Diagnosis Of Churg-Strauss Syndrome Research Articles

Diagnosis of Churg-Strauss Syndrome at 23 Weeks Gestation: a case report

Diagnosis of Churg-Strauss Syndrome at 23 Weeks Gestation: a case report

STEROID DEPENDENT ASTHMA, PERSISTENT PANSINUSITIS AND RECURRENT NASAL POLYPOSIS NON-RESPONSIVE TO THERAPY: WHAT IS NEXT?

Introduction A 15-year-old white male presented with recalcitrant asthma, chronic rhinosinusitis, and nasal polyps. Despite pharmacotherapy and sinus surgery he was unstable and steroid dependent. Case Description PMHx was unremarkable except for wheezing, nasal polyps, complete nasal blockage and CT-proven pansinusitis. A fumigatus antibodies, PPD and QFT Gold tests were negative. Total eosinophils were 3,700/mm3 and IgE was 1,788 IU/mL. The presence of asthma, eosinophilia, mononeuritis multiplex and paranasal sinus disease, suggested a diagnosis of Churg-Strauss Syndrome [Eosinophilic Granulomatosis Polyangiitis (EGPA)] a multi-systemic, clinically challenging condition because of therapy-associated adverse reactions. Benralizumab, was initiated at 30 mg SQ q 4 weeks X 3 doses then 30 mg q 8 weeks with significant clinical improvement and a dramatic drop in eosinophils to 0/mm3 on day 7. He is currently stabilized on benralizumab and has discontinued OCS. Discussion This case report clearly demonstrates that although the patient presented initially with severe asthma, EGPA was correctly diagnosed. The striking clinical improvement with benralizumab suggests its potential greater beneficial role than other anti-IL5 biologics which achieve eosinopenic effects by neutralizing circulating IL-5. Benralizumab targets IL-5Rα expressed by both mature eosinophils and eosinophil-lineage progenitor cells and accomplishes eosinophil depletion via enhanced antibody-dependent cellular toxicity (ADCC). This novel therapeutic agent merits further evaluation of clinical benefit as a promising therapeutic modality for the treatment of EGPA.

Churg-Strauss Syndrome in a Seven-year-old Boy: A Case Report

Churg-Strauss syndrome (CSS) is a type of vasculitis of small-to-medium sized vessels. This syndrome is known by a history of bronchial asthma with systemic necrotizing vasculitis and peripheral blood hypereosinophilia. It is currently called eosinophilic granulomatosis with polyangiitis (EGPA). This disease affects both genders and all age groups, but it is very rare among children. CSS diagnosis is based on clinical findings such as asthma, eosinophilia, rhinosinusitis, and signs of vasculitis in major organs. In cases where steroids alone or in combination with other immunosuppressive agents are used as treatment, the outcome and long-term survival are usually satisfying. In comparison with other types of systemic vasculitis, the mortality rate of this syndrome is low. In this study, we present the case of a 7-year-old boy with poorly controlled bronchial asthma since three years of age. This case had developed purpuric skin lesions, sinusitis, arthritis, and weakness of the limbs with symptoms of mononeuritis multiplex at the age of seven. After being admitted to our hospital, a series of studies, including complete blood count-diff, chest X-ray, paranasal sinus radiography, brain magnetic resonance imaging, nerve conduction study, spirometry, and serological tests for autoantibodies, were performed and he was diagnosed with CSS. Thereafter, he received regular corticosteroid therapy in combination with methotrexate, and his symptoms were generally well-controlled with the beginning of the treatment. The clinical characteristics, diagnosis, and management of CSS in children are also reviewed in this study.

Churg-Strauss syndrome with cardiac involvement: case illustration and contribution of CMR in the diagnosis and clinical follow-up

This report summarises three cases of Churg-Strauss syndrome (CSS) illustrating the diagnostic challenges associated with the cardiac manifestation of this disease. Here, we illustrate the role of cardiac magnetic resonance (CMR) for diagnosis and follow-up of CSS with a focus on new non-contrast T2-weighted imaging sequences for quantification of myocardial scar tissue and quantitative T2 mapping techniques, which allow the detection of myocardial edema.

