Strauss Syndrome Research Articles

Heart involvement detected by magnetic resonance in a patient with Churg–Strauss syndrome, mimicking severe asthma exacerbation

Heart involvement detected by magnetic resonance in a patient with Churg–Strauss syndrome, mimicking severe asthma exacerbation

Heart involvement in Churg–Strauss syndrome: Retrospective study in French Burgundy population in past 10 years

Introduction Heart manifestations of Churg–Strauss syndrome (CSS) are varied. In the early stages of the disease, it is difficult to distinguish between lesions that are specific to CSS and those of other etiologies. The aim of our work was to compare the characteristics of patients with heart manifestations linked or not to Churg–Strauss syndrome. Material and methods We recorded all clinical symptoms of patients with CSS hospitalized between 1998 and 2008 in Burgundy, France, and determined the possible relationships between heart symptoms and CSS. Results From a cohort of 31 patients, we found 20 with heart lesions. When heart lesions were present, we noted fewer initial symptoms of digestive disorders ( p < 0.05), lower levels of lung infiltrates and fewer anti-MPO pANCA ( p < 0.05). Heart lesions were linked to CSS in 75% of cases. Their patients were thus younger than those in the other cardiac patients ( p < 0.05), were more likely to have clinical manifestations of heart involvement at diagnosis, were less likely to have lung infiltrates on the X-ray at diagnosis and during flare-ups and less likely to have lung abnormalities on X-rays during flare-ups ( p < 0.05) and higher level of leucocytes and eosinophils at diagnosis. Conclusion Heart lesions directly attributable to CSS are frequent, severe and probably underestimated. A specific physiopathology that is not mediated by ANCA seems to be involved in the genesis of CSS-related heart lesions.

Eosinophilic vasculitis of the spinal cord associated with Churg–Strauss syndrome

Eosinophilic vasculitis has been described as part of Churg–Strauss syndrome (CSS), but it also affects the central nervous system in < 10% of cases. However, spinal cord involvement in CSS has not been reported. We report a patient with myelopathic symptoms, with a two-year history of asthma. Laboratory tests revealed leukocytosis, with 31.7% eosinophils. A chest computed tomographic scan and radiography showed patchy bilateral areas of consolidation, predominantly involving the peripheral regions of the lower lobes. Spinal cord magnetic resonance imaging (MRI) revealed high-signal-intensity lesions at the C3–C5 and T1–T4 spinal levels on T2-weighted images. A microscopic evaluation revealed an eosinophil-rich inflammatory infiltrate with fibroid necrosis of a vessel wall, and granuloma formation in the small veins was not clearly visible. To the best of our knowledge, this is the first case of eosinophilic vasculitis of the spinal cord associated with CSS, and the patient responded well to steroids.

IL‐5 expression and release from human CD34 cells in vitro; ex vivo evidence from cases of asthma and Churg–Strauss syndrome

Eosinophils develop from hematopoietic CD34(+) progenitor cells in the bone marrow (BM) under the influence of Interleukin-5 (IL-5). The primary source of IL-5 is T-lymphocytes, although other sources may exist. The aims of this study were to determine whether CD34(+) cells from human peripheral blood (PB) and BM have the capacity to produce IL-5 when stimulated in vitro, and secondly, whether an elevated number of IL-5-producing CD34(+) cells can be found in situ in ongoing eosinophilic disease. CD34(+) cells from PB and BM were stimulated in vitro, and IL-5 production and release was assessed by ELISA, ELISPOT, flow cytometry and immunocytochemistry. Blood and BM from a patient with Churg-Strauss syndrome were analyzed by flow cytometry for CD34(+)/IL-5(+) cells, and immunohistochemical staining of CD34(+)/IL-5(+) cells in bronchial biopsies from an asthmatic patient was performed. Both PB and BM CD34(+) cells can produce and release IL-5 when stimulated in vitro. In the Churg-Strauss patient, IL-5-producing CD34(+) cells were found in PB and BM. Oral glucocorticoid treatment markedly decreased the number of IL-5-positive CD34 cells in the BM. CD34(+)/IL-5(+) cells were present in a patient with asthma. CD34(+) cells in blood and BM are capable of producing IL-5 both in vitro and in vivo in humans, arguing that these cells may have the capacity to contribute to eosinophilic inflammation. Consequently, targeting CD34(+) progenitor cells that produce and release IL-5 may be effective in reducing the mobilization of eosinophil lineage-committed cells in eosinophilic-driven diseases.

