Churg-Strauss Syndrome In Patient Research Articles

Cases of Churg-Strauss syndrome in patients receiving montelukast

Churg-Strauss syndrome is a rare disorder, but in patients with asthma it may develop as an adverse effect of the administered drugs. The aim of this study was to investigate possible causal relationship between montelukast and the occurrence of Churg-Strauss syndrome. Medical literature was reviewed by searching the databases 'Medline' and 'Googlescholar', in order to detect published cases of Churg-Strauss syndrome associated with use of montelukast. In this article is included 13 publications which contain the following keywords: montelukast, Churg-Strauss syndrome and side effects. Relationship between use of montelukast and development of Churg-Strauss syndrome was not clearly causal, although montelukast was associated with development and relapse of the syndrome. This fact supports the hypothesis that leukotriene antagonists are involved in the pathogenesis of this serious disease. Special attention should be paid to appearance of new symptoms in an asthmatic patient, already treated with corticosteroids, who start receiving leukotriene antagonists, especially if the dose of corticosteroids is reduced. Definitive confirmation or rejection of the hypothesis that leukotriene antagonists are directly involved in the development of this syndrome require further investigations.

Churg-strauss syndrome

Churg–Strauss syndrome (CSS) is a rare granulomatous necrotizing small vessel vasculitis characterized by the presence of asthma, sinusitis, and hypereosinophilia. The cause of this allergic angiitis and granulomatosis is unknown. Other common manifestations are pulmonary infiltrates, skin, gastrointestinal, and cardiovascular involvement. No data have been reported regarding the role of immune complexes or cell mediated mechanisms in this disease, although autoimmunity is evident with the presence hypergammaglobulinemia, increased levels of IgE and Antineutrophil cytoplasmic antibody (positive in 40%). We report the case of a 27-year-old lady presenting with painful swelling of predominantly lower limbs with extensive vesicles and ecchymotic patches and fever shortly after stopping systemic steroids taken for a prolonged duration (2002--2010). The aim of this case report is to point to the possibility of CSS in patients presenting with extensive skin lesions masquerading as Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis Syndrome (SJS/TENS).

Churg-Strauss Syndrome in Patients Treated With Omalizumab

Churg-Strauss syndrome (CSS) is a rare systemic vasculitis associated with asthma, eosinophilia, sinusitis, and pulmonary infiltrates. CSS has been reported in association with asthma therapies. The objective is to describe the characteristics of CSS in patients treated with the anti-IgE antibody omalizumab (Xolair; Genentech Inc; South San Francisco, CA). A retrospective review of available data to identify cases of CSS was performed using the Novartis Argus global drug safety database for omalizumab in asthma patients. We identified 34 potential cases of CSS. Of these, 13 cases fulfilled at least four of the six criteria identified in the American College of Rheumatology classification criteria. Eight of the patients in these 13 definite or probable cases (62%) had CSS symptoms prior to receiving omalizumab or described symptom onset just after corticosteroid weaning. Six of the 13 patients (46%) were confirmed as having been treated with corticosteroids for what was perceived to be severe asthma; when corticosteroids were tapered in conjunction with omalizumab treatment, CSS symptoms appeared just after the tapering. There were 4 other cases of possible CSS, and the remaining 17 patients were judged to not have CSS. CSS may develop in patients receiving asthma medications who have an underlying eosinophilic disorder that is unmasked by the withdrawal of therapy with corticosteroids, or in patients who delay therapy in favor of other medications. Omalizumab treatment may unmask CSS due to the weaning of corticosteroids in some asthma patients or may delay corticosteroid treatment allowing for CSS to manifest. ClinicalTrials.gov Identifier: NCT00252135.

Fluticasone-Associated Cutaneous Allergic Granulomatous Vasculitis: An Underrecognized but Important Cause of Drug-Induced Cutaneous Churg-Strauss Syndrome

Allergic granulomatous vasculitis, or Churg-Strauss syndrome, is a small vessel, multisystem vasculitis that can affect multiple organs. It is usually idiopathic, but recent case reports have implicated leukotriene receptor antagonists (LTRA) and inhaled corticosteroids in the development of this rare syndrome. We report a case that acutely developed skin-limited Churg Strauss-like cutaneous allergic granulomatous vasculitis after initiating therapy with inhaled fluticasone and salmeterol for poorly controlled asthma symptoms. Our result thus highlights the importance of keeping the differential diagnosis of drug-induced Churg-Strauss syndrome in patients who have recently been prescribed inhaled steroids like fluticasone when they present with rashes of varying severity.

