Literature DB >> 25368130

Familial eosinophilic granulomatosis with polyangiitis in a mother and daughter.

Koray Harmanci1, Hulya Anil1, Abdulkadir Kocak1, Ener Cagri Dinleyici2.   

Abstract

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. 2014 BMJ Publishing Group Ltd.

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Year:  2014        PMID: 25368130      PMCID: PMC4225279          DOI: 10.1136/bcr-2014-206934

Source DB:  PubMed          Journal:  BMJ Case Rep        ISSN: 1757-790X


  12 in total

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Journal:  Chest       Date:  2007-02       Impact factor: 9.410

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Journal:  J Rheumatol       Date:  2003-03       Impact factor: 4.666

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7.  A nationwide survey on the epidemiology and clinical features of eosinophilic granulomatosis with polyangiitis (Churg-Strauss) in Japan.

Authors:  Ken-Ei Sada; Koichi Amano; Ritei Uehara; Masahiro Yamamura; Yoshihiro Arimura; Yoshikazu Nakamura; Hirofumi Makino
Journal:  Mod Rheumatol       Date:  2013-12-02       Impact factor: 3.023

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Journal:  Medicine (Baltimore)       Date:  1984-03       Impact factor: 1.889

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Journal:  Arthritis Rheum       Date:  2013-01

10.  An uncommon presentation of eosinophilic granulomatosis with polyangiitis: a case report.

Authors:  Giuseppe Taormina; Giuseppe Andolina; Maria Aurelia Banco; Edy Julia Costanza-Gaglio; Alice Bonura; Silvio Buscemi
Journal:  J Med Case Rep       Date:  2014-06-13
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