| Literature DB >> 28983650 |
A Jerrentrup1, T Müller2, J Mariss3, S Dylla4.
Abstract
This article presents the case of a patient with dyspnea, a history of poorly controlled asthma, sinonasal polyposis, blood eosinophilia and transient pulmonary infiltrates. The autoantibodies antinuclear antibodies, cytoplasmic anti-neutrophil cytoplasmic antibodies (c-ANCA) and perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) were negative. These symptoms are typical for eosinophilic granulomatosis with polyangiitis (EGPA, alternatively known as Churg-Strauss syndrome). The delay between onset and diagnosis in this case was several years. Although EGPA belongs to the spectrum of ANCA-associated vasculitis, less than 50% of EGPA patients are ANCA positive. Cardiac involvement (such as endomyocardial infiltration, arrhythmia and pericarditis) is the major cause of early death and a poor prognosis. In therapeutic regimens glucocorticoids and/or another immunosuppressant (e. g. cyclophosphamide, methotrexate or azathioprine) are used. The so-called five-factor score is a useful tool for assessment of prognosis.Entities:
Keywords: Asthma; Autoantibodies; Eosinophilia; Immunosuppression; Vasculitis
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Year: 2018 PMID: 28983650 DOI: 10.1007/s00108-017-0303-3
Source DB: PubMed Journal: Internist (Berl) ISSN: 0020-9554 Impact factor: 0.743