Computed tomography findings of paranasal sinuses in patients with eosinophilic granulomatosis with polyangiitis: Comparison with other eosinophilic sinus diseases and clinical relevance of their severity.

BACKGROUND: Although paranasal sinuses are one of the most representative organs affected by eosinophilic granulomatosis with polyangiitis (EGPA), they have not been studied sufficiently. The aim of this study was to compare computed tomography (CT) findings in paranasal sinuses of EGPA with those of other eosinophilic sinus diseases and elucidate the clinical relevance of their severity. METHODS: CT findings of paranasal sinuses in EGPA patients prior to therapeutic intervention (n = 30) were evaluated using the Lund-Mackay staging (LMS) system and compared with those of three control diseases [(NSAID-exacerbated respiratory disease (N-ERD), aspirin-tolerant asthma, and eosinophilic chronic rhinosinusitis without asthma (ECRS)]. We divided EGPA patients into three groups based on their LMS scores and examined their association with disease manifestation. RESULTS: Total scores of the LMS system in EGPA were significantly lower than those of N-ERD and ECRS without asthma. There was a large variation in total LMS scores in EGPA, suggesting considerable heterogeneity of their sinus lesions. Although EGPA with low LMS system scores showed only minor findings in maxillary and anterior ethmoid regions, those with high LMS system scores were characterized by high scores in the ostiomeatal complex. However, the frequencies of patients with a Five-Factor Score ≥2 and with cardiac involvement were significantly higher for EGPA with low LMS system scores. CONCLUSIONS: Although paranasal sinus lesions in EGPA were less severe than those of other eosinophilic sinus diseases, their milder CT findings may be associated with a higher frequency of extra-respiratory organ involvement.

Society for Cardiovascular Magnetic Resonance 2019 Case of the Week series.

The Society for Cardiovascular Magnetic Resonance (SCMR) is an international society focused on the research, education, and clinical application of cardiovascular magnetic resonance (CMR). The SCMR web site ( https://www.scmr.org ) hosts a case series designed to present case reports demonstrating the unique attributes of CMR in the diagnosis or management of cardiovascular disease. Each clinical presentation is followed by a brief discussion of the disease and unique role of CMR in disease diagnosis or management guidance. By nature, some of these are somewhat esoteric, but all are instructive. In this publication, we provide a digital archive of the 2019 Case of the Week series as a means of further enhancing the education of those interested in CMR and as a means of more readily identifying these cases using a PubMed or similar search engine.

ANCA-Negative Churg-Strauss Syndrome Presenting as Acute Multiple Cerebral Infarcts: A Case Report.

Eosinophilic granulomatosis with polyangiitis (EGPA, previously named Churg-Strauss syndrome) is a form of necrotizing vasculitis occurring in patients with asthma and eosinophilia. Ischemic stroke is a relatively rare complication of the disease. We report a case of a 63-year-old woman with multiple embolic infarcts, hypereosinophilia (for >7 years), and skin rash. Elevated cardiac enzymes and cardiac magnetic resonance imaging were consistent with endomyocarditis. The simultaneous presence of history of asthma, sinusitis, hypereosinophilia, and vasculitis led to the diagnosis of EGPA. This case contributes to the recent debate of the 2 possible presentations of the disease according to the ANCA (antineutrophil cytoplasmic antibodies) status. We furthermore underscore the need for careful differential diagnosis of the "ANCA negative" cases with persistent hypereosinophilia from the idiopathic hypereosinophilic syndrome.

Multiple Thromboembolic Cerebral Infarctions from the Aorta in a Patient with Churg-Strauss Syndrome.