Mycophenolate mofetil therapy in a child with Churg–Strauss syndrome

Mycophenolate mofetil therapy in a child with Churg–Strauss syndrome

Lung involvement in Churg–Strauss syndrome as related to the activity of the disease

Lung involvement in Churg–Strauss syndrome as related to the activity of the disease

Complete remission of coronary vasculitis in Churg–Strauss Syndrome by prednisone and cyclophosphamide

The heart is involved in up to 50% of all patients with Churg–Strauss syndrome, but vasculitis of the coronary arteries has only been rarely documented. We present a young patient with severe coronary aneurysms and stenotic lesions due to a Churg–Strauss vasculitis. Prompt therapy with prednisone and cyclophosphamide resulted in the complete resolution of all lesions.

Neurologic complications of Churg–Strauss syndrome – a prospective monocentric study

Churg-Strauss syndrome (CSS) is a rare systemic vasculitis. Case series with a focus on neurologic involvement are not common. With this study, we intended to evaluate the frequency and types of neurologic manifestations and complications at time of diagnosis and during follow-up of patients with CSS. In this monocentric study, consecutive patients of our hospital with first diagnosis of CSS based on the criteria of the American College of Rheumatology were included between 2001 and 2007. Each patient underwent a periodic follow-up with clinical and electrophysiologic examination. Data were obtained prospectively. Fourteen patients were included. All patients had a hypereosinophilia and a history of asthma. Twelve of 14 patients had a neurologic involvement, mainly as an acute or subacute multiplex mononeuropathy (eight patients) or an axonal polyneuropathy (three patients). Three patients suffered from a neuropathy of cranial nerves, and two patients had a cerebral infarct. Mean follow-up period was 31 months. With immunosuppressive therapy, 13 patients had no additional neurologic complications, one patient suffered from a cerebral infarct. Initial neurologic symptoms as a result of peripheral neuropathy improved, but sequelae of axonal damage were persistently detectable. Even at time of diagnosis of a CSS, neurologic manifestations are common, especially as a multiplex mononeuropathy. With a consequent immunosuppressive therapy, new neurologic complications can be avoided for the most part.

Vascularites systémiques : critères de classification, scores d’activité et scores pronostiques

Systemic vasculitides are classified according to their clinical and biological presentations and main histological features. Several classification criteria have been proposed, including those of the American College of Rheumatology in 1990, and those of the Chapel Hill Consensus Conference in 1993, which are the most used. There are currently no validated and universally accepted diagnostic criteria. Several scores assessing the activity of vasculitis have been devised, including the Birmingham vasculitis activity score (BVAS), whose latest version is named BVAS 2003. However, these activity scores are of limited relevance in everyday practice, mainly because of their relative complexity and, more importantly, because the therapeutic goal in systemic vasculitis is to achieve complete and sustained remission, i.e. a score of zero for activity, and not merely the reduction by a certain percentage of these scores. The five-factor score (FFS) is a prognostic score, developed in 1996 by the French Vasculitis Study Group, which can help in choosing the most appropriate therapy for patients with polyarteritis nodosa, Churg–Strauss syndrome, or microscopic polyangiitis. Finally, there are scores evaluating cumulative damage related to vasculitis and its treatment, and their use has now become almost systematic in clinical studies. Many efforts are currently under way at the international level to develop, update and validate all these criteria and scores.

