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By Fatih Kelesoglu ...
Semanur Özdel, Z Birsin Özçakar, Seda Şahin Kunt, Atilla H Elhan, Fatoş Yalçınkaya
Familial Mediterranean fever (FMF) is an autosomal recessive disease, characterised by recurrent, self-limited attacks of fever with serositis. Recently, it was shown that patients with early disease onset during childhood period had more severe disease. The aim of this study was to describe the demographic, clinical and genetic features of FMF patients who had late-onset disease during childhood period and to compare them to those with earlier onset patients. Files of patients who had been seen in our department between January 2013 and January 2014 were retrospectively evaluated...
July 2016: Clinical Rheumatology
Giannicola Iannella, Antonio Greco, Guido Granata, Alessandra Manno, Benedetta Pasquariello, Diletta Angeletti, Dario Didona, Giuseppe Magliulo
Granulomatosis with polyangiitis (GPA) is an autoimmune systemic necrotizing small-vessel vasculitis associated with the presence of anti-neutrophil cytoplasmic antibodies (ANCA). Oto-neurological manifestations of ANCA-associated vasculitis according to PR3-ANCA positivity and MPO-ANCA positivity are usually reported. Facial nerve palsy is usually reported during the clinical course of the disease but it might appear as the presenting sign of GPA. Necrotizing vasculitis of the facial nerve 'vasa nervorum' is nowadays the most widely accepted etiopathogenetic theory to explain facial damage in GPA patients...
July 2016: Autoimmunity Reviews
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