BACKGROUND: Ischemic stroke is a rare complication of Churg-Strauss syndrome (CSS) and its pathogenesis has not been well clarified yet. We report a case of cerebral infarction in a patient with CSS due to embolism from a thrombus on the wall of the aorta. CASE: A 39-year-old man had multiple cerebral infarctions with symptoms of mild left hemiparesis and reduced vision. He was clinically diagnosed to have CSS based on remarkable eosinophilia, history of asthma, sinusitis, pulmonary infiltrates, and histologically proven extravascular eosinophilic infiltrates in the specimen of gastric mucosa. Cerebral angiography did not show any stenotic lesions in cerebral arteries. A thrombus was detected on the wall of the aorta by transesophageal echocardiography, which was considered as the source of embolism. The thrombus resolved on follow-up examination 3 months after the onset of the stroke. CONCLUSIONS: This is the first case report on cerebral infarction caused by aortogenic thromboembolism in a CSS patient. Other than cerebral vasculitis, embolism from cardiovascular system, including the wall of the aorta, is a possible cause of cerebral infarctions in a CSS patient.

Cardiopulmonary and Gastrointestinal Manifestations of Eosinophil- associated Diseases and Idiopathic Hypereosinophilic Syndromes: Multimodality Imaging Approach.

Eosinophil-associated diseases (EADs) are a diverse group of disorders characterized by an increase in circulating or tissue eosinophils. Cardiopulmonary and gastrointestinal system involvement can be due to primary EAD with no known cause or can be secondary to known systemic disease. The cardiopulmonary spectrum of EADs comprises simple pulmonary eosinophilia, acute eosinophilic pneumonia, chronic eosinophilic pneumonia, Churg-Strauss syndrome, allergic bronchopulmonary aspergillosis, bronchocentric granulomatosis, parasitic infections, and idiopathic hypereosinophilic syndrome. Eosinophilic gastrointestinal disorders include eosinophilic esophagitis, eosinophilic gastroenteritis, and eosinophilic colitis. Diagnosis is often challenging and requires a combination of clinical and imaging features along with laboratory findings. The absolute eosinophil count in peripheral blood and the percentage of eosinophils in bronchoalveolar lavage fluid are crucial in evaluation of various eosinophilic lung diseases. Although chest radiography is the initial imaging modality used in suspected cases of pulmonary EAD, multidetector computed tomography may demonstrate more characteristic pulmonary patterns, nodules, and subtle parenchymal abnormalities. Barium esophagography is used to assess mucosal abnormalities and the length and diameter of esophageal strictures. Magnetic resonance imaging is superior in providing valuable information in select patients, especially in evaluation of cardiac and gastrointestinal system involvement. Many patients require a multimodality imaging approach to enable diagnosis, guide treatment, and assess treatment response. Knowledge of the clinical features and imaging findings of the spectrum of EADs involving the lungs, heart, and gastrointestinal tract permits optimal patient care.

Rapid normalization of vasculitis-induced left ventricular dysfunction related with multiple cardiac thrombi.

We present a young female patient admitted in the emergency department with pulmonary edema, severely impaired left ventricular function, and simultaneous intracardiac thrombi in left and right ventricle as well as in right atrium, at echocardiography. A magnetic resonance tomography showed excess myocardial tissue edema and diffuse gadolinium enhancement. Blood analysis showed an elevated eosinophils count. The patient showed a rapid normalization of left ventricular function as well as resolution of intracardiac thrombi and myocardial tissue edema 3 months after proper treatment with cyclophosphamide and steroids for Churg-Strauss syndrome.

Kimura's disease associated necrotizing eosinophilic vasculitis presenting with recurrent peripheral arterial occlusive disease: a case report and review of the literature.

A 33-year-old Chinese man with 9-year history of Kimura's disease (KD) was admitted with a 1-month history of recurrent claudication. He did not have any clinical discomfort and had not taken any preventive medication in the past. He accepted percutaneous transluminal angioplasty and the pathologic diagnosis was reportedly consistent with necrotizing eosinophilic vasculitis. This is the rare reported case of KD associated necrotizing eosinophilic vasculitis presenting with recurrent peripheral arterial occlusive disease and the difficulties encountered in establishing an accurate diagnosis with unusual presentations. This case also highlights the possibility of recurrent complications without aggressive medical treatment in such unusual eosinophilic disorders.

Cardiogenic shock, asthma, and hypereosinophilia.

