Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a case of allergic granulomatosis and angiitis of Churg-Strauss. The patient is a 40-year-old woman who satisfied the clinico-pathological triad of asthma, tissue and blood eosinophilia, and granulomatous vasculitis. We also measured serum eosinophil cationic protein (ECP) levels in this patient. In the active stage, the serum ECP level was higher than in healthy controls, declining in the remission stage, a finding suggesting that ECP is involved in the pathogenesis and course of Churg-Strauss disease.
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PMID:Allergic granulomatosis and angiitis of Churg-Strauss. A case of high serum level of eosinophil cationic protein. 135 59

Eosinophil cationic protein (ECP) is exclusively secreted only by the eosinophilic leukocyte. In this study the ECP concentration in the serum was measured in patients (n = 155) with various skin disorders and compared with the number of circulating eosinophils. The presence of activated eosinophils in the skin was also studied immunohistochemically using the monoclonal antibody EG-2, which recognizes both the eosinophil protein X (EPX/EDN) and ECP. EG-2 distinctly revealed these proteins in the eosinophils and their granules. Non-activated eosinophils were studied with the monoclonal antibody EG-1. In most cases this did not disclose any more eosinophils and often it was located more diffusely and not seldom on collagen fibers. Elevated serum ECP but normal numbers of circulating eosinophils were found in half of the patients with progressive plaque psoriasis and long-standing daily chronic urticaria. In patients with prurigo nodularis, papular erythematous eruptions, vasculitis, purpura and toxic drug reactions, Wells' syndrome, porphyria cutanea tarda and persistent light reaction the serum ECP was increased, although in some cases the number of circulating eosinophils was normal. In these disorders an increased number of activated eosinophils was found in the skin. Both serum ECP and the number of activated eosinophils normalized when the patients' condition improved. In atopic dermatitis the serum ECP and the number of activated eosinophils in the skin were increased only during exacerbation of the disease. High serum levels of ECP and activated eosinophils in the skin are frequent findings in many skin disorders in spite of often normal blood eosinophil counts.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Eosinophilic cationic protein (ECP) in skin disorders. 168 31

Mouse monoclonal antibodies were raised to the storage and secreted forms of eosinophil cationic protein (ECP), and were used to study the presence of activated eosinophils and secreted ECP in the tissues of patients with a variety of allergic diseases. Immunocytochemical localization was shown with alkaline phosphatase-linked second antibodies, and fast-red substrate. Deposition of a red reaction product indicated sites in tissues where eosinophils had become activated, and where secreted ECP was present. Activated eosinophils and secreted ECP were found together in (1) skin lesions of patients with chronic urticaria, (2) gut lesions of patients with eosinophilic gastroenteritis and ulcerative colitis, and (3) tissues containing granulomas in patients with allergic granulomatosis and vasculitis - the Churg and Strauss syndrome. These results show the value of these techniques for determining the sites of eosinophil activation and secretion in allergic diseases. They support the suggestion that ECP may be involved in the development of these tissue lesions.
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PMID:Tissue localization of human eosinophil cationic proteins in allergic diseases. 400 81

We reported clinical and laboratory findings of 5 patients with Churg-Strauss syndrome (CSS), especially association with asthma symptoms. Subjects included 3 males and 2 females with a mean age of 53.8 year-old. In all 5 patients symptoms of neuropathy; mononeuritis multiplex and in some patients, other vasculitic symptoms; fever, diarrhea, abdominal pain and skin eruptions, were noted. These clinical features and laboratory findings; marked peripheral eosinophilia and elevation of serum ECP were normalized after steroid therapy. We investigated the relation between the occurrence of CSS and the symptoms of asthma. The mean duration of asthma in this series was 17.2 years, and 4 cases were atopic and one was non-atopic asthma. In previous publications, asthmatic symptoms were severe at the onset of the disease and progressed thereafter. In our 5 cases, however, the severities of bronchial asthma were mild of two cases, moderate of two and severe of only one, moreover severe asthmatic attacks were shown in only 2 cases when the manifestation of systemic vasculitis occurred. In conclusion, although CSS has been thought that one of complications of bronchial asthma, the occurrence of CSS are not necessarily correlated with symptoms of bronchial asthma.
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PMID:[Clinical features of allergic granulomatosis and angiitis (Churg-Strauss syndrome). Association with asthma symptoms]. 899 May 26

