belegen eine kontinuierliche Verbesserung der Prognose quo ad vitam [93,192,248]. In jüngerer Zeit konnte eine Lebenserwartung, die sich nicht signifikant von der der Allgemeinbevöl-kerung unterschied, bei einem Großteil der AAV-Patienten unter Behandlung durch spezialisierte Zentren erreicht und nachgewiesen werden [93,164,205]. Aus vergleichbaren Zeiträumen und unter ähnlichen (teils jedoch älteren) Therapiestandards gewonnene Daten zeigen ebenso wie historische Daten bei dezentraler Behandlung eine nach wie vor gesteigerte Mortalität gegenüber der Normalbevölkerung [1,53,55,146].…”

Section: üBergeordnete Empfehlungen üBergeordnete Empfehlungunclassified

S1 guidelines Diagnostics and treatment of ANCA-associated vasculitis

Schirmer

Moosig²

2017

Z Rheumatol

Self Cite

View full text Add to dashboard Cite

“…The majority of patients with EGPA respond to glucocorticoids ± immunosuppressive agents 3 , though a significant proportion of them develop relapses and may require biologic treatment 4 . Novel biomarkers of disease activity for EGPA are needed because the clinical value of eosinophil count, serum IgE, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP) in treated patients is low 5 .…”

Section: To the Editormentioning

confidence: 99%

Eotaxin-3 as a Biomarker of Activity in Established Eosinophilic Granulomatosis with Polyangiitis

2016

View full text Add to dashboard Cite

A vasculitis centre based management strategy leads to improved outcome in eosinophilic granulomatosis and polyangiitis (Churg–Strauss, EGPA): monocentric experiences in 150 patients

Abstract: Regular re-evaluation and target-orientated adaption of therapy may lead to normalization of life expectancy and attenuation of disease progression. Continued centre based interdisciplinary treatment should be standard of care.

Cited by 233 publications

References 27 publications

Clinical and Serological Features of Eosinophilic and Vasculitic Phases of Eosinophilic Granulomatosis with Poliangiitis: a Case Series of 15 Patients

Clinical and Serological Features of Eosinophilic and Vasculitic Phases of Eosinophilic Granulomatosis with Poliangiitis: a Case Series of 15 Patients

S1 guidelines Diagnostics and treatment of ANCA-associated vasculitis

Eotaxin-3 as a Biomarker of Activity in Established Eosinophilic Granulomatosis with Polyangiitis

Contact Info

Product

Resources

About