RADIOLOGICAL DIAGNOSIS
Diagnosis of previous case
J Bras Pneumol 2004;30(1):87
Churg-Strauss Syndrome
High-resolution on 16-track multidetector scanner
Cross-sectional CT image and sagittal reconstruction demonstrate trickening of the interlobular septa mainly in the anterior lung regions. Also note marked peribronchial and perivascular thickening particularly in the left lower lobe and patchy ground glass opacities in the left lung.
Comments
Churg-Strauss syndrome es a rare systemic disease of unknown cause characterized by the combination of allergy, peripheral eosinophilia, necrotizing vasculitis, and extravascular granulomatous inflammation. The vast majority of patients are asthmatics. The main sites of involvement are the lungs heart, skin, and nervous system. Diagnosis requires the presence of at least four of the following manifestations: 1) asthma, 2) eosinophilia greater than 10%, 3) neuropathy, 4) transient or migratory pulmonary opacities, 5) sinusitis, and 6) extravascular eosinophils on biopsy.
The characteristic histologic findings consist of necrotizing vasculitis involving small and medium size vessels and extravascular necrotizing granulomas. A large number of eosinophils are present in the involved arteries and veins and the adjacent parenchyma.
The radiologic manifestations are variable and best demonstrated on high-resolution CT. The most findings on high-resolution CT consist of ground glass opacities or airspace consolidation involving mainly the peripheral lung regions. Less common manifestations include single or multiple nodules tha may occasionally cavitate, thickening of the peribronchial and perivascular interstitium and interlobular septa. The septal thickening can be secondary to left heart failure due to cardiac involvement by churg-Strauss syndrome or, less commonly, be secondary to parenchymal interstitial involvement by the disease. The patient being illustrated had normal cardiac function and biopsy proven parenchymal disease. Other manifestations of Ghurg-Strauss syndrome include unilateral or bilateral pleural effusions and, rarely, mediastinal lymphadenopathy. Bronchial wall thickening and bronchiectasis may be seen but these probably are manifestations of underlying asthma rather than Churg-Struss syndrome.
In a patient with asthma, the presence of interlobular septal thickening with or without associated peripheral ground glass opacities or consolodation is suggestive of churg-Strauss syndrome. The majority of patients show rapid improvement with corticosteroids. The differential diagnosis in this patient included insterstitial edema due to left heart failute and lymphangitic carcinomatosis.
References
1. Fraser RS, Müller NL, Colman N, Paré PD. Diagnosis of diseases of the chest. Fourth edition. WB Saunders Company. Philadelphia, 1999.
2. Worthy AS, Müller NL, Hansel DM, Flower CDR. Churg-Strauss Syndrome: The spectrum of pulmonary CT findings in 17 patients. AJR 1998;170:297-300.
3. Johkoh T, Müller NL, Akira M, Ichikado K et al. Eosinophilic lung diseases: Diagnostic accuracy of thin-section CT in 111 patients. Radiology 2000;216:773-780.
Acertadores do caso de Janeiro/Fevereiro 2004
André Luis dos Santos Becker - Hospital Fátima, Caxias do Sul, RS
Rubens Altair Amaral de Padua - Hospital Vaz Monteiro, Lavras, MG
Saulo Maia da Vila Melo - Hospital São Lucas, Aracaju, SE
Shirley Coletty dos Santos - Câmara dos Deputados, Brasília/DF
Publication Dates
-
Publication in this collection
17 June 2004 -
Date of issue
Apr 2004
