特发性嗜酸粒细胞增多症、变应性肉芽肿性血管炎、非霍奇金淋巴瘤合并嗜酸粒细胞增多的临床特点比较

目的探讨特发性嗜酸粒细胞增多症(HES)、变应性肉芽肿性血管炎(CSS)、非霍奇金淋巴瘤(NHL)合并嗜酸粒细胞增多在临床表现、实验室检查、脏器受累等方面的异同,为三种疾病的鉴别诊断提供帮助。方法分析北京协和医院所有明确诊断为淋巴瘤、CSS的住院患者经查询病程存在慢性嗜酸粒细胞持续性升高者及所有明确诊断为HES的住院患者并加以比较,正态分布计量资料比较采用t检验,计量资料非正态和方差齐性条件,则采用非参数秩和检验,计数资料比较用χ2检验。结果 HES及CSS比较,嗜酸粒细胞绝对计数、IgE升高等均无统计学差异,而神经系统及肺部受累、淋巴结肿大、炎性指标升高等差异有统计学意义(P≤0.05)。CSS患者神经系统受累多见于外周神经,而HES多为中枢神经系统。CSS血管受累主要为血管炎,而HES多为动静脉血栓形成。HES与NHL合并嗜酸粒细胞增多比较,嗜酸粒细胞绝对值、淋巴结肿大、肺部受累、LDH升高均有统计学差异(均P≤0.05)。嗜酸粒细胞常见的浸润脏器如神经系统、胃肠道等在NHL合并嗜酸粒细胞增多病例中不多见。结论 HES、CSS、NHL合并嗜酸粒细胞增多三类疾病临床特点各有不同,诊断及鉴别需要通过仔细的脏器评估检查、实验室检查及密切的临床随诊。     

A case of nephrotic syndrome in a patient with Churg–Strauss syndrome

Renal involvement in Churg–Strauss syndrome (CSS) is not uncommon, but nephrotic syndrome is rarely reported in patients with CSS. A 25-year-old woman with a long history of bronchial asthma presented to our hospital with a chief complaint of generalized edema. Laboratory studies revealed normocytic normochromic anemia, marked eosinophilia, positive anti-neutrophil cytoplasmic antibody, hypoalbuminemia, and hypercholesterolemia. Urinalysis showed heavy proteinuria (4+) without significant casts. The 24-hour urinary protein excretion was 6.5 g with a selective index of 0.35. Echocardiography and X-ray of the paranasal sinuses suggested restrictive cardiomyopathy and maxillary sinusitis, respectively. Diagnoses of CSS and nephrotic syndrome were made on the basis of clinical and laboratory findings. Renal biopsy was performed, and pathologic findings revealed focal segmental glomerulosclerosis with mesangiolysis. The patient’s clinical symptoms and proteinuria improved markedly after combined treatment with corticosteroids and cyclophosphamide. Herein, we report a case of nephrotic syndrome in a patient with CSS.     

Cardiac involvements in hypereosinophilia‐associated syndrome: Case reports and a little review of the literature

Hypereosinophilia‐associated syndrome is a rare group of systemic diseases without certain underlying causes. Hypereosinophilic syndrome (HES) and eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg–Strauss syndrome (CSS), are initial considerations, when underlying causes remains unexplained despite of complete evaluation of hypereosinophilia. In this study, we report two rare cases, one case of HES with Loeffler endocarditis, and the other one of EGPA with restrictive cardiomyopathy mimicking myocardial infarction, to further address differential chief cardiac manifestations between HES and EGPA. Key roles of echocardiography played in detection of cardiac involvements, diagnosis, and prognosis prediction are also highlighted.     

