Wegener's granulomatosis: clinical experience with eighteen patients.

ABSTRACT

Wegener's granulomatosis is being recognised with increasing frequency in India. Our 18, histologically confirmed, patients had a clinical profile similar to that described from developed countries. Delayed diagnosis led to the death of nine patients, usually within days of hospital admission, due to extensive vasculitis and renal failure. Tuberculosis was the most frequently considered diagnosis and 12 patients had been treated for it in spite of progressive clinical deterioration. Those who could be adequately treated with low dose daily cyclophosphamide and corticosteroids did well. Six of seven such patients are alive and well 1-8 years later. We believe that if prompt lung biopsy and ANCA determination are resorted to in patients with "resistant tuberculosis", it will greatly expedite case detection, diagnosis and optimum treatment of this remediable disease.