Kampo medicines improved blood test and QOL in two vasculitis cases of Churg&amp;ndash;Strauss syndrome and Henoch&amp;ndash;Sh&amp;ouml;nlein purpura after inadequate treatment with conventional therapies

Based on the tenets of traditional Chinese medicine (TCM) theory, Kampo medicines were selected and applied to two cases of Churg-Strauss syndrome and Henoch-Shönlein purpura. Two vasculitis syndrome patients exhibited persistent symptoms and abnormal blood tests after treatment with conventional therapies. As the two cases had "blood stagnation" and "damps and heat" and one had a "yang deficiency" in terms of TCM theory, we applied certain selected Kampo medicines. In case 1, the patient presented with hypereosinophilia, venous thrombosis, pulmonary infarction, decreased platelet count, ulner nerve palsy and Raynaud's phenomena, which led to a diminished quality of life. After starting the Kampo medicines, the patient improved quickly and recovered within 11 months. In case 2, persistent purpura, abdominal pain, and bloody feces quickly improved and disappeared after Kampo treatment. After starting the Kampo medicines, prednisolone was stopped at 21 days without any sign of relapse to date. Kampo medicines helped clear the persistent abnormal symptoms and laboratory findings of vasculitis syndromes, Churg-Strauss syndrome and Henoch-Shönlein purpura, which had responded inadequately to the conventional therapies administered.

Cytokines, chemokines and gene expresion patterns in BAL fluid of children after stem cell transplantation

received steroid pulse therapy to ITP and alveolar hemorrhage. The steroid was gradually tapered. Examination of open lung biopsy was performed, and that tissue supported the presence of pulmonary hemorrhage without capillaritis. Thus the diseases that cause capillaritis, for example, Wegener’s granulomatosis, druginduced, connective tissue disease and Churg–Strauss syndrome were denied. Immunofluorescence staining is negative, and then we diagnosed idiopathic pulmonary hemosiderosis (IPH). There are no articles about comorbid disorder of IPH and ITP. The girl of ITP and IPH are reported, but she also had systemic lupus erythematosus (SLE). We make a presentation with bibliographical consideration.

Churg–Strauss syndrome: evidence for disease subtypes?

Churg-Strauss syndrome (CSS) is a rare systemic small-vessel necrotizing vasculitis. Its main clinical characteristics, some potentially life-threatening, are now well known, as are its usual successive phases, from allergic rhinitis to asthma, and finally vasculitis. Conversely, physiopathogenetic mechanisms are not completely elucidated and clearly multiple, thereby suggesting the existence of different disease subtypes. Almost 40% of CSS patients have circulating antineutrophil cytoplasm autoantibodies (ANCAs), mostly directed against myeloperoxidase. ANCA-positive patients suffer more frequently from renal disease, peripheral nervous system involvement and/or alveolar hemorrhage, whereas frequent cardiac involvement, lung infiltrates and/or systemic manifestations are more common in those who are ANCA-negative. However, their respective global outcomes do not clearly differ. Patients might also be categorized according to other, more subtle clinical, radiological and/or biological parameters, for example, cardiac magnetic resonance imaging abnormalities or genetic background. Because of its practical and therapeutic repercussions, the priority remains the prompt, relatively easy identification of the most severely affected patients at CSS diagnosis, before searching for and trying to classify subsets. Large, collaborative studies are needed to determine whether other subgroups might be associated with outcomes and warrant different, and possibly new, therapeutic strategies.

Patient with Churg–Strauss syndrome complicated by acute progressive neuropathy who was successfully treated by prompt administration of an oral corticosteroid

The Journal of DermatologyVolume 37, Issue 1 p. 111-112 Patient with Churg–Strauss syndrome complicated by acute progressive neuropathy who was successfully treated by prompt administration of an oral corticosteroid Takehiko KANEKO, Takehiko KANEKO Departments of DermatologySearch for more papers by this authorYukiko NISHIYAMA, Yukiko NISHIYAMA Departments of DermatologySearch for more papers by this authorTomoko TAKEZAWA, Tomoko TAKEZAWA Allergy and Respiratory Medicine, The Fraternity Memorial Hospital, Tokyo, JapanSearch for more papers by this authorYasuo TO, Yasuo TO Allergy and Respiratory Medicine, The Fraternity Memorial Hospital, Tokyo, JapanSearch for more papers by this author Takehiko KANEKO, Takehiko KANEKO Departments of DermatologySearch for more papers by this authorYukiko NISHIYAMA, Yukiko NISHIYAMA Departments of DermatologySearch for more papers by this authorTomoko TAKEZAWA, Tomoko TAKEZAWA Allergy and Respiratory Medicine, The Fraternity Memorial Hospital, Tokyo, JapanSearch for more papers by this authorYasuo TO, Yasuo TO Allergy and Respiratory Medicine, The Fraternity Memorial Hospital, Tokyo, JapanSearch for more papers by this author First published: 24 January 2010 https://doi.org/10.1111/j.1346-8138.2009.00757.xCitations: 1 Takehiko Kaneko, M.D., Ph.D., Department of Dermatology, The Fraternity Memorial Hospital, 2-1-11 Yokoami, Sumida-ku, Tokyo 130-8587, Japan. Email: cew65890@nyc.odn.ne.jp Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article.Citing Literature Volume37, Issue1Special Issue: Systemic Sclerosis (pages 1-84)January 2010Pages 111-112 RelatedInformation

