Ear, nose and throat manifestations of Churg-Strauss syndrome

Bacciu, Andrea; Bacciu, S; Mercante, Giuseppe; Ingegnoli, Francesca; Grasselli, Chiara; Vaglio, Augusto; Pasanisi, Enrico; Vincenti, Vincenzo; Garini, G; Ronda, Nicoletta; Ferri, Teore; Corradi, Domenico; Buzio, Carlo

doi:10.1080/00016480500437435

Cited by 121 publications

(125 citation statements)

References 21 publications

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“…However, the two patients in the vasculitic phase did not have nasal polyposis. These rates were similar to those of other studies [10][11][12]. The rate of NSAID hypersensitivity was similar (66%) to our previous study [12].…”

Section: Discussionsupporting

confidence: 92%

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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

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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.

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Section: Discussionsupporting

confidence: 92%

“…Nasal polyposis and recurrent sinusitis characterize the prodromic EGPA phase [10]. Nasal polyps affect approximately half of the patients and can recur after surgery in patients not receiving immunosuppressive therapy [11].…”

Section: Discussionmentioning

confidence: 99%

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

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“…The patients had been diagnosed at internal medicine departments (nephrology, clinical immunology, rheumatology, pulmonary medicine, and others) of general hospitals in Northern Italy. CSS was diagnosed based on the presence of asthma, hypereosinophilia (Ͼ10%, or Ͼ1,500 cells/mm 3 ), and clinical manifestations consistent with systemic vasculitis, with or without histologic confirmation (4). The absence of hypereosinophilia was not considered an exclusion criterion in patients receiving steroids for asthma if histologic evidence of vasculitis or extravascular eosinophils was available (8).…”

Section: Methodsmentioning

confidence: 99%

“…It usually occurs in patients with asthma and eosinophilia, and has a heterogeneous clinical spectrum that includes constitutional symptoms, sinusitis, pulmonary infiltration, peripheral neuropathy, and skin (e.g., purpura, nodules), renal (e.g., isolated urinary abnormalities, rapidly progressive glomerulonephritis [RPGN]), and gastrointestinal manifestations (3)(4)(5)(6). Antineutrophil cytoplasmic antibodies (ANCAs) are present in ϳ40% of patients, usually in those developing clinical features resulting from active small-vessel vasculitis (e.g., RPGN, purpura) (7,8).…”

mentioning

confidence: 99%

HLA–DRB4 as a genetic risk factor for Churg‐Strauss syndrome

Vaglio

Martorana

Maggiore

et al. 2007

Arthritis & Rheumatism

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171

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Objective. To explore the association between HLA alleles and Churg-Strauss syndrome (CSS), and to investigate the potential influence of HLA alleles on the clinical spectrum of the disease.Methods. Low-resolution genotyping of HLA-A, HLA-B, and HLA-DR loci and genotyping of TNFA ؊238A/G and TNFA ؊308A/G single-nucleotide polymorphisms were performed in 48 consecutive CSS patients and 350 healthy controls.Results.

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“…Although uncommon, the disease may present with sinonasal involvement. 3,29 Sinonasal tract involvement consists of sinonasal polyps and chronic sinusitis with eosinophils. Necrotizing vasculitis is only rarely seen in sinonasal specimens.…”

Section: Autoimmune Disease/vasculitis: Antineutrophilic Cytoplasmic mentioning

confidence: 99%

Differential Diagnosis of Necrotizing Sinonasal Lesions

Montone

2015

Archives of Pathology &Amp; Laboratory Medicine

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Context A number of entities may result in necrosis in the sinonasal tract and lead to significant morbidity and mortality. These include infections, necrotizing vasculitis, neoplastic processes, and drug dependency. This review will concentrate on the differential diagnosis of sinonasal necrotizing lesions. Objective To review the differential diagnoses of necrotizing destructive lesions of the sinonasal tract. Data Sources The current literature was reviewed to provide updated information regarding the differential diagnosis of sinonasal necrotizing lesions, including infectious disease processes; antineutrophilic cytoplasmic antibody–associated vasculitides; neoplastic processes, particularly natural killer/T-cell lymphomas; and drug abuse. Conclusions The differential diagnosis of necrotizing sinonasal lesions is broad, with often overlapping diagnostic features that lead to diagnostic challenges. Ancillary tests such as special stains and immunohistochemical studies can offer significant assistance.

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Ear, nose and throat manifestations of Churg-Strauss syndrome

Cited by 121 publications

References 21 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

HLA–DRB4 as a genetic risk factor for Churg‐Strauss syndrome

Differential Diagnosis of Necrotizing Sinonasal Lesions

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