Eosinophilic granulomatosis with polyangiitis (EGPA) (formerly Churg-Strauss) is a multisystem syndrome associating asthma, hypereosinophilia,and signs of peripheral vasculitis. We report the case of a 21-year-old man admitted for cardiogenic shock revealing a severe left and right ventricular dysfunction. Hypereosinophilia, history of asthma, and peripheral neuropathy strongly suggested the diagnosis of EGPA. Cardiac magnetic resonance imaging confirmed heart involvement with a diffuse subendocardial late gadolinium enhancement. The patient was successfully treated with systemic corticosteroids, intravenous cyclophosphamide, and inotropes. At 6-month follow-up, the patient is doing well. Cardiac involvement is rare in EGPA and often carries poor prognosis. Specific early steroid therapy may improve hemodynamic at short term, allowing postponing the need for circulatory mechanical support or heart transplantation.

Eosinophilic Granulomatosis with Polyangitis Presenting as Cardiac Tamponade.

Eosinophilic granulomatosis with polyangitis (EGPA; earlier called Churg-Strauss syndrome) is a small-vessel necrotising vasculitis typically characterised by asthma, lung infiltrates, extra-vascular necrotising granulomas and hyper-eosinophilia. Cardiac disease is a major contributor to disease-related deaths in EGPA. We describe the case of a 39-year-old woman with late onset asthma, allergic rhinosinusitis, and high extra-vascular and peripheral blood eosinophilia, presenting with peripheral neuropathy and pericardial effusion. Early therapy with intravenous corticosteroids led to resolution of the pericardial effusion and significant clinical improvement. The present case also highlights the importance of being vigilant about potentially fatal cardiac complications in patients with EGPA.

[A clinical analysis of twenty-five cases of eosinophilic lung disease].

OBJECTIVE: To improve the diagnosis and treatment of eosinophilic lung disease. METHODS: Patients who were diagnosed with eosinophilic lung disease and hospitalized in the First Affiliated Hospital of Guangxi Medical University Hospital were retrospectively analyzed from January 2004 to August 2012. Data of etiology, clinical manifestation, imaging and pathological features, diagnosis and treatment were recorded. RESULTS: A total of 25 patients were diagnosed with eosinophilic lung disease including 9 chronic eosinophilic pneumonia, 6 churg-strauss syndrome, and 10 cases of parasitic infection of which two patients were the simple pulmonary eosinophilia (Löffler syndrome). Eosinophil counts in peripheral blood and bronchoalveolar lavage fluid (BALF) were increased. Arterial gas analysis showed varying degree of hypoxemia, which pulmonary function tests showed restrictive, obstructive, mixed ventilatory dysfunction. Chest CT showed bilateral flaky, streak or flake diffuse ground-glass infiltrates and reticular opacities. Results of pulmonary biopsy or skin biopsy identified diffuse eosinophil infiltration. Corticoidsteroid therapy alone or combined with immunosuppressive agents were both effective. CONCLUSION: (1) Liver fluke and other food-borne parasites are the most common causes in eosinophilic lung disease; followed by unexplained chronic acidophilic granulocyte pneumonia; (2) In addition to histopathological evidence, the diagnosis of eosinophilic lung disease was made comprehensively based on clinical features, laboratory test, the BALF analysis, and imaging data.

An aggressive and lethal course of Churg-Strauss syndrome with alveolar hemorrhage, intestinal perforation, cardiac failure and peripheral neuropathy.

Churg-Strauss syndrome (CSS) is a rare type of necrotizing vasculitis affecting small to medium-sized vessels typically characterized by asthma, lung infiltrates, necrotizing granulomas and hypereosinophilia. Herein, we describe a case of CSS presenting severe and aggressive course. A 35-year-old male patient with weight loss, dyspepsia, dyspnea and hemoptysis was admitted. The laboratory analyses indicated a remarkable eosinophilia, elevated levels of serum total IgE and positive cANCA. Thorax CT findings were suggestive of alveolar hemorrhage. Bronchoalveolar lavage revealed alveolar hemorrhage with eosinophilia and transbronchial lung biopsy showed eosinophilic vasculitis. Cardiac enzymes were increased and murmurs were audible revealing cardiomyopathy proven by echocardiography. Pulse cyclophosphamide and methyl prednisolone was immediately started. On the 21st day, intestinal perforation developed and urgent surgery was performed. During a follow-up, although a radiological improvement was observed in the chest X-ray, cardiac failure, peripheral neuropathy and skin lesions developed and high-dose intravenous immunoglobulin and anti-TNF therapy (adalimumab) were applied. Despite the therapy, he died from heart failure and septicemia at 68th day of therapy.

