Hypereosinophilic syndrome associated with antineutrophil cytoplasmic antibody]

A 68-year-old man was admitted to our hospital with complaints of fever, cough, and shortness of breath. He had several erythematous maculae on the trunk and experienced hypesthesia in his lower extremities. Laboratory data showed marked eosinophilia (20,235/mm3) and enhanced hepatobiliary enzymes. Chest X-ray films and computed tomographic scans revealed diffuse patchy infiltrative changes in the lungs. Histologic findings confirmed eosinophilic infiltration of the skin, liver, and lungs. A diagnosis of hypereosinophilic syndrome (HES) was made in accordance with clinical criteria proposed by Chusid et al. The patient was positive for antineutrophil cytoplasmic antibodies (a marker for vasculitis). This suggested a clinical picture resembling Churg-Strauss syndrome (CSS) despite the lack of bronchial asthma. The findings in this report could contribute to a better understanding of the diversity of HES cases, several of which are considered to represent a continuum of pathologies sharing an etiology similar to that of CSS.     

A case of allergic granulomatosis and angiitis without symptoms of asthma]

We present a case of allergic rhinitis in a 68-year-old woman in whom eosinophilia was found when she complained of common cold-like symptoms. The patient noticed a mass lesion on her left neck, which improved with antibiotic treatment, but her coughing continued and edema of both lower extremities appeared. She was admitted to our hospital, because of abnormalities in her electrocardiogram and cardiomegaly seen in a chest radiograph. The discomfort due to the edema in the soles of both feet remained even after steroid therapy. Her chest radiograph revealed ground-glass opacity, and a transbronchial lung biopsy revealed granulation tissue with the infiltration of eosinophils into the interstitium. Allergic granulomatosis angiitis was diagnosed because of granulomatosis vasculitis resulting from sural nerve biopsy. This was a rare case of allergic granulomatosis angiitis because her lung function was normal, she had no history of bronchial asthma, and there were no clear symptoms of bronchial asthma.     

Churg-Strauss syndrome (allergic granulomatous angitis) presenting with ileus caused by ischemic ileal ulcer

We report a rare case of Churg-Strauss syndrome (CSS) in a 41-year-old Japanese man with a history of middle-age onset of bronchial asthma who had severe abdominal pain. He presented with ileus caused by an annular ulcer of the ileum, attributable to mucosal ischemia resulting from necrotizing vasculitis of the mesenteric artery. He also had marked hypereosinophilia (51.5%), elevated serum IgE levels (34040 IU/ml), and generalized enlargement of the superficial cervical lymph nodes, containing eosinophilic granulomas. A stenotic lesion caused by an annular ulcer in the ileum was found and resected by laparotomy. Microscopic examination of the resected specimen revealed luminal narrowing or occlusion of small arteries in the ulcer base, subserosa, and mesenterium resulting from marked fibrotic intimal thickening with fragmentation or lack of the internal elastic lamina. These findings were diagnosed as vasculitis, scar stage. The postoperative course was uneventful, with the patient receiving a maintenance dose of prednisolone (10–15 mg/day) for 7 years subsequently. We must carefully diagnose and treat patients with middle-age onset asthma, because the symptom may be a lung manifestation of CSS, in which various organs including gastrointestinal tract are involved as a result of systemic necrotizing vasculitis. Received Jan. 30, 1997; accepted June 27, 1997     

Case of multiple intrapulmonary lymph nodes]

A 45-year-old man who had hypertension, hyperthyroidism, and bronchial asthma was admitted to our hospital because of a low-grade fever and chest pain. The physical findings and laboratory data were almost all within normal limits except for evidence of mild inflammation and liver dysfunction. The chest X-ray findings seemed normal, but a computed tomography (CT) scan showed multiple nodules in both lower lung fields. We suspected the cryptococcosis or lung cancer. Biopsy by video-assisted thoracoscopic surgery (VATS) yielded a diagnosis of multiple intrapulmonary lymph nodes. In cases with the above radiologic findings, careful attention should be paid to making the differential diagnosis between intrapulmonary lymph nodes and primary lung cancer. The promotion of diagnostic imaging and advances in techniques have made it easier to identify small peripheral nodules in the lungs, and we now know of their existence. Solitary intrapulmonary lymph nodes are encountered frequently, but multiple or increasing numbers of nodes, as in our case, are very rare. Moreover, because cases with elevated CEA levels have been reported, differentiation from lung cancer appears to be important.     