Combined Churg–Strauss syndrome and allergic bronchopulmonary aspergillosis – case report and review of the literature

A rare case of combined Churg–Strauss syndrome (CSS) and allergic bronchopulmonary aspergillosis (ABPA) was presented. A 41‐year‐old woman was diagnosed with CSS based upon asthma, eosinophilia (23%), chest radiographic findings, paranasal sinusitis, peripheral neuropathy and positive p‐ anti‐neutrophil cytoplasmic antibodies (pANCA). The diagnosis of ABPA was established on the pathological findings of allegic mucin impaction and fungal hyphae on lung biopsy. It was further proved by positive serum IgE and IgG antibodies specific to afumigatus. The clinical investigation features were reviewed in the patients with combined CSS and ABPA. All patients had the time sequence of the development of CSS after ABPA uniformly, suggesting immunopathogenesis involving the emergence of CSS. The role of lung biopsy in the diagnosis of the condition was emphasized. Please cite this paper as: Ren S. Combined Churg–Strauss syndrome and allergic bronchopulmonary aspergillosis – case report and review of the literature. Clin Respir J 2013; 7: e6–e10.     

Churg–Strauss syndrome presenting with massive pericardial effusion

We describe a case of Churg-Strauss syndrome (CSS) presenting with a massive pericardial effusion without overt myocardial dysfunction. A 60-year-old man was referred to our hospital because of exertional dyspnea and fever. Initial chest multidetector-row computed tomography showed a massive pericardial effusion. The presence of eosinophilia, infiltrates of both lungs, pathological evidence of necrotizing vasculitis associated with eosinophilic infiltration, and history of asthma fulfilled the criteria of CSS. Massive pericardial effusion can be the first manifestation of cardiac involvement in CSS.     

The hypereosinophilic syndrome. Report of a case with successful medical treatment following cardiac biopsy

Together with pronounced blood eosinophilia an endomyocardial biopsy is the method of choice in diagnosing and assessing the severity of the systemic disorder hypereosinophilic syndrome (HES). Eosinophilia is not uncommon in several connective tissue diseases which may share clinical manifestations with HES. We report a case of HES, investigated and followed up with cardiac biopsies. A missing right radial artery pulsation gave rise to several differential diagnostic considerations.     

A case of hypereosinophilic syndrome presenting with intractable gastric ulcers

We report a rare case of hypereosinophilic syndrome (HES) presenting with intractable gastric ulcers. A 71-year-old man was admitted with epigastric pain. Initial endoscopic findings revealed multiple, active gastric ulcers in the gastric antrum. He underwent Helicobacter pylori (H pylori ) eradication therapy followed by proton pump inhibitor (PPI) therapy. However, followup endoscopy at 4, 6, 10 and 14 mo revealed persistent multiple gastric ulcers without significant improvement. The proportion of his eosinophil count increased to 43% (total count: 7903/mm3). Abdominal-pelvic and chest computed tomography scans showed multiple small nodules in the liver and both lungs. The endoscopic biopsy specimen taken from the gastric antrum revealed prominent eosinophilic infiltration, and the liver biopsy specimen also showed eosinophilic infiltration in the portal tract and sinusoid. A bone marrow biopsy disclosed eosinophilic hyperplasia as well as increased cellularity of 70%. The patient was finally diagnosed with HES involving the stomach, liver, lung, and bone marrow. When gastric ulcers do not improve despite H pylori eradication and prolonged PPI therapy, infiltrative gastric disorders such as HES should be considered.     

Hypereosinophilic syndrome complicated by myelofibrosis]

Hypereosinophilic syndrome (HES) with myelofibrosis was diagnosed in a 36-year-old man on the basis of bone marrow biopsy findings and clinical features. Although the patient was treated with steroid (1 mg/kg), hydroxyurea, and immunosuppressive therapy, eosinophilia persisted. Patients with HES and myelofibrosis are usually unresponsive to antineoplastic agents and/or immunosuppressants. However, cyclosporin may be an effective alternative for such patients.     

