ANCA-associated vasculitis in Greek siblings with chronic exposure to silica

We present the case of two siblings with similar environmental exposure to silica. Both of them developed perinuclear antineutrophil cytoplasmic antibody (p-ANCA)-associated vasculitis with pulmonary-renal syndrome. p-ANCAs were present with antimyeloperoxidase specificity on capture enzyme-linked immunosorbent assay. Treatment with corticosteroids and cyclophosphamide resulted in resolution of the clinical picture. Chronic exposure to silica is the leading environmental factor associated with ANCA-positive vasculitis. Several clusters of systemic vasculitis have been described. Positive and negative human leukocyte antigens (HLA) have been reported in systemic vasculitis. Affected brothers in our case shared one parental HLA haplotype. To the best of our knowledge, this is the first report of a family cluster of silica-induced, ANCA-associated systemic vasculitis with members sharing some of their HLA antigens.