In considering the history of diseases, this syndrome has more recently been separated from the diseases of connective tissue for diagnostic and prognostic reasons.
This disease was first recognized by Klinger1 in 1931, but Wegener,2 in 1936, described and characterized the features which distinguish this condition. In 1954, Fahey and his associates3 more clearly established the clinical and pathological findings. Since then there have been increasing reported cases in Europe and the United States.
Wegener's granulomatosis has had a difficult path in this short period, because the identifying characteristics of this disease resemble many conditions and invade many systems. No doubt, this has retarded its establishment as a separate entity and continues to frustrate clinicians in their differential diagnosis.
The plethora of terms applied to what is now known as Wegener's granulomatosis gives an idea of the protean nature of this process: necrotizing granulomatosis and angitis, respiratory-renal