Churg-Strauss syndrome complicated by colon erosion, acalculous cholecystitis and liver abscesses

We report on a case of Churg-Strauss syndrome (CSS) with colon erosion, cholecystitis and liver abscesses. A 21-year-old woman with a history of bronchial asthma for 3 years was admitted with a complaint of abdominal pain. Laboratory findings included remarkable leukocytosis and eosinophilia, and a colonoscopy revealed erosion from the rectum to the ileocecal region. In addition, a colonic biopsy specimen showed necrotizing vasculitis and marked eosinophilic infiltration. On the basis of the clinical features and histopathological findings, she was diagnosed with CSS and subsequently treated with oral prednisolone, after which the eosinophilia and abdominal pain disappeared. However, on the 15th d in hospital she developed cholecystitis and liver abscesses. She was therefore treated with antibiotics and as a result went into clinical remission.     

Peripheral T-cell lymphoma not otherwise specified (PTCL-NOS) in a patient with hypereosinophilic syndrome showing multiple nodules on chest computed tomography

Hypereosinophilic syndrome (HES) encompasses both myeloproliferative and lymphoproliferative diseases. We encountered a rare case of lymphocytic HES followed by malignant T cell lymphoma, who was diagnosed as eosinophilic pneumonia upon the first visit. During the clinical course, the transition of the chest CT findings from bilateral multifocal ground-glass opacities and consolidations to bilateral scattered multiple small nodules was impressive and suggestive. Given the increased risk of developing T-cell lymphoma, patients with HES (especially lymphocytic-HES) should be monitored on a regular basis to detect this complication as early as possible.     

Multiple colonic ulcers caused by Churg-Strauss syndrome in a 15-year-old girl

Churg–Strauss syndrome (CSS), or allergic granulomatous angiitis, is an uncommon vasculitic syndrome that is found mainly in middle-aged adults. We describe a 15-year-old girl with CSS, diagnosed by histological findings and characteristic clinical features. The patient experienced two episodes of catastrophic gastrointestinal vasculitis, resulting in resection of 150 cm of small intestine and right hemicolectomy. Colonoscopic examination showed multiple colonic ulcers with active bleeding. The clinical course of the patient was grave and refractory to the therapy of steroid and cytotoxic drugs. In the world literature only two patients with multiple colonic ulcers caused by CSS have been reported, and very rare cases of childhood-onset CSS have been published. We reviewed CSS in children and found that the prognosis was poorer than that in adults. Received: 4 December 2000 / Accepted: 9 March 2001     

A Case with Multiple Colonic Ulcers Simulating Churg-Strauss Syndrome

Abstract: Churg-Strauss syndrome (CSS), a relatively rare disorder which is associated with serious complications, has a highly variable course and several possible manifestations. We present the case of a 35-year-old woman with a history of bronchial asthma, admitted for evaluation of lower abdominal pain and melena, whose clinical course had certain features in common with CSS. On admission, the white blood cell count was 45,300/mm³ with 65% eosinophils, and the serum immunoglobulin E (5,300 u/ml) level was remarkably elevated. At colonoscopy, there were shallow ulcers, irregular in shape, throughout the large intestine. Abdominal pain and melena were relieved by oral administration of prednisolone. Most previously reported cases have not been recognized as having colonic involvement until surgery or autopsy. In only a few reports of CSS and related disorders were colonoscopic examination findings described.     

Acute painless monocular visual loss due to central retinal artery occlusion in a patient with Churg–Strauss vasculitis

Ocular involvement in Churg–Strauss syndrome (CSS) is infrequent. We describe a case of a 50-year-old woman, with blood eosinophilia, involvement of the respiratory tract, skin, and peripheral nervous system, fulfilling the American College of Rheumatology criteria for CSS, who presented with left foot drop followed by left acute painless visual loss. Central retinal artery occlusion was diagnosed by fundoscopic findings (retinal whitening with a cherry-red spot). CSS was confirmed by sural nerve biopsy. Despite treatment with high-dose corticosteroids, cyclophosphamide, and anticoagulant therapy, visual acuity was not substantially improved. Acute blindness in CSS has been rarely described. Even more rarely, central retinal artery occlusion has been found to be the underlying cause of this infrequent clinical manifestation in CSS.     

