Cryptic Wegener's granulomatosis revealed after 18 years

A 48-yr-old female is described. She presented with subglottic stenosis requiring permanent tracheostomy, followed after 2 yr by an inflammatory polyarthritis, treated as rheumatoid. Eighteen years later, a vasculitic illness characterized by rash, weight loss, fever, and mononeuritis multiplex drew attention to the likelihood of a unifying diagnosis. A solitary lung opacity in the absence of nodules elsewhere and a positive ANCA (albeit of perinuclear specificity), made the diagnosis of Wegener's granulomatosis most likely. The combined exhibition of systemic corticosteroids and immunosuppressive drugs was only required 18 yr after onset, and illustrates the relatively benign course that this disorder can take.