Churg-Strauss syndrome presenting with acute myocarditis and cardiogenic shock

Churg-Strauss syndrome (CSS) is a multisystem disorder characterised by asthma, prominent peripheral blood eosinophilia, and vasculitis signs. We report the case of a 22 year-old man admitted to the intensive care unit for acute myocarditis complicated with cardiogenic shock. Eosinophilia, history of asthma, lung infiltrates, paranasal sinusitis, glomerulonephritis, and abdominal pain suggested the diagnosis of CSS. Cardiac MRI confirmed cardiac involvement with a diffuse subendocardial delayed enhancement of the left ventricular wall, and a left ventricular ejection fraction (LVEF) of 30%. Acute myocarditis was confirmed with myocardial biopsy. The patient was successfully treated with systemic corticosteroids, intravenous cyclophosphamide, vasopressor inotropes, intra-aortic balloon pump and mechanical ventilation, and was discharged 21 days later. One year after diagnosis, the patient was asymptomatic. The eosinophilic cell count was normal. Follow-up MRI at one year showed LVEF of 40% with persistent delayed enhancement. Cardiac involvement by CSS requires immediate therapy with corticosteroids and cyclophosphamide, which may allow recovery of the cardiac function.     

Churg–strauss syndrome: importance of colonoscopy and endoscopic biopsy in establishing diagnosis

A 31‐year‐old Japanese man was admitted with complaints of left lower abdominal pain and diarrhea. He had a history of bronchial asthma since he was 26 years old. On admission, his eosinophil count was 4100/mm ³ and increased as high as 12 000/mm ³ . A neurological examination disclosed mononeuritis multiplex with paresthesia in both lower and upper extremities. Because of hematochezia, colonoscopy was performed and disclosed multiple colonic ulcers and erosions from the rectum to the ascending colon. Endoscopic biopsy showed fibrinoid necrosis of small‐sized vessels with moderate infiltration of eosinophils in the mucosa and submucosa. He was suspected of being a case with Churg–Strauss syndrome (CSS; allergic granulomatous angiitis), and prednisolone of 30 mg/day was administered. After the administration of prednisolone, his eosinophil count decreased dramatically. His symptoms also improved gradually, except for numbness in the extremities. Three weeks later, colonoscopy revealed improvement of the mucosal appearance, but the colonic ulcers and erosions were still present. Endoscopic biopsy showed exravascular granuloma with moderate infiltration of eosinophils in the submucosa. Colonoscopy and endoscopic biopsy may be important in establishing the diagnosis of CSS with intestinal symptoms.     

Treatment of asthma with antileukotrienes and Churg-Strauss syndrome

Since antileukotriene treatment for asthma was introduced, there has been debate about whether such therapy can lead to Churg-Strauss Syndrome (CSS) or whether CSS is simply inhibited by the use of steroids, as various authors have suggested. We report a case in which we suspected CSS in a patient with bronchopulmonary, cutaneous and analytical signs and whom we treated with oral steroids. After clinical improvement, one year later, steroids were replaced by antileukotrienes, after which the same clinical picture developed. The vasculitis characteristic of CSS was confirmed pathologically.     

Churg-Strauss Vasculitis in a Patient Treated with Omalizumab

Omalizumab is a new anti-IgE treatment for severe-persistent allergic asthma. In this case presentation, we report the clinical features of a patient with Churg-Strauss syndrome (CSS) diagnosed after five months of omalizumab treatment. Administration of anti-IgE quickly improved asthma symptoms and enabled the gradual reduction and suspension of systemic steroids. After the suspension of steroids, vasculitis became evident and CSS was diagnosed. Here, we report the clinical course of this patient to evaluate the efficacy of omalizumab in CSS.     

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.     

Churg-Strauss Vasculitis in a Patient Treated with Omalizumab

Omalizumab is a new anti-IgE treatment for severe-persistent allergic asthma. In this case presentation, we report the clinical features of a patient with Churg-Strauss syndrome (CSS) diagnosed after five months of omalizumab treatment. Administration of anti-IgE quickly improved asthma symptoms and enabled the gradual reduction and suspension of systemic steroids. After the suspension of steroids, vasculitis became evident and CSS was diagnosed. Here, we report the clinical course of this patient to evaluate the efficacy of omalizumab in CSS.     