Churg-Strauss syndrome (CSS) is a form of primary vasculitis characterized by allergy and angiitis. In the organ systems involved (lung, heart, peripheral nervous system, and so forth), eosinophilic infiltration can be found. Eosinophilia and normochromic normocytic anemia are leading laboratory findings together with elevated IgE. New seromarkers for the activation of endothelial cells, lymphocytes, and eosinophils (soluble thrombomodulin, soluble interleukin-2 receptor, eosinophil cationic protein) may be able to predict a relapse. Antineutrophil cytoplasmic antibodies are found in only approximately 50% of all patients with CSS, and their diagnostic value is questionable. Etiologically, hyperresponsiveness to an antigenic stimulus seems to underlie the syndrome. In asthmatics, cysteinyl leukotriene receptor type 1 antagonists are reported to trigger the disease. Cytokine profile findings on the cells involved in CSS remain contradictory. Some think CSS may be a Th2-mediated disease; its pathophysiology is not known fully. Interleukin-5 and tumor necrosis factor-alpha are elevated in serum and fluid of bronchoalveolar lavage, suggesting target cytokines for future treatment protocols. Treatment consists of glucocorticoid monotherapy. Data on outcome and effectiveness is lacking for other immunosuppressive regimens, such as cyclophosphamide or glucocorticoid plus cyclophosphamide. Treatment with interferon-alpha has been effective in patients refractory to glucocorticoid plus cyclophosphamide.
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PMID:Churg-Strauss syndrome: update on recent developments. 1179 Sep 90

Clinicopathological studies were performed on the visceral organs and the sural nerve of a male patient with Churg-Strauss syndrome (CSS) in order to understand the mechanisms of peripheral nervous system damage. A 67-year-old man, with a 2-year history of bronchial asthma, developed acutely painful paraplegia and dyspnea. Laboratory data showed a leukocytosis, an elevated serum creatinine kinase (CK) and marked eosionophilia. Autoantibodies including p- and c-ANCA were negative. Electrophysiological studies revealed a severe sensory-motor neuropathy of multiple mononeuritis type. Steroid pulse therapy performed a day after biopsy of skin, muscle and sural nerve was effective in resolving his respiratory and neurological dysfunction but a perforation of an intestinal ulcer occurred which required surgical intervention. In the biopsied sural nerve and the surgically resected intestine and mesentery there was vasculitis with fibrinoid necrosis accompanied by numerous eosinophils and macrophages containing eosinophil cationic protein (ECP). These findings suggest that in addition to ischemic changes due to vasculitis some neurotoxic substances generated by the eosinophils may be involved in the development of neuropathy in CSS.
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PMID:Clinicopathological studies of peripheral neuropathy in Churg-Strauss syndrome. 1256 71

Churg-Strauss syndrome (CSS) is a rare multi-system vasculitis; some cases have been reported in Korea. The aim of this study is to describe the clinical features, treatment outcome, and long-term follow-up of CSS from a single Korean medical center. Between 1995 and 2004, seventeen patients were diagnosed with CSS at the Department of Medicine of the Samsung Medical Center, Sungkyunkwan University School of Medicine. The diagnosis of CSS is based on the classification criteria of the American Collage of Rheumatology. All patients had asthma. As in other case series, the lung, peripheral nervous system, and skin were the most commonly involved organs. During the active stage of the disease, most of the patients exhibited peripheral blood eosinophilia and an elevated serum eosinophil cationic protein level. Ten patients were treated with pulses of methylprednisolone followed by tapering and cyclophosphamide, and the others were treated with corticosteroids alone. The outcomes after long-term follow-up were generally good. One patient who was refractory to initial treatment died of heart failure during the follow-up period. CSS was highly variable in its presentation and course. The manifestations may range from mild symptoms to life-threatening conditions. The outcome after long-term follow-up was as good as that of previous studies.
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PMID:Churg-Strauss syndrome: the clinical features and long-term follow-up of 17 patients. 1661 12

