Wegener's Granulomatosis
Pathophysiology: A granulomatous, presumably an
immunologically mediated inflammatory process largely involving
small blood vessels. The inflammation leads to nodular areas in
the lungs which may cavitate. Local pulmonary hemorrhages
occur. Repair may be complete, or may be incomplete with
varying degrees of resultant lung fibrosis.
Classic Wegener’s: multisystem involving lungs, sinuses and
kidneys.
Limited Wegener’s: involves only the lungs, sparing the
kidneys.
This disease is one of the classic Pulmonary Renal Syndromes
(PRS).
Clinical Clues: Sinusitis, renal disease with hematuria and
hemoptysis.
Consider other Pulmonary Renal Syndromes: (e.g.
polyarteritis nodosa and other vasculitides, PRS following URI’s
(common, involving young women, likely viral etiology),
Goodpasture’s syndrome)
CXR/CT Findings:
-
Classic:
medium to large (centimetres) pulmonary nodules often with
cavitation **
-
Cavitary areas
often in upper 2/3 of lungs
-
May see air/blood
levels in cavities
-
Cavity wall thick
and fuzzy with surrounding airspace changes (lung bleeding)
-
Focal areas of
airspace disease: lung hemorrhage
-
No significant
adenopathy
-
No significant
pleural reaction
“Aunt Sophies”: gamut of cavitary lung nodule(s) ±
airspace disease
-
infections:
granulomatous (e.g. tuberculosis and fungal; staphylococcal
pneumonia with cavitation, septic emboli, lung abscesses)
-
tumor:
cavitating primary or metastatic cancer (e.g. squamous cell
cancer, any cancer)
-
other vasculitides:
e.g. polyarteritis nodosa
-
traumatic
pneumatocele
-
complicated bullae
or blebs: infection or bleeding
-
infected
bronchogenic cyst(s)
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