대한흉부영상의학회 Korean Society of Thoracic Radiology

Discussion

Diagnosis With Brief Discussion

Diagnosis

Churg-Strauss syndrome

Radiologic Findings

Chest radiograph shows a multifocal patchy consolidation with multiple nodular opacities and linear opacities in both lungs.
Axial chest CT shows multiple ill-defined centrilobular nodules, bronchial wall thickening and prominent interlobular septal thickenings, combined with patchy consolidations and GGOs in both lungs. Multiple mediastinal, hilar and interlobar LN enlargements are also seen.
PNS water’s view radiograph shows mild irregular mucoperiosteal thickening in both maxillary sinuses, suggesting sinusitis.
The patient had asthma, eosinophilia greater than 10% of the white blood cell differential count (10.8%), migratory or transient pulmonary opacities, and paranasal sinus abnormalities; the diagnosis of Churg-Strauss syndrome was made.
He was administered steroid pulse therapy. Follow-up chest CT scan showed markedly decreased extent of previous lesions but a newly developed focal consolidation in the RLL. Last follow-up chest PA, obtained 5 months later, revealed marked improvement of the previous consolidation, nodular opacities, and linear opacities.

Brief Review

Churg–Strauss syndrome (allergic angiitis and granulomatosis) is a rare vasculitis of unknown etiology defined by the 1994 International Consensus Conference as an ‘‘eosinophil-rich, granulomatous inflammation involving the respiratory tract and necrotizing vasculitis affecting small to medium sized vessels, with associated asthma and eosinophilia.’’
The most typical clinical presentation of Churg-Strauss syndrome (CSS) is the appearance of vasculitic manifestations in patients with known allergic rhinitis, sinus polyposis, and late onset asthma, usually preexisting for 5-10 years. General symptoms (i.e., fever or weight loss), mononeuritis multiplex, and/or necrotic cutaneous purpura are the most frequent manifestations at disease onset, in combination with elevated blood eosinophilia and inflammatory syndrome.
The diagnosis of CSS can be made if four or more of the following six findings are present: asthma, eosinophilia greater than 10% of the white blood cell differential count, neuropathy, migratory or transient pulmonary opacities, paranasal sinus abnormalities, and extravascular eosinophils revealed at biopsy.
At radiography, CSS usually appears as bilateral nonsegmental consolidations
or reticulonodular opacities.
The most common high-resolution CT manifestations consist of areas of ground-glass attenuation or consolidation in either a patchy or a predominantly peripheral distribution; the areas of consolidation can be transient. Less common abnormalities include small centrilobular nodules and, occasionally, larger nodules measuring 0.5 to 3.5 cm in diameter. Unilateral or bilateral pleural effusions are seen in approximately 30% of patients, and hilar or mediastinal lymphadenopathy in a small number of cases.
The primary therapy for CSS is systemic glucocorticoids. An additional immunosuppressive agent is typically added in patients with more advanced or refractory disease and in those whose disease flares with tapering of systemic glucocorticoids.

References

Keywords

Lung, Vasculitis,