Asthma bronchiale und Churg-Strauss-Syndrom

Churg-Strauss syndrome (CSS), which is synonymous with eosinophilic granulomatosis with polyangiitis, is characterized by mostly severe bronchial asthma, eosinophilia and systemic vascular inflammation which can have many different forms of organ manifestation. It represents an important therapy-relevant differential diagnosis of severe asthma. Typical features are a course which runs in phases with a prodromal phase, a phase of eosinophilic tissue infiltration and a vasculitis phase. Depending on the clinical presentation and detection of antineutrophil cytoplasm antibodies (ANCA) two phenotypes can be distinguished. Bronchial asthma in CSS is treated according to the standards for asthma therapy and in the vasculitis phase a combined therapy with systemic corticosteroids and immunosuppressants is often necessary depending on the severity of the disease and organ manifestations. Current data suggest positive effects of biologicals. A review of the pathogenesis, clinical features, diagnostics and therapy of CSS is given based on a current literature search.     

An elderly case of microscopic polyangiitis following bronchial asthma

A 78-year-old woman who had bronchial asthma for 15 years developed dysesthesia, neuralgia, muscle weakness and atrophy in both feet and her left hand. Prednisolone (maximum dose 80 mg) was not effective, and she displayed gait disturbance. Laboratory findings showed leukocytosis (neutrophils dominant), renal dysfunction, elevation of CRP and positive P-ANCA. Biopsy revealed vasculitis, and reduced density of myelinated fibers. Although steroid pulse therapy improved neuralgia and renal dysfunction, severe disability of motor function and sensory disturbance still remained. Vasculitis syndrome following bronchial asthma indicated Churg-Strauss syndrome (CSS), while severe renal dysfunction and lack of eosinophilia were symptoms compatible with microscopic polyangiitis (MPA). The present case showed properties of both MPA and CSS.     

Clinical analysis of intrapulmonary lymph nodes]

We encountered 12 cases (9 men, 3 women) of intrapulmonary lymph nodes, discovered by chest radiography or chest CT and identified by thoracoscopic lung biopsy (in 10 cases), open lung biopsy (1 case) or lobectomy (1 case). We also studied the literature related to intrapulmonary lymph nodes in Japanese. Many intrapulmonary lymph nodes were found in the lower lung field, few in the upper lung field. All intrapulmonary lymph nodes were spherical and were located under the pleura, but we were not able in some cases to differentiate them from malignancies by the CT scanfindings. We could not diagnose them or rule out malignancy before surgery. Pathological findings revealed that all of them showed anthracosis. Silicotic changes were found in three cases. We consider that thoracoscopy is useful in making a definite diagnosis if peripheral pulmonary lesions cannot be diagnosed. We emphasize that intrapulmonary lymph nodes should be taken into consideration in differential diagnoses of small nodular lesions in the lung.     

A case of hypereosinophilic syndrome associated with eosinophilic endocarditis

A 66-year-old woman, who had had bronchial asthma, was admitted to our hospital because she suffered from fever, productive cough, wheezing, dyspnea, and chest pressure sensation. Her chest X-ray showed migrating infiltration and marked cardiomegaly. Her ECG at the admission revealed abnormal Q wave and T wave inversion, though that of 3 years before had been almost normal. Hematology showed leukocytosis and eosinophilia of 8,000/mm3 without abnormal cells. All immunological tests were negative and the specific cause of the eosinophilia was unknown. 2 weeks after admission, she complained of severe chest pain suddenly and her ECG showed ST elevation on V1-4 and serum CPK level was elevated to 290 IU/l. By the thrombolytic agent and anticoagulant therapy, her symptom was lightened immediately. 2 months later, we made her cardiac catheterization and myocardial biopsy. Her LVG showed a small aneurysm of the apex, though her CAG was normal finding. The biopsy revealed moderate fibrosis and cellular infiltration including a few eosinophils. We thought that eosinophilic endocarditis had existed first, and secondary embolism continued led to the small infarction. The hypereosinophilia was spontaneously normalized 2 months after admission, but the patient complained of myalgia and sensory disturbance of extremities. The biopsy of quadriceps muscle could prove neither infiltration of eosinophils nor vasculitis. But we diagnosed mononeuritis multiplex due to hypereosinophilia. Judging from various symptoms and laboratory findings, this case was included to the hypereosinophilic syndrome. We also thought allergic granulomatosis and angitis as one of the differential diagnoses, but histologically vasculitis was not proved. In this case, eosinophilia was disappeared without using corticosteroids.(ABSTRACT TRUNCATED AT 250 WORDS)     

Three cases of small intrapulmonary lymph nodes coincidental with primary lung cancer]

