[Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)]

Internist (Berl). 2018 May;59(5):497-504. doi: 10.1007/s00108-017-0303-3.
[Article in German]

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.

Keywords: Asthma; Autoantibodies; Eosinophilia; Immunosuppression; Vasculitis.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Antibodies, Antineutrophil Cytoplasmic / blood
  • Churg-Strauss Syndrome* / diagnosis
  • Churg-Strauss Syndrome* / drug therapy
  • Cyclophosphamide / therapeutic use
  • Granulomatosis with Polyangiitis*
  • Humans
  • Immunosuppressive Agents / therapeutic use

Substances

  • Antibodies, Antineutrophil Cytoplasmic
  • Immunosuppressive Agents
  • Cyclophosphamide