Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Churg-Strauss syndrome
- Radiologic Findings
- Additional past history: Asthma, peripheral eosinophilia (31%), h/o sinusitis, allerginc rhinitis.
This patient had migrating infiltrations in the lung. The initial HRCT showed non-segmental and bilateral, lobular GGO in both cental lungs. Three months later, this patient had new, multiple, mass-like consolidations without cavitation and GGO in the right lung. At admission to our hospital after 20 months from the initial presentation, HRCT showed new, multiple, centrilobular nodules with non-segmental distribution in both lungs and mild bronchial wall thickening. Biopsy revealed eosinophilic infiltration in the interstitium and blood vessels. On PNS CT, the patient also had bilateral, maxillary sinusitis.
- Brief Review
- Churg-Strauss syndrome (allergic angiitis and granulomatosis) is a clinicopathologic entity of necrotizing vasculitis of small-sized vessel and extravascular granulomas by infiltration of eosinophil, mainly involving the lung, skin, kidney, GI tract, nervous system, paranasal sinus. Asthma and peripheral eosinophilia are observed in this syndrome. In Churg-Strauss syndrome, transient migrating pulmonary opacity is observed and bilateral, non-segmental and geographic lobular consolidation or ground glass opacity is common finding on CT. Multifocal centrilobular nodules with mild bronchial wall thickening is also observed. Differential dianosis includes chronic eosinophilic pneumonia and Wegener’s granulomatosis. Clinically, there are three distinct phases: (1) a prodromal phase that may persist for many years, consisting of asthma, often preceded by allergic rhinitis; (2) a second phase of marked peripheral blood eosinophilia and eosinophilic tissue infiltrates, which may recur over a period of years; and (3) a third, life-threatening vasculitic phase. 5-year survival rate is 60%.
American College of Rheumatology (ACR) 1990 criteria for the classification of Churg-Strauss syndrome (CSS). Classified as CSS if at least four of six criteria are present.
1. Asthma: History of wheezing or diffuse high-pitched expiratory rhonchi.
2. Eosinophilia: Eosinophilia >10% on differential white blood cell count.
3. Mono- or polyneuropathy: Development of mononeuropathy, multiple mononeuropathies, or polyneuropathy (glove/ stocking distribution) attributable to systemic vasculitis.
4. Pulmonary infiltrates, non-fixed: Migratory or transitory pulmonary infiltrates (not including fixed infiltrates). attributable to vasculitis.
5. Paranasal sinus abnormality: History of acute or chronic paranasal sinus pain or tenderness or radiographic opacification of the paranasal sinuses.
6. Extravascular eosinophils: Biopsy including artery, arteriole or venule showing accumulations of eosinophils in extravascular areas.
- Please refer to
Case 262, -
- References
- 1. Choi YH, Im JG, Han BK, Kim JH, Lee KY, Myoung NH. Thoracic manifestation of Churg-Strauss syndrome: radiologic and clinical findings. Chest. 2000;117:117-124.
2. Seo JB, Im JG, Chung JW, Song JW, Goo JM, Park JH, Yeon KM. Pulmonary vasculitis: the spectrum of radiological findings. Br J Radiol 2000;73:1224-1231.
3. Lanham, JG, Elkon, KB, Pusey, CD, et al. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome. Medicine 1984;63:65-81.
- Keywords
- Lung, Eosinophilic lung disease, Churg Strauss syndrome, Vasculitis,