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Case Reports
. 2020 Apr 1;59(7):991-995.
doi: 10.2169/internalmedicine.3744-19. Epub 2019 Dec 6.

Familial Eosinophilic Granulomatosis with Polyangiitis in a Sister and Brother

Yuriko Ueki  1   2 , Chiyako Oshikata  1   2   3 , Yoshihito Asai  1 , Takeshi Kaneko  3 , Naomi Tsurikisawa  1   2   3
Affiliations
Case Reports

Familial Eosinophilic Granulomatosis with Polyangiitis in a Sister and Brother

Yuriko Ueki et al. Intern Med. .

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a vasculitis characterized by an abnormally high number of eosinophils in the peripheral blood and tissues. EGPA is an extremely rare disorder, with an incidence of 0.5 to 3.7 new cases per million people per year and an overall prevalence of 2.4 to 14 per million adults. There is little knowledge about the genetic factors that influence this disease. There are only two reports of familial EGPA: one in Japan and one in Turkey. We herein report a third case of familial EGPA in a brother and sister who were negative for myeloperoxidase-antineutrophil cytoplasmic antibodies.

Keywords: ANCA-associated vasculitis; IVIG; asthma; eosinophilic granulomatosis with polyangiitis; familial onset; mepolizumab.

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Conflict of interest statement

The authors state that they have no Conflict of Interest (COI).

Figures

Figure 1.
Figure 1.
Clinical course of treatment and relapse in Case 1: brother. IVIG: intravenous immunoglobulin, mPSL: methyl prednisolone, MTX: methotrexate, PSL: prednisolone
Figure 2.
Figure 2.
Clinical course of treatment and relapse in Case 2: sister. AZP: azathioprine, CyA: cyclosporine A, IVCY: intravenous cyclophosphamide, IVIG: intravenous immunoglobulin, MTX: methotrexate, PSL: prednisolone
See this image and copyright information in PMC

References

    1. Jennette JC, Falk RJ, Bacon PA, et al. . 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides. Arthritis Rheum 65: 1-11, 2013. - PubMed
    1. Gibelin A, Maldini C, Mahr A. Epidemiology and etiology of Wegener granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome and Goodpasture syndrome: vasculitides with frequent lung involvement. Semin Respir Crit Care Med 32: 264-273, 2011. - PubMed
    1. Watts RA, Carruthers DM, Scott DG. Epidemiology of systemic vasculitis: changing incidence or definition? Semin Arthritis Rheum 25: 28-34, 1995. - PubMed
    1. Tsurikisawa N, Morita S, Tsuburai T, et al. . Familial Churg-Strauss syndrome in two sisters. Chest 131: 592-594, 2007. - PubMed
    1. Harmanci K, Anil H, Kocak A, Dinleyici EC. Familial eosinophilic granulomatosis with polyangiitis in a mother and daughter. BMJ Case Rep: 2014. - PMC - PubMed

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