Five distinct clinical. syndromes of pulmonary angiitis and granulomat
osis are currently recognized: Wegener granulomatosis, lymphomatoid gr
anulomatosis, necrotizing sarcoid granulomatosis, bronchocentric granu
lomatosis, and allergic angiitis and granulomatosis (Churg-Strauss syn
drome). Patients typically present in middle age with fever, cough, he
moptysis, dyspnea, or chest discomfort. Upper airway involvement such
as sinusitis suggests Wegener granulomatosis. Medical renal disease is
associated with Wegener granulomatosis and Churg-Strauss syndrome, As
thma may be present in bronchocentric granulomatosis and Churg-Strauss
syndrome. Pathologic examination of these entities demonstrates vascu
litis, granulomatous inflammation, and parenchymal necrosis, The radio
logic manifestations of pulmonary disease are varied, but the most typ
ical appearance is that of multiple nodules or masses that may I demon
strate cavitation, Diffuse multifocal air-space opacities with or with
out cavitation may also be seen. Pulmonary hemorrhage is a well-known
presenting manifestation of Wegener granulomatosis and, less commonly,
of Churg-Strauss syndrome. Because of the multifocal lung involvement
in these diseases, pulmonary metastases and infectious causes are oft
en considered in the differential diagnosis. Affected patients are tre
ated with cytotoxic agents and corticosteroids. The prognosis is varia
ble, depending on the specific syndrome, but may be favorable in the a
bsence of significant complications.