Vertigo and Parinaud's syndrome as presentation of Churg-Strauss syndrome.

Intracranial complications are rare in Churg-Strauss syndrome (CSS). Cerebral infarctions are the most common intracranial presentation, usually after the clinical diagnosis of CSS had been established. We present a case of vertigo and Parinaud's syndrome as presentation of CSS in an asthmatic patient. Clinical examination revealed upward gaze limitation and bilateral midriasis. A cranial computed tomography scan showed a small round hypoattenuating lesion located in the right thalamic-mesencephalic region, which was later confirmed by magnetic resonance imaging. There was eosinophilia of more than 50%, and p-ANCA were positive. After steroid treatment was started, vertigo and diplopia resolved, and eosinophilia was reduced. After 24 months follow-up, the patient remains stable, with negative p-ANCA, taking 20 mg prednisone daily. Further magnetic resonance exams have shown findings that were similar to those of previous studies. This case shows how vertigo and transient diplopia may be the first symptoms of neurological complications in patients with CSS.     

Clonal Th2 lymphocytes in patients with the idiopathic hypereosinophilic syndrome

Idiopathic hypereosinophilic syndrome (HES) and Gleich's syndrome are related disorders characterized by persistent or recurrent hypereosinophilia of unknown origin. Elevated IgE levels and polyclonal hypergammaglobulinaemia are considered as markers of benign outcome in this setting as they are generally associated with predominant cutaneous manifestations and favourable response to glucocorticoid therapy. In a previous study, we identified a clonal population of CD3-CD4+ Th2-like lymphocytes secreting interleukin (IL)-5 and IL-4 in peripheral blood of a patient fulfilling the diagnostic criteria of HES with associated serum hyper-IgE. We now extend this observation by describing identical findings in three additional patients, and we compare their clinical and biological parameters with five other patients with HES. Chromosomal abnormalities were detected in purified CD3-CD4+ Th2 cells from three patients, among whom one developed anaplastic null cell lymphoma. We therefore suggest that a careful search for T-lymphocyte clonality and cytogenetic changes should be included in the work-up of HES for adequate management.     

A retrospective study of bronchial hyperresponsiveness in patients with asthma before the onset of Churg-Strauss syndrome.

Asthma is one of the most common clinical symptoms in Churg-Strauss syndrome (CSS). However, it is not known how lung function and bronchial hyperresponsiveness (BHR) prior to the development of CSS differs from asthmatics do not develop CSS. This retrospective cohort study was conducted to predict the onset of CSS and facilitate diagnosis in the early phase of the disease. We examined 24 pre-CSS asthmatic patients and 294 non-CSS asthmatic patients for clinical features, percent forced expiratory volume at 1 second (%FEV1), BHR to acetylcholine, and evaluated eosinophils (%) in the peripheral blood at their first hospital visit for asthma treatment. All of the 24 pre-CSS patients had adult-onset asthma. The asthma of 87.5% of pre-CSS patients at the first hospital visit before the onset of CSS was severe and was complicated by sinusitis. The eosinophils (%) in the peripheral blood was significantly higher than in non-CSS asthmatic patients. The %FEV1 in both the patients with severe asthma and the patients who developed CSS was lower than in patients with mild or moderate asthma. However, BHR in pre-CSS patients was significantly better than in non-CSS patients with severe asthma and was as mild as in patients with mild asthma. Patients who developed CSS had clinically severe asthma before the onset of CSS. The severity of their asthma was related to airflow limitation and eosinophilic inflammation in the peripheral blood, but not to BHR. These findings should prove useful in future early diagnosis and treatment of CSS.     

