Pulmonary angiitis and granulomatosis: radiologic-pathologic correlation.
Frazier AA, Rosado-de-Christenson ML, Galvin JR, Fleming MV
Five distinct clinical syndromes of pulmonary angiitis and granulomatosis
are currently recognized: Wegener granulomatosis, lymphomatoid granulomatosis,
necrotizing sarcoid granulomatosis, bronchocentric granulomatosis, and
allergic angiitis and granulomatosis (Churg-Strauss syndrome). Patients
typically present in middle age with fever, cough, hemoptysis, 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. Asthma may be present in bronchocentric
granulomatosis and Churg-Strauss syndrome. Pathologic examination of these
entities demonstrates vasculitis, granulomatous inflammation, and parenchymal
necrosis. The radiologic manifestations of pulmonary disease are varied,
but the most typical appearance is that of multiple nodules or masses that
may demonstrate cavitation. Diffuse multifocal air-space opacities with
or without 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 often considered
in the differential diagnosis. Affected patients are treated with cytotoxic
agents and corticosteroids. The prognosis is variable, depending on the
specific syndrome, but may be favorable in the absence of significant complications.