The mechanics of left ventricular dysfunction in patients with Churg-Strauss syndrome.

BACKGROUND: Heart is frequently involved in Churg-Strauss syndrome (CSS). However, the mechanics of left ventricular (LV) dysfunction in CSS has not been studied. OBJECTIVE: To assess the mechanics of LV function and to characterize the contribution of longitudinal, circumferential and rotational deformation to LV dysfunction in CSS. METHODS: We enrolled 22 CSS patients (eight males, mean age 43.2 ± 9.5 years) in remission of their disease and 22 sex- and age-matched healthy subjects. All patients underwent conventional and two-dimensional speckle-tracking echocardiography. Global longitudinal, circumferential and rotational deformation parameters were calculated. RESULTS: CSS subjects demonstrated lower LV ejection fraction (EF) than controls (56.6 ± 15.0% vs 63.8 ± 3.4%; P < 0.05). When compared to those with LVEF ≥ 50% (n = 14), CSS patients with LVEF < 50% (n = 7) had decreased global peak-systolic longitudinal and circumferential strain/strain rate (all P < 0.001) and tended to have lower global peak-systolic radial strain (P = 0.05). There were no differences between these two subgroups in global peak-systolic radial strain rate and LV twist/torsion. When comparing individual systolic and diastolic parameters early diastolic longitudinal and circumferential strain rate demonstrated the highest correlation with corresponding global longitudinal and circumferential peak-systolic strain/strain rate (r < -0.80, P < 0.001 for all correlations). CONCLUSIONS: In CSS LV systolic dysfunction strongly correlates with longitudinal and circumferential, but not radial or rotational systolic components, indicating that impaired LV systolic function may result predominantly from impaired contraction of inner and middle, but not outer myocardial fiber layers. The spatial correspondence between systolic and diastolic deformation parameters suggests the similar impact of pathologic process on systolic and diastolic function in CSS.

123I-MIBG imaging detects cardiac involvement and predicts cardiac events in Churg-Strauss syndrome.

PURPOSE: In Churg-Strauss syndrome (CSS) it is important to detect cardiac involvement, which predicts poor prognosis. This study evaluated whether (123)I-metaiodobenzylguanidine (MIBG) scintigraphy could detect cardiac damage and predict cardiac events in CSS. METHODS: (123)I-MIBG scintigraphy was performed in 28 patients with CSS, 12 of whom had cardiac involvement. The early and delayed heart to mediastinum ratio (early H/M and delayed H/M) and washout rate were calculated by using (123)I-MIBG scintigraphy and compared with those in control subjects. RESULTS: Early H/M and delayed H/M were significantly lower and the washout rate was significantly higher in patients with cardiac involvement than in those without and in controls (early H/M, p = 0.0024, p = 0.0001; delayed H/M, p = 0.0002, p = 0.0001; washout rate, p = 0.0012, p = 0.0052 vs those without and vs controls, respectively). Accuracy for detecting cardiac involvement was 86% for delayed H/M and washout rate and 79% for early H/M and B-type natriuretic peptide (BNP). Kaplan-Meier analysis showed significantly lower cardiac event-free rates in patients with early H/M ≤ 2.18 and BNP > 21.8 pg/ml than those with early H/M > 2.18 and BNP ≤ 21.8 pg/ml (log-rank test p = 0.006). CONCLUSION: Cardiac sympathetic nerve function was damaged in CSS patients with cardiac involvement. (123)I-MIBG scintigraphy was useful in detecting cardiac involvement and in predicting cardiac events.

Unusual finding in pediatric Churg-Strauss: renal lesions on CT.

After a 19-year-old female experienced several weeks of unrelieved fevers, an abdominal CT revealed multiple low-attenuation renal lesions. As the differential included lymphoma, infections and infarcts, a core biopsy of the kidney was performed, which revealed changes consistent with Churg-Strauss syndrome.