Background: Wegener's granulomatosis is the prototype of pulmonary angiitis and granulomatosis and a systemic vasculitic syndrome of unknown etiology. Wegener's granulomatosis can involve virtually any and often a multitude of organ-tissues.
Patients and methods: This survey of 216 patients provides a histological documentation and pertinent literature review of both the common and uncommon, but with emphasis on the uncommon, manifestations of Wegener's granulomatosis.
Results: The common manifestations of the disease include the classic triad of upper airway, lung, and kidney, in 87%, 69%, and 48% of the patients, respectively. The less common manifestations involve the skin, central nervous system, eye and orbit, heart, breast, salivary gland, gastrointestinal tract, spleen, and male and female urogenital tracts; each of these accounts for less than 15% in all cases and below 5% for most of the patients.
Conclusions: The manifestations of Wegener's granulomatosis in many of the uncommon anatomical sites of involvement may be distinctive or atypical and therefore, the histopathological diagnosis must be correlated with clinical and laboratory test findings.