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Loic Guillevin - One of the best experts on this subject based on the ideXlab platform.
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diagnosis and classification of eosinophilic granulomatosis with polyangiitis formerly named churg strauss Syndrome
Journal of Autoimmunity, 2014Co-Authors: Luc Mouthon, B Dunogue, Loic GuillevinAbstract:Recently, a group of experts in the field suggested to rename Churg-Strauss Syndrome as eosinophilic granulomatosis with polyangiitis (EGPA). This condition, first described in 1951, is a rare small- and medium-sized-vessel vasculitis characterized by an almost constant association with asthma and eosinophilia, and, by the presence of anti-myeloperoxidase (MPO) antineutrophil cytoplasm antibodies (ANCA) in 30-38% of the patients. Vasculitis typically develops in a previously asthmatic and eosinophilic middle-aged patient. Asthma is severe, associated with eosinophilia and extrapulmonary symptoms. Most frequently EGPA involves the peripheral nerves and skin. Other organs, however, may be affected and must be screened for vasculitis, especially those associated with a poorer prognosis, such as the heart, kidney and gastrointestinal tract, as assessed by the recently revised Five-Factor Score (FFS). Recent insights, particularly concerning clinical differences associated with ANCA status, showed that EGPA patients might constitute a heterogeneous group. Thus, EGPA patients with anti-MPO ANCA suffered more, albeit not exclusively, from vasculitis symptoms, such as glomerulonephritis, mononeuritis multiplex and alveolar hemorrhage, whereas ANCA-negative patients more frequently develop heart involvement. This observation led to the hypothesis that EGPA might be divided into different clinical and pathophysiological subtypes, which could be managed better with more specifically adapted therapies. For now, EGPA treatment still relies mainly on corticosteroids and, when necessary for patients with poorer prognoses, combined immunosuppressant drugs, especially cyclophosphamide. Overall survival of EGPA patients is good, despite not uncommon relapses.
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serum eosinophil cationic protein a marker of disease activity in churg strauss Syndrome
Annals of the New York Academy of Sciences, 2007Co-Authors: P Guilpain, Loic Guillevin, Jeanfrancois Auclair, Mathieu C Tamby, Amelie Servettaz, Alfred Mahr, Bernard Weill, Luc MouthonAbstract:The cytotoxic proteins released by activated eosinophils should play a role in the development of Churg-Strauss Syndrome (CSS). Eighteen patients (15 males and 3 females, age 41 +/- 13.3 years) with CSS according to the American College of Rheumatology criteria were included in the study. Thirteen serum samples from 11 patients were obtained at the time of disease flare, and the sera from 6 of them were also obtained at the time of clinical remission. Sera from seven other patients were obtained in clinical remission. Anti-neutrophil cytoplasm antibodies were detected in four (22.2%) patients. Fifteen healthy individuals were used as controls. Mean eosinophil count differed significantly between CSS patients with active disease and patients in clinical remission (3,407/mm(3) vs. 258/mm(3); P < 0.01), between CSS patients with active disease and healthy individuals (3,407/mm(3) vs. 211/mm(3); P < 0.01). Mean serum ECP levels differed significantly between patients with active or inactive disease (219 microg/L vs. 56.8 microg/L; P < 0.0001), between patients with active disease and healthy individuals (219 microg/L vs. 26.2 microg/L; P < 0.0001), but not between patients with inactive disease and healthy individuals (ns). Peripheral blood eosinophils count correlated with serum ECP during CSS flares disease (R = 0.6264; P < 0.05) and during periods of remission (R = 0.4798; P < 0.05). Our results support that ECP might be used as a disease activity marker in CSS.
