Familial eosinophilic granulomatosis with polyangiitis in a mother and daughter

Harmancı K., Anil H., Kocak A., Dinleyici E. Ç.

BMJ Case Reports, vol.2014, 2014 (Scopus) identifier identifier

  • Publication Type: Article / Article
  • Volume: 2014
  • Publication Date: 2014
  • Doi Number: 10.1136/bcr-2014-206934
  • Journal Name: BMJ Case Reports
  • Journal Indexes: Scopus
  • Eskisehir Osmangazi University Affiliated: Yes


Copyright 2014 BMJ Publishing Group. All rights reserved.A 17-year-old girl was admitted to our unit with weight loss, dyspnoea, arthralgia and sinusitis. Her medical history was noteworthy for bronchial asthma and she required systemic steroid therapy. Her mother had a history of eosinophilic granulomatosis with polyangiitis (EGPA). Laboratory tests revealed excessive eosinophilia and elevated erythrocyte sedimentation. The assay for peripheral antineutrophil cytoplasmic antibodies was negative. Histopathological examination of lung biopsy revealed EGPA. The patient was treated with methylpredinosolone; her eosinophil count normalised and she began to improve clinically and radiographically. There is no genetic factor to influence susceptibility to this disease. To the best of our knowledge, this is the second report of familial EGPA disease in the literature, with a mother and daughter both being affected. EGPA disease should be kept in mind in a patient with uncontrolled asthma and eosinophilia with a positive family history for EGPA.