Wegener's Granulomatosis in a Family

Abstract

Wegener's granulomatosis (WG) is a rare disease. The true prevalence in Denmark is not known, nor do any review articles apparently give any figures. The diagnosis is based upon clinical and pathological characteristics, with necrotizing vasculitis affecting upper and/or lower respiratory tracts, smaller arteries and veins, and glomerulonephritis (1). A description of a patient population is given by Fauci (2).

The aetiology of WG is still unknown, although the demonstration of autoantibodies against cytoplasmic constituents of neutrophils and monocytes (ANCA) in this disease substantiates an autoimmune pathogenesis (3, 4, 5).

We describe here a family of 8 siblings, of whom 2 have been diagnosed independently as having WG based on clinical, serological and pathological findings. Because of this unusual event we decided to investigate the 6 other siblings in the family.