Recent progress in the genetics of Wegener's granulomatosis and Churg–Strauss syndrome

Recently, numerous studies have been performed to elucidate the genetic background of Wegener's granulomatosis and Churg-Strauss syndrome (CSS). Many of these investigations suffer from low statistical power, inconsistent case classification and other error-prone study designs. The majority of these findings has to be considered preliminary, if not spurious. We summarize the most important and robust findings. HLA-DPB1, the association of which with Wegener's granulomatosis has been discovered some years ago, is still the strongest and best replicated risk locus for this condition. Yet, no association is demonstrable for CSS, in which another HLA locus, HLA-DR, seems to be more important. Vice versa, a strong association with IL10 promotor polymorphisms was detected in CSS but not in a large Wegener's granulomatosis panel. Numerous other associations, including CTLA4, CD226 and copy number polymorphisms of FCGR3B still need further investigation, before reliable conclusions can be drawn. In order to be able to evaluate critically the genetic background of Wegener's granulomatosis and CSS future projects should take into account several aspects of study design. These preconditions include sufficient numbers of cases (i.e. statistical power) and a clear-cut classification of these cases, thus allowing differentiated analyses of certain disease subgroups.

Churg–Strauss syndrome: retrospective study in Burgundian population in France in past 10 years

Churg-Strauss Syndrome (SCS) is a systemic vasculitis associated with asthma and eosinophilia. The aim of our work is to describe this pathology in the Burgundian population in France. We counted from the hospitalisation data-processing summaries, the whole of the SCS hospitalised in Burgundy between 1998 and 2008. During the follow-up, the clinical and paraclinical characteristics of every patient were collected. The average prevalence is of 11.3 per million inhabitants and the incidence is of 1.2 new cases per million inhabitants per annum. There exists however, a great prevalence disparity and incidence amongst the various departments of the area. The patient's average follow-up is of 7.7 years. In 23% of the cases one finds a starting factor for vasculitis. The delay between the first signs and the diagnostic is an average of 61 months. The ANCA are positive in 26% of cases and of anti-myeloperoxidase specificity in 83% of cases (P < 0.001). The most profitable biopsies are essentially cutaneous and neuromuscular. At the diagnostic, two-third of the patients have had a treatment adapted according to the current recommendations based on the Five Factor Score. The remission rate within a 1-year period is of 77%. The remission is strongly correlated to the therapeutic protocol associating corticoids and cyclophosphamide (P < 0.05). In conclusion, the prevalence of SCS in our area is similar to that observed in other European regions. However, this vasculitis remains a difficult and often a tardive diagnostic pathology.

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.

Bowel perforations in a patient affected by Churg–Strauss syndrome under high-dose steroid treatment: will alternative drugs reduce risk of surgery?

Churg-Strauss syndrome is a relapsing-remitting vasculitis that frequently involves digestive system. Ischemic perforation of the large bowel is relatively rare and potentially life threatening. We report a case treated with high dose of steroids for a relapsing of Churg-Strauss vasculopathy that underwent emergency surgery for multiple large-bowel perforations. Massive use of steroids is common for controlling relapse of this disease, but this increases the risk of intestinal perforation. A prompt switching to alternative drugs when intestinal tract is involved should be considered in order to prevent surgery.