Churg–Strauss syndrome and leukotriene antagonist use: a respiratory perspective

Background:Churg–Strauss syndrome (CSS) is a rare granulomatous small vessel vasculitis that occurs against a background of longstanding asthma. Leukotriene antagonists (LTAs) are used in the management of asthma and may...

Index of Suspicion

Index of Suspicion

Responsabilité des antileucotriènes dans la survenue d'un syndrome de Churg et Strauss

Churg-Strauss syndrome is a systemic necrotizing vasculitis involving small and medium-sized vessels. Classic features include asthma and hypereosinophilia. Antineutrophil cytoplasm antibodies (ANCA) are detected in about 40% of patients. Churg-Strauss syndrome has been reported in patients receiving leukotriene modifiers for asthma, in particular, leukotriene receptor antagonists (LTRA) (montelukast, zafirlukast or pranlukast). Clinical manifestations cases do not differ in these cases from those in Churg-Strauss syndrome without antileukotriene exposure. It is increasingly less likely that LTRA is the direct cause of this syndrome in those patients, although this hypothesis has not been completely ruled out. In many patients, LTRA treatment is prescribed because of worsening asthma, which is an early sign of Churg-Strauss syndrome. LTRA for asthma patients should be prescribed with great care, especially in cases of atypical or rapidly aggravated asthma. The onset of Churg-Strauss syndrome in patients treated with LTRA usually requires that they stop this treatment. Prescription of LTRA In patients with Churg-Strauss syndrome should be discussed with specialists.

Churg‐strauss syndrome presenting with salivary gland enlargement and respiratory distress

The full expression of Churg-Strauss syndrome (CSS) is characterized by eosinophilia, a necrotizing vasculitis of small to medium blood vessels, and extravascular granuloma formation. The development of CSS typically occurs on a background of asthma, allergic rhinitis, or nasal polyps (1). A number of allergic, infectious, neoplastic, and idiopathic diseases may present with blood as well as tissue eosinophilia, sharing patterns of organ involvement and clinical features that are similar to those of CSS (2). Prompt distinction of CSS from these other entities is essential to the timely institution of appropriate treatment and the prevention of the potentially devastating complications of this disorder: congestive heart failure, severe gastrointestinal ischemia, central nervous system involvement, crippling vasculitic neuropathy (i.e., mononeuritis multiplex), glomerulonephritis, and others (3). In CSS, allergic manifestations and upper respiratory tract symptoms may occur anywhere frommonths to many years before the onset of systemic vasculitis. Empiric glucocorticoid treatment of the atopic features that characterize the disease’s early phase may suppress the emergence of vasculitis, at least temporarily. Conversely, the introduction of more effective inhaled agents for the management of asthma (e.g., the leukotriene inhibitors) is believed to unmask latent CSS in many patients by permitting reductions in the systemic glucocorticoids used to treat reactive airway disease (4). In the absence of pathologic evidence of destructive inflammation within blood vessel walls, distinguishing early CSS from other forms of eosinophilia is challenging. The pathologic diagnosis of CSS has traditionally been linked to the detection of necrotizing vasculitis accompanied by presence of eosinophilic granulomas (1). All of the patients described in 1951 by Churg and Strauss died from their conditions. Consequently, the clinical and pathologic features of the cases described in the original report of the disease were advanced. Moreover, complete postmortem examinations were available on all patients. In the current era, when awareness of CSS is higher, diagnostic testing is more facile, and the availability of effective therapy is widespread, the diagnosis of CSS is more likely to be suspected in the clinic than at postmortem. The authors describe a case of CSS in a patient with long-standing asthma and rhinitis who developed enlarged salivary glands, lymphadenopathy, pulmonary infiltrates, and respiratory distress. Identification of nonvasculitic eosinophilic tissue infiltrates within the salivary glands and skin in the context of the patient’s overall clinical picture led to the diagnosis of CSS at an early stage, the prompt institution of therapy, and a good outcome.