Churg-Strauss syndrome (CSS) is a rare form of systemic vasculitis occurring in patients with asthma and hypereosinophilia; however, its mechanisms involved in the severe tissue inflammation with vasculitis are poorly understood. High mobility group box 1 (HMGB1) protein, originally identified as a DNA binding protein, also has potent pro-inflammatory and proangiogenic properties. In this study, we hypothesized that HMGB1 might be associated with CSS, and examined serum HMGB1 levels and compared those of asthma patients and healthy volunteers. We also investigated HMGB1 expression in the lesion, and eosinophil HMGB1 amount in CSS patients. We found that the serum HMGB1 levels in CSS patients were significantly higher than those of asthma patients and healthy volunteers. Eosinophils in the CSS lesion expressed HMGB1 and HMGB1 level in eosinophils from CSS patients was significantly higher than that of asthma patients, while there was no significant difference in HMGB1 levels in peripheral mononuclear cells. The serum HMGB1 level in CSS patients decreased after the steroid therapy, and showed significant positive correlations with several molecules, including soluble interleukin-2 receptor, soluble thrombomodulin, and eosinophil cationic protein in sera. We propose that HMGB1 might contribute to the pathogenesis of CSS.
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PMID:Increased serum high mobility group box-1 level in Churg-Strauss syndrome. 1905 49

Kawasaki disease (KD) is a systemic febrile vasculitis particular coronary artery involvement. Eosinophilia has been found in our and other studies in KD. This study further investigates whether eosinophil-related T helper 2 (Th2) cytokines or the activation marker (eosinophil cationic protein - ECP) is involved in KD with coronary artery lesions (CAL). A total of 95 KD patients were enrolled for this study. Plasma samples were subjected to the measurement of interleukin (IL)-4, IL-5, and eotaxin by Luminex-Bedalyte multiplex beadmates system and to the measurement of ECP by fluoroimmunoassay. Patients with KD had higher eosinophils than controls. Eosinophil-related mediators: IL-4, IL-5, eotaxin, and ECP levels were also higher in KD patients than controls before intravenous immunoglobulin (IVIG) treatment. After IVIG treatment, ECP decreased but IL-4, IL-5, and eotaxin increased significantly. The higher the IL-5 and eosinophil levels after IVIG treatment, the lower rate of CAL was found. Changes of eosinophils after IVIG treatment were positively correlated to changes of IL-5 levels but not ECP levels. An increase of eosinophils and IL-5, but not ECP levels after IVIG treatment, was inversely correlated with CAL formation in KD.
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PMID:Association of lower eosinophil-related T helper 2 (Th2) cytokines with coronary artery lesions in Kawasaki disease. 1943 83

An increased risk of thrombosis has been described in patients with hypereosinophilic syndromes, including Churg-Strauss syndrome (CSS). We report the case of a 43-year-old man with CSS who presented with asthma, pansinusitis, blood eosinophilia (9650/microL), peripheral neuropathy, cutaneous eosinophilic vasculitis, and a positive result for antineutrophil cytoplasmic antibodies. An analysis of plasma during active disease revealed elevated levels of prothrombin fragment 1+2 (marker of thrombin generation) (832 pM; normal range, 68-229 pM) and D-dimer (marker of fibrin degradation) (2300 ng/mL; normal range, 130-250 ng/mL), which indicate an increased risk of thrombosis. Both parameters returned to normal values during remission after immunosuppressive treatment. Skin histology showed leukocytoclastic vasculitis with numerous eosinophils in the dermal infiltrate. Immunohistochemistry revealed expression of tissue factor by skin-infiltrating eosinophils, as confirmed by colocalization with eosinophil cationic protein, a classic marker of eosinophil granulocytes. In conclusion, we present a patient with active CSS and a prothrombotic state that reverted during remission achieved by immunosuppressive therapy.
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PMID:Prothrombotic state in Churg-Strauss syndrome: a case report. 2131 5


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