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polyarteritis nodosa microscopic polyangiitis and churg strauss Syndrome
Lupus, 1998Co-Authors: F Lhote, Pascal Cohen, Loic GuillevinAbstract:Polyarteritis nodosa (PAN), first described by Kussmaul and Maier, is a well-known form of necrotizing angiitis whose manifestations are weight loss, fever, asthenia, peripheral neuropathy, renal involvement, musculoskeletal and cutaneous manifestations, hypertension, gastrointestinal tract involvement, and cardiac failure. Recently individualized from PAN, microscopic polyangiitis (MPA) is a systemic vasculitis of small-size vessels whose clinical manifestations are very similar to those of PAN, but it is characterized by the presence of rapidly progressive glomerulonephritis (RPGN), which is nearly constant, and pulmonary involvement usually absent in PAN. Churg Strauss Syndrome (CSS) is a disorder characterized by hypereosinophilia and systemic vasculitis similar to that of PAN and occurring in individuals with asthma and allergic rhinitis. Considering the etiologies of PAN, primary and secondary vasculitides can also be distinguished because PAN can be the consequence of hepatitis B virus (HBV) infection and sometimes of other etiologic agents. The prognosis of systemic vasculitides has been transformed by corticosteroids that are, except in HBV-related PAN, the basic treatment. Immunosuppressive drugs, especially cyclophophamide, have also contributed to improving the prognosis, but their precise role in the management of these vasculitides is still being elucidated.
Guillevin L - One of the best experts on this subject based on the ideXlab platform.
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Polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss Syndrome. Clinical aspects and treatment.
Rheumatic diseases clinics of North America, 1995Co-Authors: Lhote F, Guillevin LAbstract:In this article, three systemic vasculitides--polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss Syndrome--are reviewed, focusing on advances in the understanding of the pathogenesis, new classification nomenclature, and major clinical features of these vasculitides. Distinguishing criteria between polyarteritis nodosa and microscopic polyangiitis are also presented in detail, and recent advances in the clinical management of these diseases according to appropriate therapeutic strategies are recommended.
Bernhard Hellmich - One of the best experts on this subject based on the ideXlab platform.
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interferon alpha for maintenance of remission in churg strauss Syndrome a long term observational study
Clinical and Experimental Rheumatology, 2010Co-Authors: Claudia Metzler, Wolfgang L Gross, Elena Csernok, Bernhard HellmichAbstract:BACKGROUND Interferon-alpha has been successfully used for induction of remission in patients with Churg-Strauss Syndrome, but data on its ability to prevent relapses and its safety during long-term use are lacking. OBJECTIVES To examine the safety and efficacy of interferon-alpha for mainten-ance of remission in Churg-Strauss Syndrome. PATIENTS AND METHODS In a prospective open-label long-term observational study, 13 patients with CSS in stable remission received interferon-alpha (3 x 3 Mio. I.U/week s.c.) for maintenance of remission. Primary end point was the incidence of relapses. Secondary end points were the doses of concomitant prednisolone and the frequency adverse events. RESULTS After a median follow up of 64 month three patients were still on treatment with interferon-alpha all with a dose of 9 million units/week. In nine patients, interferon-alpha was discontinued for lack of efficacy (n=5), due to adverse events (n=2), or both (n=2) after median of 63 months (15-153) of therapy. A total of 3 major and 18 minor relapses occurred in 10 of the 13 patients with a median time to first relapse of 17 months (range 5-46). Sera of relapsing patients did not contain antibodies against interferon-alpha. In 6 relapsing patients treatment was switched to cyclophosphamide (n=4) or methotrexate (n=2). Four episodes of worsened asthmatic symptoms associated with a mild rise of blood eosinophils occurred in 3 patients and resolved following a transient increase of the oral prednisolone dosage. After 49 months one patient died probably due to a relapse. IFN-alpha was ceased prematurely, because of autoimmune-thyreoiditis in one, depression in another and cerebral leukoencephalopathy in two patients. Overall, 18 infectious episodes with need of antimicrobial treatment were observed.CONCLUSIONS. Recombinant interferon-alpha appears to be partially effective in the prevention of major relapses in patients with Churg-Strauss Syndrome. Due to numerous side effects and infections during long-term administration its use should be restricted to patients with contraindications against conventional immunosuppressive therapies.
