Churg-Strauss Syndrome Associated with Montelukast Therapy

Churg-Strauss syndrome is a rare form of eosinophilic vasculitis associated with asthma. Several cases of eosinophilic conditions including Churg-Strauss syndrome have recently been reported in asthmatic patients being treated with antileukotriene receptor antagonists. However, whether these drugs have a direct pathogenic role remains controversial. We describe two patients who developed Churg-Strauss syndrome after starting treatment with montelukast.     

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     

A Case of Cronkhite-Canada Syndrome with Spontaneous Kemission

Abstract: A 64-year old woman visited our hospital with a chief complaint of diarrhea of nearly one year's duration. One month before seeking medical treatment at our hospital, she noticed dysgeusia, hyperpigmentation of the skin, and nail shrinkage and loss without hypoalbuminemia. Endoscopic examination demonstrated numerous sessile and semipedunculated polyps in the stomach and colon. Histological examination of biopsy specimens showed epithelial hyperplasia with cystic dilation, interstitial edema and infiltration of inflammatory cells. Based on these characteristic clinical and pathological features, a diagnosis of Cronkhite-Canada syndrome was made. As her symptoms gradually improved, she was placed under observation without specific treatment. The initial magnifying colonoscopy revealed enlargement of epithelial glands and cryptal dilation in both the protruding lesions and the intervening mucosa. On follow-up magnifying colonoscopic examination, small islets of abnormal glandular tissue persisted for 1.5 years but did eventually disappear. Magnifying colonoscopy may be useful for observation of the healing process in patients with Cronkhite-Canada syndrome.     

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 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.     

Low-Dose Cyclosporin for Multiple Colonic Ulcers Associated with Mixed Connective Tissue Disease

This report describes a patient with multiple colonic ulcers and mixed connective tissue disease. The histological findings of the colonic lesions showed vasculitis with T-cell infiltration, and the peripheral T cells were frequently in the activated phase of the cell cycle. In this patient, low-dose cyclosporin treatment (2.5 mg/kg/day) inhibited the T-cell activation in the peripheral lymphocytes and was very effective in the gastrointestinal disorder, which might be related to T-cell activation. This case suggests the possibility that even low-dose cyclosporin can exert a great influence on peripheral T cells and directly inhibit T-cell activation, thereby improving symptoms related to T-cell activation. Received: 3 June 1998 / Accepted: 26 October 1998     

Repressed ileal artery aneurysms in Churg-Strauss syndrome following combination treatment with glucocorticoid and cyclophosphamide

Churg-Strauss syndrome (CSS) is a systemic small-vessel vasculitis characterized by asthma and eosinophilia. We report a case of CSS complicated by multiple aneurysms in the small intestine. A 57-year-old man was admitted to our hospital with fever and blood eosinophilila. During admission, aneurysms in his small intestine ruptured and partial resection of the small intestine was required. After combination therapy of glucocorticoid (GC) and cyclophosphamide (CYC) for 6 months, aneurysms in the small intestine disappeared. The gastrointestinal involvements usually result in poor prognosis in CSS. However, combination therapy of GC and CYC might be effective for remission of aneurysms caused by small vessel vasculitis.     

Churg-Strauss syndrome as an unusual cause of spontaneous atraumatic intra-pericardial thrombosis.

Intra-pericardial thrombosis is usually due to haemorrhage into the pericardial space or after traumatic pericardiocentesis. An unusual case of spontaneous atraumatic intra-pericardial thrombosis due to Churg-Strauss syndrome, which responded well to immunosuppressive therapy, is presented.     

Churg-Strauss syndrome triggered by hyposensitization to Alternaria fungus

Churg-Strauss syndrome is a systemic vasculitis of unknown ethiology. Exposure to putative triggers have been described. We report a case of Churg-Strauss syndrome developing after specific immunotherapy with Alternaria fungus.     

Churg-Strauss syndrome and the leukotriene receptor antagonist pranlukast

The authors studied eight cases of Churg-Strauss syndrome (CSS) associated with the use of pranlukast, a common cysteinyl leukotriene receptor antagonist (LTRA) in Japan. The patients who received pranlukast showed significantly increased peripheral blood eosinophil count, neurological disability scores, and poor responses to corticosteroid in comparison with those patients not receiving pranlukast. We suggest that preceding administration of pranlukast aggravates clinical presentations of CSS.     

Successful Treatment of a Patient with Severe Churg-Strauss Syndrome by a Combination of Pulse Corticosteroids, Pulse Cyclophosphamide, and High-Dose Intravenous Immunoglobulin

A 24-year-old woman with a 4-year history of bronchial asthma suffered from bloody sputum, numbness of the extremities, elevated eosinophil count, and hypoxemia. A diagnosis of alveolar hemorrhage was made by bronchoalveolar lavage. Echocardiogram revealed severe hypokinesis of the left ventricular wall. Her respiratory condition deteriorated despite administration of pulse corticosteroids. A second pulse corticosteroids and pulse cyclophosphamide followed by high-dose intravenous immunoglobulin brought about a dramatic improvement of alveolar hemorrhage, cardiac impairment, and peripheral neuropathy. Levels of antimyeloperoxidase-antineutrophil cytoplasmic antibodies, soluble thrombomodulin, soluble interleukin-2 receptor, eosinophil cationic protein were elevated and returned to the normal range in remission. The combination of pulse corticosteroids, pulse cyclophosphamide, and high-dose intravenous immunoglobulin seemed effective for the acute phase of severe Churg-Strauss syndrome.     