A case of Churg-Strauss syndrome discovered with hematemesis]

A 68-years-old Japanese woman was hospitalized emergently because of hemorrhagic gastric ulcer. For the hospitalization period, elevated levels of white blood cell count, eosinophilic leucocyte count, serum IgE and positive MPO-ANCA were recognized. With considering clinical course and these laboratory findings, we diagnosed Churg-Strauss syndrome (CSS). Steroid therapy in combination with cyclophosphamide was effective. CSS is a rare disease, but we should discriminate this disease when we encounter gastrointestinal bleeding of unknown etiology, especially PPI-resistant gastric ulcer.     

A case of hypereosinophilic syndrome presenting mid-ventricular obstruction

A 59-year-old man with hypereosinophilic syndrome (HES) who had been maintained with low-dose prednisolone for 5 years developed the characteristic features of hypertrophic cardiomyopathy. Left ventricular endomyocardial biopsy revealed no eosinophilic infiltration but extensive myocardial fibrosis. Cardiac involvement in HES presents as endocardial fibrosis, resulting in a clinical presentation of restrictive cardiomyopathy. HES heart disease can also present dilated cardiomyopathy, but myocardial hypertrophy has only rarely been noted in conjunction with HES. This report concerns a patient with HES who had clinical and hemodynamic evidence of asymmetric septal hypertrophy with mid-ventricular obstruction. Received: August 1, 2001 / Accepted: October 26, 2001     

Churg–Strauss syndrome: a retrospective study of 11 cases from a single center in Japan

Objective: To investigate the clinical characteristics of patients with Churg–Strauss syndrome (CSS), including symptoms, blood chemistry and immunological findings. Patients and methods: We retrospectively investigated the records of 11 patients (six female and five male) with CSS admitted to our hospital from September 2003 to October 2009. Results: Eight patients had preceding symptoms including bronchial asthma and allergic rhinitis. Seven patients showed eosinophilia. Nine patients had mononeuritis multiplex. Positive findings of myeloperoxidase‐antineutrophil cytoplasmic antibody (MPO‐ANCA) were found in five patients. Neither clinical manifestations nor laboratory findings were correlated with positivity for MPO‐ANCA. However, the MPO‐ANCA‐positive group showed a higher level of blood urea nitrogen and proteinuria than those negative for MPO‐ANCA. Ten patients recovered after starting steroid or immunosuppressive therapy, although one patient died of unknown etiology. Conclusion: Although general assessments based on various factors such as medical history, clinical manifestation and laboratory studies are indispensable in CSS, MPO‐ANCA might be useful as a predictor of renal dysfunction in patients with CSS.     

Management of hypereosinophilic syndrome: a prospective study in the era of molecular genetics

Hypereosinophilic syndrome (HES) is a heterogeneous group of disorders characterized by unexplained persistent primary eosinophilia causing end-organ damage. We conducted a prospective cohort study of patients fulfilling the diagnostic criteria for HES. Of 20 patients considered eligible for the study, 2 were found to have clonal myeloid disorders, limiting the diagnosis of "true" HES to 18 patients. No patient carried the FIP1L1-PDGFRA fusion gene or other imatinib-responsive translocations. A clonal interleukin-5-producing T-cell population was not detected in any patient. Common manifestations at presentation were pulmonary, cutaneous, and neurologic involvement; serositis; and gastrointestinal involvement. Only 3 patients developed cardiac involvement. Fifteen of the HES patients were administered first-line combined treatment with steroids and hydroxyurea. Nine patients achieved complete response, while 6 attained only partial response. Imatinib was administered to 3 HES patients who had been pretreated with steroids, resulting in complete hematologic and clinical response in 2 patients and no response at all in 1. Further treatment of the latter patient with steroids and hydroxyurea also proved ineffective. We conclude that the therapeutic approach should be individualized according to molecular findings. We consider the coadministration of corticosteroids and hydroxyurea to be an effective combination for the treatment of FIP1L1-PDGFRA-negative HES.