Churg-Strauss syndrome in the absence of asthma

Rationale Churg-Strauss Syndrome (CSS) consists of necrotizing vasculitis of small to medium sized arteries, necrotizing extravascular granulomas, and prominent tissue eosinophilia. CSS classically begins with a prolonged period of allergic rhinitis, nasal polyposis, and asthma. This is followed by the development of peripheral blood eosinophilia and eosinophilic tissue infiltrates, with culmination in a systemic vasculitic phase. Many published series investigating CSS exclude patients without asthma. Clinicians may overlook the diagnosis of CSS in patients who lack asthma. Methods We report the case of a 57-year-old male in whom CSS was diagnosed in the absence of asthma or bronchial hyperresponsiveness. Results The patient had a nine-month history of progressive lower extremity weakness and pain accompanied by a peripheral eosinophilia (absolute eosinophil count approximately 5500 cells per microliter). Past medical history was significant for interstitial lung disease of unknown etiology, with a restrictive pattern on pulmonary function testing. A methacholine challenge test was negative. Definitive diagnosis of CSS was made with muscle and nerve biopsies, which demonstrated necrotizing vasculitis of medium-sized arteries, predominately lymphocytic extravascular granulomas with few eosinophils, and eosinophil-rich endomysial inflammatory infiltrate with evidence of eosinophil degranulation. The combination of clinical and laboratory features in this case meets four of the six 1990 ACR diagnostic criteria for CSS, confirming the diagnosis with greater than 99% specificity. Conclusions Churg-Strauss Syndrome does occur in the absence of asthma. Clinicians should not exclude the possibility of this diagnosis in patients lacking asthma.

Leukotriene antagonists and the Churg-Strauss syndrome

Background and Objectives: Leukotriene antagonists (LTAs), or antileukotrienes, are a new group of anti-inflammatory drugs used for the treatment of asthma. They might substitute for or allow tapering of corticosteroids in asthmatic patients. These drugs have been associated with the development of Churg-Strauss syndrome (CSS), a rare form of vasculitic angiitis. It is unclear whether the development of CSS is a direct drug effect or an unmasking of a preexisting condition on withdrawal of steroids for asthma. We present a case of CSS in a patient treated with montelukast and review the literature to analyze the association between LTAs and the development of CSS. Method: We reviewed the literature using MEDLINE from February 1966 to October 2000. To the cases identified, we present an additional case of a patient who underwent a diagnostic lung biopsy. Results: Twenty-two case reports of patients receiving LTAs who developed CSS were identified. The onset of CSS occurred 2 days to 10 months after starting treatment with LTAs. All patients had received inhaled or oral steroids for asthma. The interval between the last oral corticosteroid treatment and CSS onset ranged from 3 days to 8 months. Conclusions: To date, there is no compelling evidence that the development of CSS in asthmatic patients receiving LTAs results from a direct drug effect. Rather, it appears that tapering of corticosteroids in these patients unmasks the multiorgan manifestations of the disease. We believe that the use of LTAs should not be influenced by the apparent increase in the incidence of CSS and that these are still safe drugs for asthma. Semin Arthritis Rheum 31:218-227. Copyright 2002, Elsevier Science (USA). All rights reserved.

Churg-Strauss Syndrome in Patients Receiving Montelukast as Treatment for Asthma

We previously reported eight patients who developed Churg-Strauss syndrome in association with zafirlukast treatment for asthma and postulated that the syndrome resulted from unmasking of a previously existing condition due to corticosteroid withdrawal and not from a direct drug effect. The availability of montelukast, a new leukotriene receptor antagonist with a different molecular structure, permitted us to test this hypothesis. Our goals were to ascertain whether the Churg-Strauss syndrome developed in patients taking montelukast and other novel asthma medications, and to describe potential mechanisms for the syndrome. Case series. Outpatient and hospital practices of pulmonologists in the United States and Belgium. Four adults (one man, three women) who received montelukast as treatment for asthma; two women who received salmeterol/fluticasone therapy, but not montelukast. Churg-Strauss syndrome developed in the four asthmatic patients who received montelukast. In each case, there was a long history of difficult-to-control asthma characterized by multiple exacerbations that had required frequent courses of oral systemic corticosteroids or high doses of inhaled corticosteroids for control. Two other asthmatics who received fluticasone and salmeterol but not montelukast therapy developed the same syndrome with tapering doses of oral or high doses of inhaled corticosteroids. The occurrence of Churg-Strauss syndrome in asthmatic patients receiving leukotriene modifiers appears to be related to unmasking of an underlying vasculitic syndrome that is initially clinically recognized as moderate to severe asthma and treated with corticosteroids. Montelukast does not appear to directly cause the syndrome in these patients.