Melanie B. Peachell, MD; Nestor L. Muller, MD, PhD

Disclosures

Semin Respir Crit Care Med. 2004;25(5) 

In This Article

Churg-Strauss Syndrome

Churg-Strauss syndrome is a rare condition seen almost exclusively in patients with asthma and characterized by the presence of systemic vasculitis, extravascular granulomatous inflammation, and eosinophilia.[23,24]

The most common radiological manifestations consist of transient, patchy, nonsegmental areas of consolidation without predilection for any lung zone.[24,25,26] The areas of consolidation can be symmetric or asymmetric and may have a peripheral distribution similar to that seen in chronic eosinophilic pneumonia[25,26] (Fig. 5). Less common manifestations include small or large nodules, unilateral or bilateral pleural effusions, and hilar lymphadenopathy.

Churg-Strauss Syndrome. (A) Computed tomographic (CT) scan at the level of the inferior pulmonary veins shows patchy bilateral areas of consolidation involving mainly the peripheral regions of the lower lobes. (B) CT scan at the level of the right hemidiaphragm shows bronchial wall thickening (arrows) and patchy peripheral areas of consolidation in the lower lobes. The patient was a 52-year-old man.

High-resolution CT frequently demonstrates bilateral ground-glass opacities with or without associated areas of consolidation. In one review of the high-resolution CT findings in 17 patients, 11 (60%) had areas of ground-glass attenuation or consolidation in either a patchy or a predominantly peripheral distribution.[27] Two patients had small centrilobular nodules, two had multiple nodules measuring 0.5 to 3.5 cm in diameter, and one had interlobular septal thickening. Similar findings were reported in a second study of nine patients with Churg-Strauss syndrome.[24]

Interlobular septal thickening and small effusions in patients with Churg-Strauss syndrome may be secondary to cardiac involvement or, occasionally, be seen in patients with Churg-Strauss Syndrome and normal cardiac function[27] (Fig. 6). A single case has been reported in which high-resolution CT demonstrated enlarged peripheral arteries, some of which had an irregular, stellate configuration and which correlated with the presence of vasculitis histologically.[28] Pleural effusions are seen on CT in ~30% of cases.[16] Hilar and mediastinal lymphadenopathy can occur but are uncommon.

Churg-Strauss Syndrome. (A) Posteroanterior chest radiograph shows small bilateral lower lobe consolidation, linear opacities, and small pleural effusions. (B) Computed tomographic image at the level of the carina demonstrates smooth thickening of the interlobular septa (arrows). The patient was a 69-year-old woman with no clinical or echocardiographic evidence of left heart failure. The septal thickening was due to pulmonary involvement.

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