Successful Treatment of a Patient with Severe Churg-Strauss Syndrome by a Combination of Pulse Corticosteroids,Pulse Cyclophosphamide,and High-Dose Intravenous Immunoglobulin

A 24-year-old woman with a 4-year history of bronchial asthma suffered from bloody sputum, numbness of the extremities, elevated eosinophil count, and hypoxemia. A diagnosis of alveolar hemorrhage was made by bronchoalveolar lavage. Echocardiogram revealed severe hypokinesis of the left ventricular wall. Her respiratory condition deteriorated despite administration of pulse corticosteroids. A second pulse corticosteroids and pulse cyclophosphamide followed by high-dose intravenous immunoglobulin brought about a dramatic improvement of alveolar hemorrhage, cardiac impairment, and peripheral neuropathy. Levels of antimyeloperoxidase-antineutrophil cytoplasmic antibodies, soluble thrombomodulin, soluble interleukin-2 receptor, eosinophil cationic protein were elevated and returned to the normal range in remission. The combination of pulse corticosteroids, pulse cyclophosphamide, and high-dose intravenous immunoglobulin seemed effective for the acute phase of severe Churg-Strauss syndrome.     

A Case of Kissing Esophageal Ulcers of Unknown Etiology

Abstract: A 41-year-old man with esophageal ulcers of unknown etiology is reported. Endoscopic examination was performed for evaluation of swallowing difficulty. The lesions were located in the middle portion of the esophagus. There were no findings correlating with either peptic ulcer or reflux esophagitis. In addition, he had no history of provoking factors, such as bacterial, chemical, traumatic or physical agents. Although the cause of these lesions was not clarified, we diagnosed acute benign ulcers following histological examination of the biopsy specimens. The patient was treated with a proton pump inhibitor and sodium alginate. The dysphagia and other symptoms subsided promptly, and the lesions healed completely within three weeks. We discuss herein the relationship between endoscopic findings and the etiology of kissing ulcer.     

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.     

Coronary involvement in Churg-Strauss syndrome

Systemic autoimmune diseases are themselves a relevant and independent risk factor for atherosclerosis and coronary ectasia. We describe a case of a 58-year-old Caucasian man who was admitted to our department for unstable angina. History of asthma, paranasal sinus abnormality, and peripheral eosinophilia given a high suspicion of Churg-Strauss syndrome (CSS). Diagnosis was performed with 5 of the 6 American College of Rheumatology criteria.The knowledge that CSS is often associated with significant coronary artery involvement and the persistence of chest pain led us to performing immediately a coronary angiography.Coronary angiography showed diffuse ectasic lesions, chronic occlusion of left anterior descending artery with homocoronary collateral circulation from left circumflex artery and subocclusive stenosis in the proximal tract of posterior descending artery.The early recognition of CSS, an aggressive invasive diagnostic approach, and an early appropriate therapy are important to prevent the progressive and permanent cardiac damage in these patients.In the setting of a multidisciplinary approach, careful cardiac assessment is an essential step in CSS, even in mildly symptomatic patients.     

Cardiac involvement in Churg-Strauss syndrome: a follow-up of three cases

Three cases of Churg-Strauss syndrome with myocardial involvementare reported. Cardiac disease is known to be the major causeof death in Churg-Strauss syndrome. Aggressive therapy (steroidsand cyclophosphamide) may cure the myocardial dysfunction associatedwith the disease.     

Double-balloon endoscopy-diagnosed multiple small intestinal ulcers in a Churg-Strauss syndrome patient

Churg-Strauss syndrome(CSS) is a systemic vascular disorder characterized by severe bronchial asthma hypereosinophilia,and allergic rhinitis.Small intestina ulcers associated with CSS are a relatively rare manifestation that causes gastrointestinal bleeding.Multiple deep ulcers with an irregular shape are characteristic of small intestinal involvement of CSS.Video-capsuleendoscopy(VCE),double-balloon endoscopy(DBE) and Spirus assisted enteroscopy have been developed recently and enabled observation of the small intestine In this case report,we have described a patient with CSS who had multiple deep ulcers in the jejunum detected by oral DBE.Since severe gastrointestinal(GI) involvement has been identified as an independent factor associated with poor outcome,the careful investigation of GI tract must be needed for CSS patients with GI symptoms.We describe the usefulness of DBE for diagnosis of small intestinal ulcers in patient with CSS.     

Allergic granulomatosis and angiitis in the absence of asthma and blood eosinophilia: a rare presentation of limited Churg-Strauss syndrome

Allergic granulomatosis and angiitis, also known as Churg-Strauss syndrome (CSS), is an uncommon vasculitis of unknown etiology. We report a 21-year-old male patient with fatigue, dry cough, and progressive dyspnea. He had no history of asthma or eosinophilia. Thorax computed tomography showed bullous/cystic areas with thin walls in varying sizes (5–15 mm). Histopathological examination of the open lung biopsy revealed granulomatous infiltration with histiocytes and eosinophilic leukocytes. This extremely rare variant of CSS is discussed.