Cogan's syndrome with antineutrophil cytoplasmic autoantibody

Cogan's syndrome is a rare disease characterised by non-syphilitic interstitial keratitis with vestibuloauditory dysfunction, including loss of hearing, tinnitus, and vertigo.1 We report here a case of Cogan's syndrome positive for antineutrophil cytoplasmic antibody (ANCA). This case is interesting in consideration of the pathogenesis of this syndrome. In May 1999 a 61 year old man was admitted to our hospital with fever and myalgia localised to the lower part of both legs. He had a history of lung tuberculosis at 22 years old, chronic sinusitis at 30, and bilateral otitis media at 53. On admission, laboratory tests showed a white blood cell count of 11.2×109/l and a CRP level of 63 mg/l. Serum levels of creatine kinase, alanine aminotransferase, and aspartate aminotransferase, and a urinary examination were normal. A serological test was positive for perinuclear ANCA (pANCA) and myeloperoxidase ANCA (MPO-ANCA; 105 EU/ml (normal <10 EU/ml)), but negative …