Author(s)

Abdurhman S. Al Arfaj^1*, Mohammad Al Anazi², Najma Khalil¹, Akbar Ali Khan Pathan^2,3, Narsimha Reddy Parine²

¹Department of Medicine, Rheumatology Division, College of Medicine, King Saud University, Riyadh, KSA.
²Department of Biochemistry, College of Science, King Saud University, Riyadh, KSA.
³Integrated Gulf Biosystems, Riyadh, KSA.

Eosinophilic granulomatosis with polyangiitis (EGPA) is an uncommon ANCA associated vasculitic disorder characterized by systemic necrotizing vasculitis of small vessels occurring exclusively among patients with bronchial asthma and tissue eosinophilia. Familial EGPA is extremely rare. Only two case reports have been published so far. We present a Saudi family with 3 cases of EGPA and almost three-fourths of family members affected by asthma. We explored genetic basis of EGPA in this family and found that genes were mutated in four affected siblings suggesting genetic involvement in susceptibility to EGPA.

Familial Eosinophilic Granulomatosis with Polyangiitis, Churg-Strauss Syndrome, ANCA Associated Vasculitis

Al Arfaj, A. , Al Anazi, M. , Khalil, N. , Pathan, A. and Parine, N. (2017) Familial Eosinophilic Granulomatosis with Polyangiitis. Open Journal of Rheumatology and Autoimmune Diseases, 7, 137-146. doi: 10.4236/ojra.2017.73013.