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a phase ii study of interferon alpha for the treatment of refractory churg strauss Syndrome
Clinical and Experimental Rheumatology, 2008Co-Authors: Claudia Metzler, A Schnabel, W L Gross, Bernhard HellmichAbstract:Objective Uncontrolled vasculitis is the major cause of early death in Churg-Strauss Syndrome and standard therapy is not always effective. This study was carried out to examine the safety and efficacy of interferon-alpha for induction of remission in patients with refractory Churg-Strauss Syndrome. Methods In a prospective open-label trial, seven patients with Churg-Strauss Syndrome refractory to cyclophosphamide or methotrexate received interferon-alpha (3 million. I.U. 3 times weekly s.c.) for induction of remission. Primary end point was the successful induction of remission. Prednisolone was tapered according to the study protocol. Results All seven patients entered remission after 3 months of treatment. Five patients reached complete remission while in two patients, residual asthmatic complaints persisted. The mean Birmingham Vasculitis Activity Score (BVAS) decreased from 6.4 (+/-2.8) to 0 (+/-0) (p=0.017). Clinical improvement in response to interferon-alpha allowed to taper concomitant prednisolone according to the protocol from a mean dose of 20.4 mg/d (+/-13.3) to 6.9 mg/d (+/-1.9) (p=0.068). During a follow up of 6 months all patients remained in remission. In one patient, leukencephalopathy without clinical symptoms was seen on MRI after 61 months of treatment, as previously reported. In this cohort, no other cases of leukencephalopathy were observed during long-term follow-up. Constitutional symptoms related to the injection of interferon-alpha responded well to paracetamol. A transient leukopenia was found in two patients. Conclusion Interferon-alpha appears to be an effective and well-tolerated treatment for induction of remission in patients with refractory Churg-Strauss Syndrome.
Karina A. Keogh - One of the best experts on this subject based on the ideXlab platform.
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Leukotriene receptor antagonists and Churg-Strauss Syndrome: cause, trigger or merely an association?
Drug Safety, 2013Co-Authors: Karina A. KeoghAbstract:Concern has been raised in the medical literature that the use of leukotriene receptor antagonists for the treatment of asthma may be associated with an increased incidence of Churg-Strauss Syndrome, a rare small-vessel vasculitic Syndrome. This review provides a critical appraisal of the literature to address this question. The incidence of Churg-Strauss Syndrome in the general population is one to four cases per million. In patients with asthma it is 20–60 cases per million patient-years, which is similar to that seen in a population receiving leukotriene receptor antagonists. There is no evidence for a direct causative role of leukotriene receptor antagonists in the development of Churg-Strauss Syndrome. There may be multiple other non-causative reasons for an association, including the fact that these agents may be initiated in patients who are already in the process of developing Churg-Strauss Syndrome, or that the use of leukotriene receptor antagonists leads to a reduction in corticosteroid use, which in turn allows the Churg-Strauss Syndrome to be ‘unmasked’.
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churg strauss Syndrome clinical presentation antineutrophil cytoplasmic antibodies and leukotriene receptor antagonists
The American Journal of Medicine, 2003Co-Authors: Karina A. Keogh, Ulrich SpecksAbstract:PURPOSE: To determine the association of antineutrophil cytoplasmic antibodies (ANCA) and leukotriene receptor antagonists with disease activity in a large series of patients with Churg-Strauss Syndrome. METHODS: Potential subjects were identified by a computerized search of the Mayo Clinic Rochester database for the years 1990 to 2000. Patients meeting one of three classification schemes for Churg-Strauss Syndrome were included. RESULTS: Ninety-one patients met the inclusion criteria. Clinical manifestations were similar to those in previous reports. Mortality was similar to that in the general population. ANCA testing was performed in 74 patients. Seventy-three percent (n = 22) of the 30 patients tested before therapy were ANCA positive, as were 75% (n = 12) of the 16 patients tested during a disease flare. In comparison, 16% (n = 8) of the 49 tested during remission were ANCA positive. Serial measurements indicated a correlation of ANCA levels with disease activity. Central nervous system involvement was the only clinical manifestation that correlated with ANCA status (P = 0.05). Twenty-three patients received leukotriene receptor antagonists, of whom 16 (70%) began treatment before diagnosis and 6 (27%) began during remission. Two of those treated after diagnosis relapsed. In 1 patient the relation between disease and leukotriene receptor antagonist use could not be determined. Use of leukotriene receptor antagonists did not affect the time between onset of asthma and manifestations of vasculitis, and was not correlated with organ manifestations, except sinus disease. CONCLUSION: No one classification scheme identified all patients. Churg-Strauss Syndrome has a better prognosis than other ANCA-associated vasculitides. ANCA status correlates with disease activity, whereas a pathogenic role for leukotriene receptor antagonists in the development of Churg-Strauss Syndrome was not noted.
