Wegener's granulomatosis presenting with sclerokeratitis diagnosed by antineutrophil cytoplasmic autoantibodies (ANCA).

A 23-year-old male presented with bilateral sclerokeratitis. He reported recent bronchitis, sinusitis, dyspnea on exertion, hemoptysis, arthralgias and myalgias. Wegener's granulomatosis was diagnosed by a positive antineutrophil cytoplasmic autoantibody (ANCA) test and a nasal and subglotic biopsy showing granulomatous inflammation. Treatment with cyclophosphamide, systemic corticosteroids and trimethoprim/sulfamethoxazole resulted in resolution of the sclerokeratitis and remission of the disease.