Familial vasculitides: Churg-Strauss syndrome and Wegener's granulomatosis in 2 first-degree relatives.
Churg-Strauss syndrome (CSS) and Wegener's granulomatosis (WG) are uncommon primary vasculitides, characterized by the involvement of the small to medium size vessels and by the frequent presence of serum antineutrophil cytoplasmic antibodies (ANCA). The pathogenesis of ANCA associated vasculitides is unclear, but roles for both genetic and environmental factors have been suggested. Familial cases of WG, but not CSS, have been reported. We describe the occurrence of CSS in a man and, 5 years later, WG in his son. These patients live together in an urban area of Northern Italy and share the HLA haplotype A*03; B*07; C*w07; DRB 1*0404, DQB 1*0302. To our knowledge, this is the first report of the familial clustering of CSS and WG in first-degree relatives.
Manganelli
Paolo
P
Diapartimento Osteo-Articolare, Unità Operativa di Reumatologia e Medicina Interna, Azienda Ospedaliera di Parma, Italy. pmanganelli@ao.pr.it
Giacosa
Roberto
R
Fietta
Pieranna
P
Zanetti
Adele
A
Neri
Tauro Maria
TM
D000328:Adult; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D015267:Churg-Strauss Syndrome / Q000235:genetics* / Q000276:immunology; D005190:Family; D014890:Granulomatosis with Polyangiitis / Q000235:genetics* / Q000276:immunology; D015234:HLA-A Antigens / Q000235:genetics; D015235:HLA-B Antigens / Q000235:genetics; D015236:HLA-C Antigens / Q000235:genetics; D006683:HLA-DQ Antigens / Q000235:genetics; D059866:HLA-DQ beta-Chains; D006684:HLA-DR Antigens / Q000235:genetics; D059811:HLA-DRB1 Chains; D006239:Haplotypes; D006801:Humans; D008297:Male; D008875:Middle Aged
D019268:Antibodies, Antineutrophil Cytoplasmic; D015234:HLA-A Antigens; D015235:HLA-B Antigens; D015236:HLA-C Antigens; D006683:HLA-DQ Antigens; D059866:HLA-DQ beta-Chains; C069051:HLA-DQB1 antigen; D006684:HLA-DR Antigens; D059811:HLA-DRB1 Chains