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

Abstract

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.

Cardiac Involvement in Eosinophilic Granulomatosis with Polyangiitis: A Meta-analysis of 62 Case Reports

“…However, ANCA positivity occurs in only 40.0% of cases. 65 Cardiac involvement can be detected in both ANCA-positive and ANCA-negative patients, although patients with more clinically overt heart involvement are mainly ANCA negative. 66 , 67 …”

“…The pathogenesis of the disease is not clearly defined; however, it is often assumed to be an autoimmune disease due to the presence of ANCA. 65 Among the ANCA-associated vasculitis, EGPA is more commonly associated with cardiac involvement. 66 Cardiac involvement in patients with EGPA is a result of the direct activity of the disease affecting any part of the heart.…”

Eosinophilic granulomatosis with polyangiitis in a Nigerian woman

“…The first prodromal phase characterised by allergic rhinitis, sinusitis, nasal polyposis, asthma and may last up to 30 years. Second stage is characterised by peripheral blood and tissue eosinophilia, third stage is characterised by constitutional symptoms and vasculitis involving small to medium-sized vessels 6 7. Although these phases do not necessarily follow that order, our patient had allergic rhinitis and chronic sinusitis for more than 20 years before the onset of asthma, constitutional symptoms and peripheral neuropathy.…”

Eosinophilic granulomatosis with polyangiitis: diagnosis and treatment-related issues

“…В другом исследовании продемонстрирована аналогичная картина поражения органов-мишеней наряду с альвеолярным кровоизлиянием в подгруппе АНЦА-позитивных пациентов. В обоих исследованиях васкулит реже выявлялся у серонегативных пациентов, хотя обнаружена более высокая распространенность кардиомиопатии и таких системных проявлений, как недомогание и лихорадка [15,16].…”