We report three cases of intrapulmonary lymph nodes coincidental with primary lung cancers. In the first case, a 56-year-old man had a small subpleural nodule in the right lower lobe associated with adenocarcinoma of the right upper lobe. The small nodule was 10 mm in diameter and was difficult to differentiate radiologically from intrapulmonary metastasis. Wedge resection of this small nodule was performed. Histologic examination of the nodule revealed an intrapulmonary lymph node with anthracotic pigmentation. A right upper lobectomy for adenocarcinoma was performed, and was completely curative. The second patient, a 77-year-old woman, had adenocarcinoma in the right lower lobe diagnosed by transbronchial biopsy. Computed tomography (CT) revealed a small nodule 5 mm in diameter in the right upper lobe. Histologic examination of the small wedge-resected nodule revealed a lymph node. Lower lobectomy was performed with completely curative results. In the third case, a 63-year-old man was admitted for investigation of a mass shadow in the right upper lobe. CT showed a small nodular shadow in the right middle lobe, confirmed histologically to be lymphoid tissue, in addition to the original mass, which was diagnosed by transbronchial biopsy as squamous cell carcinoma. Upper lobectomy was performed and proved to be curative. Small intrapulmonary lymph nodes are very difficult to diagnose by methods other than surgical resection. When a small intrapulmonary nodule is detected in a patient with primary lung cancer, the presence of an intrapulmonary lymph node must be considered. If radiological characters compatible with an intrapulmonary lymph node are found and there is no other metastasis indicating poor prognosis, it should be considered as a possible indication for surgical resection.     

Familial Churg-Strauss syndrome in two sisters

Churg-Strauss syndrome (CSS) is an uncommon systemic vasculitis with an increase in the number of eosinophils in the peripheral blood and tissues. Its pathogenesis is unknown, and there is no evidence that genetic factors influence susceptibility to this disease. We present a case of familial CSS in two sisters with atopic-type bronchial asthma and negative perinuclear anti-neutrophil cytoplasmic antibody results. We investigated the human leukocyte antigen typing of the sisters and their six living siblings but found no evidence for heritability of CSS. To our knowledge, this is the first report of familial CSS.     

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.     

Rare manifestations of Churg–Strauss syndrome: coronary artery vasospasm, temporal artery vasculitis, and reversible monocular blindness—a case report

Churg–Strauss syndrome (CSS) is a rare illness with clinical findings characterized by asthma, eosinophilia, and vasculitis affecting medium and small-sized arteries and veins in a variety of organs. Involvement of the temporal arteries by non-giant cell eosinophilic vasculitis in CSS is quite rare and has only been published as isolated case reports or small patient series. Myocardial infarction due to coronary artery vasospasm is an unusual manifestation of CSS. We describe a case of a 39-year-old woman who had two myocardial infarctions due to severe coronary artery vasospasm and was diagnosed with CSS based on a temporal artery biopsy. During the course of her treatment, she also had another rare manifestation of CSS, monocular blindness reversible with immunosuppressive therapy.     

Churg–Strauss syndrome associated with leukotriene receptor antagonists (LTRA)

Churg–Strauss syndrome (CSS) is a rare vasculitic disorder that generally occurs in patients with bronchial asthma. CSS is being increasingly recognized in asthmatic patients treated with leukotriene receptor antagonists. However, the nature of this relationship remains to be elucidated. The present report describes three asthmatic patients who developed clinical manifestations highly suggestive of CSS, although one patient lacked the presence of eosinophilia. The patient, however, exhibited biopsy-proven cutaneous necrotizing vasculitis, which improved after withdrawal of montelukast. The second patient presented with systemic constitutional signs including fever, malaise, arthralgias, clinical jaundice, peripheral blood eosinophilia, and biopsy-proven eosinophilic hepatitis. The third patient also had circulating eosinophilia, scleritis, and arthritis. All patients improved after discontinuation of the leukotriene receptor antagonist (montelukast).     

Nephropathy,polyneuropathy, and gastroenteritis in a child with Churg–Strauss syndrome

Churg–Strauss syndrome (CSS) is a serious but rare pauci-immune vasculitis of small- and medium-sized blood vessels. It is commonly seen in association with bronchial asthma and/or allergic disorders. The syndrome is characterized by the presence of asthma, hypereosinophilia, and vasculitis in any part of the body. Vasculitis is often associated with significant distortion of normal functions. A rather severe case of CSS in an 8-year-old Nigerian girl with asthma and allergic rhinoconjunctivitis is reported. She presented with multiple morbidities, namely, vasculitic polyneuropathy and also nephritic–nephrotic syndrome that eventuated in acute renal failure after an onset of vasculitic gastroenteritis. Routine screening of all asthmatic patients for CSS is advocated.