• A Jerrentrup, T Müller, J Mariss, and S Dylla.
• Zentrum für Unerkannte und Seltene Erkrankungen, Universitätsklinikum Gießen und Marburg, Standort Marburg, Baldingerstr., 35043, Marburg, Deutschland. jerrentr@med.uni-marburg.de.
• Internist (Berl). 2018 May 1; 59 (5): 497-504.

AbstractThis 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.

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