Luc Mouthon - One of the best experts on this subject based on the ideXlab platform.
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diagnosis and classification of eosinophilic granulomatosis with polyangiitis formerly named churg strauss Syndrome
Journal of Autoimmunity, 2014Co-Authors: Luc Mouthon, B Dunogue, Loic GuillevinAbstract:Recently, a group of experts in the field suggested to rename Churg-Strauss Syndrome as eosinophilic granulomatosis with polyangiitis (EGPA). This condition, first described in 1951, is a rare small- and medium-sized-vessel vasculitis characterized by an almost constant association with asthma and eosinophilia, and, by the presence of anti-myeloperoxidase (MPO) antineutrophil cytoplasm antibodies (ANCA) in 30-38% of the patients. Vasculitis typically develops in a previously asthmatic and eosinophilic middle-aged patient. Asthma is severe, associated with eosinophilia and extrapulmonary symptoms. Most frequently EGPA involves the peripheral nerves and skin. Other organs, however, may be affected and must be screened for vasculitis, especially those associated with a poorer prognosis, such as the heart, kidney and gastrointestinal tract, as assessed by the recently revised Five-Factor Score (FFS). Recent insights, particularly concerning clinical differences associated with ANCA status, showed that EGPA patients might constitute a heterogeneous group. Thus, EGPA patients with anti-MPO ANCA suffered more, albeit not exclusively, from vasculitis symptoms, such as glomerulonephritis, mononeuritis multiplex and alveolar hemorrhage, whereas ANCA-negative patients more frequently develop heart involvement. This observation led to the hypothesis that EGPA might be divided into different clinical and pathophysiological subtypes, which could be managed better with more specifically adapted therapies. For now, EGPA treatment still relies mainly on corticosteroids and, when necessary for patients with poorer prognoses, combined immunosuppressant drugs, especially cyclophosphamide. Overall survival of EGPA patients is good, despite not uncommon relapses.
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serum eosinophil cationic protein a marker of disease activity in churg strauss Syndrome
Annals of the New York Academy of Sciences, 2007Co-Authors: P Guilpain, Loic Guillevin, Jeanfrancois Auclair, Mathieu C Tamby, Amelie Servettaz, Alfred Mahr, Bernard Weill, Luc MouthonAbstract:The cytotoxic proteins released by activated eosinophils should play a role in the development of Churg-Strauss Syndrome (CSS). Eighteen patients (15 males and 3 females, age 41 +/- 13.3 years) with CSS according to the American College of Rheumatology criteria were included in the study. Thirteen serum samples from 11 patients were obtained at the time of disease flare, and the sera from 6 of them were also obtained at the time of clinical remission. Sera from seven other patients were obtained in clinical remission. Anti-neutrophil cytoplasm antibodies were detected in four (22.2%) patients. Fifteen healthy individuals were used as controls. Mean eosinophil count differed significantly between CSS patients with active disease and patients in clinical remission (3,407/mm(3) vs. 258/mm(3); P < 0.01), between CSS patients with active disease and healthy individuals (3,407/mm(3) vs. 211/mm(3); P < 0.01). Mean serum ECP levels differed significantly between patients with active or inactive disease (219 microg/L vs. 56.8 microg/L; P < 0.0001), between patients with active disease and healthy individuals (219 microg/L vs. 26.2 microg/L; P < 0.0001), but not between patients with inactive disease and healthy individuals (ns). Peripheral blood eosinophils count correlated with serum ECP during CSS flares disease (R = 0.6264; P < 0.05) and during periods of remission (R = 0.4798; P < 0.05). Our results support that ECP might be used as a disease activity marker in CSS.