Diagnostic and therapeutic management of Churg–Strauss syndrome

Churg–Strauss syndrome is a rare small-vessel vasculitis that is associated with asthma, granulomatous inflammation, peripheral/tissue eosinophilia and a positive antineutrophil cytoplasmic antibody status (in approximately 40% of patients). The disease can be organ- and life-threatening, either due to tissue eosinophil infiltration such as myocarditis or due to vasculitis manifestations, for example glomerulonephritis. Furthermore, life-threatening disease can also occur due to the side effects of immunosuppression, for example, infection. A thorough diagnostic work-up should be performed in order to identify all organs involved and to rule out other disorders with similar features, such as hypereosinophilic syndrome. Therapeutic management is conducted according to disease stage and activity. Glucocorticoids remain the mainstay of therapy; however, further immunosuppressants (e.g., cyclophosphamide for life-threatening disease) are usually required. Future promising therapy options target cytokines involved in the disease process, such as IL-5.

Eosinophilia and Thrombophilia in Churg Strauss Syndrome: A Clinical and Pathogenetic Overview

During the past decade, there has been an increased description of Churg Strauss syndrome (CSS) characterized by vascular occlusions possibly linked to the thrombogenic potential of the eosinophil that is poorly appreciated. The purpose of this overview is 3-fold: the first to evaluate the available prevalence of thrombosis in Churg Strauss series, the second to demonstrate that any vascular district may be affected, and the third to describe the pathogenesis of thrombosis in CSS. A Pubmed, EMBASE, and Google search of CSS series from 1951 to date revealed a prevalence of arterial occlusion ranging between 3.1% and 18.7% and a prevalence of venous occlusion between 5.8% and 30%, whereas a specific survey for venous thromboembolism in CSS yielded a prevalence of 8.1%. Eosinophils store and release tissue factor as well as other cationic proteins: the former initiates coagulation while the latter inhibits natural anticoagulant activity and activate platelets eventually culminating in excessive thrombin generation and clot formation. In addition, antineutrophil cytoplasmic antibodies may shift the endothelial lining to proadhesive and prothrombotic surface. It is hoped that the review will represent a basis to foster novel research on this topic.

Epidemiology of ANCA associated vasculitis

ANCA associated vasculitis (AAV) comprises three syndromes with systemic vasculitis (Wegener’s granulomatosis (WG), Churg Strauss syndrome (CSS) and icroscopic polyangiitis (MPA)), which all involve small and medium sized vessels and are associated with antibodies against cytoplasmatic antibodies in neutrophils (ANCA). Polyarteritis nodosa (PAN) is included in this review as it also affects medium sized vessels, and has many clinical findings in common with the AAV.Since the recognition of ANCA, increasing data have become available on the epidemiology of these vasculitidis. WG constitutes half of the AAV and its prevalence has increased from 30/million in the late 1980’s in the USA to 160/million in this century in northern Europe. The prevalence for the whole group of primary systemic vasculitides is now 300/million in Sweden. The annual incidence of WG increased from 6.0/million to 14/million during the 1990’s in Tromsø, but it is unknown if this is a true increase or the result of an increased awareness of the diagnosis. For the whole group of AAV, the annual incidence in most more recent studies is relatively constant over time and by geographical location, ranging from 13 to 21/million. Nonetheless there are interesting differences in the prevalence of specific vasculitis between different geographical areas, as well as for sub specificities of ANCA.There seems to be a South-North gradient for WG and PR3-ANCA with high figures reported from northern Europe and southern New Zealand. In European studies WG is 90% PR3-ANCA positive. MPA which is predominantly MPO-ANCA associated are more frequent in the Mediterranean countries and also has an increasing gradient towards east-Asia, as almost all AAV in China and Japan are diagnosed as MPA, predominantly MPO-ANCA positive.There are also some ethnic and gender differences. WG is most prevalent among Caucasians in the USA and in people with European ancestors in Paris and in New Zealand, less frequent in Africans and Asians. Several studies have shown that the highest incidence of WG is in males 60-70 years old. Females are younger at onset, and in children WG is most frequent in girls. With better treatment (cyclophosphamide and corticosteroids), the survival of AAV has increased considerably. However disease control is not optimal, as most of the vasculitis present a remitting-relapsing course and organ damage is considerable. Hence, we are still looking to improve treatment regimens