Abstract WMP70: Yield of Screening Tests for Systemic Vasculitis in Ischemic Stroke in Young Adults

Background and Purpose: The causes of ischemic stroke in young adults are more heterogeneous than in the elderly. Sometimes ischemic stroke in young adults is complicated by a systemic vasculitis, which can involve the brain. Therefore, a panel for systemic vasculitis could be considered as one of the etiological work-up of ischemic stroke in young adults. However, little is known about the yield of screening tests for systemic vasculitis in young ischemic stroke patients. Methods: Consecutive patients aged 18 to 45 years with ischemic stroke between January 2011 and June 2017 were included. They all underwent screening tests for systemic vasculitis including rheumatoid factor (RF), antinuclear antibody (ANA), antineutrophil cytoplasmic antibodies (ANCAs), anticardiolipin antibody, lupus anticoagulant, antiphospholipid antibody, anti-DNA antibody, and anti-Ro/SSA and La/SSB antibodies. Results: Among consecutive 2,938 acute ischemic stroke patients during study period, 177 patients (6.0%) were aged 18-45 years. The mean age was 39.0 years. Vasculitis panel screening results were positive in three patients (1.7%). They all had other symptoms or signs of systemic vasculitis. Two female patients with positive ANA and anti-DNA antibody were finally diagnosed as systemic lupus erythematosus (SLE). One patient was compatible with the diagnosis of anti-phospholipid antibody syndrome, and the other patient’s stroke was thought to be related to SLE vasculitis. They all had clinical symptoms of SLE including malar rash and Raynaud phenomenon. One male patient showed positive pANCA. A year previously, he was diagnosed with asthma. Inflammation markers in peripheral blood exams were elevated (CRP 8.21 mg/dl, leukocyte count 17.7/nl), the rate of eosinophilic cells increased (42%). He fulfilled the criteria for the diagnosis of Churg-Strauss syndrome. Conclusions: In the absence of systemic symptoms or signs of vasculitis, vasculitis panel screening in young ischemic stroke patients has a low yield. It’s better to consider individual clinical features and cost effectiveness before screening tests for systemic vasculitis in ischemic stroke in young adults.

Churg-Strauss vasculitis in patient who received montelukas

Introduction. Montelukast is a selective leukotriene receptor antagonist. One of side effects of this drug class is the Churg-Strauss syndrome (CSS). There is still no reliable evidence whether the expression of this syndrome could be masked by high doses of corticosteroids and become expressed by termination of corticosteroid use, or whether it could be a consequence of leukotriene receptor antagonists use. Case report. Female patient, aged 49 years, was hospitalized with symptoms of fever, dyspnea, cough and increased sputum production with occasional hemoptysis. She was treated for asthma during the previous year. Leukocyte differential formula registered 44% of eosinophils. IgE value was extremely elevated, with value measured to 580 kU/L and eosinophile cation protein value was 15.1 ?g/L. Computed tomography of the chest described changes in the form of ground glass located in all lobes of the right lung and in the upper lobe of the left lung. Computed tomography of paranasal sinuses described changes that could resemble to polyposis, chronic sinusitis, and possible granulomatosis. Mononeuritis of peroneal nerve of the right leg was proven by electromyographic examination. Bone marrow biopsy indicated hypercellularity with domination of eosinophilic granulocytes (30%). Five out of six criteria were noted in patient?s clinical presentation, after which the diagnosis of CSS was set. The patient began treatment with high doses of corticosteroids while montelukast was discontinued which resulted in disease remission. Conclusion. Although there is no evidence that leukotriene modifiers cause the CSS in all patients with asthma, in case of frequent exacerbations with the appearance of pulmonary infiltrates, eosinophilia and paranasal sinus abnormalities make one think of this form of vasculitis.

Churg-Strauss syndrome: Report of 3 cases

Churg-Strauss syndrome (CSS) is a vasculitis of medium to small sized vessels. Diagnosis is mainly clinical with fi ndings of asthma, eosinophilia, rhinosinusitis and signs of vasculitis in major organs. This is a rare syndrome of unknown etiology, affecting both genders and all age groups. Outcome and long-term survival are usually good with steroids alone or in combination with immunosuppressive agents. The syndrome has a low mortality rate compared with other systemic vasculitides. We report three patients with CSS from Ardabil, who presented with asthma, pansinusitis, peripheral eosinophilia, peripheral neuropathy, cutaneous eosinophilic vasculitis. The diagnosis of CSS was made by the diagnostic criteria of American College of Rheumatology (ACR). CSS is typically suspected in patients whose asthma is poorly controlled on moderate doses of inhaled glucocorticoids. Prolonged treatment of asthma with glucocorticoid therapy may partially or totally suppress the usual clinical signs of untreated CSS. The disease may therefore not become evident until glucocorticoids are reduced or stopped.

Churg Strauss Syndrome with Migratory Polyarthritis

Churg Strauss Syndrome (CSS) is a rare systemic vasculitis associated with asthma, eosinophilia, sinusitis and pulmonary infiltrates. A 49 years old woman was admitted with migratory polyarthritis and Churg Strauss Syndrome. We will discuss the findings that lead to the diagnosis of CSS and we will raise the possibility that she might have a new syndrome that includes CSS with migratory polyarthritis. Until now there have no reports describing patients with CSS and arthritis. There have been some cases with arthrlagia, but our case represents the first case that was admitted with arthritis. This case is a novel case describing a new combination of migratory arthritis in a patient suffering from CSS.

Churg-Strauss Syndrome in Childhood

Churg-Strauss syndrome is a rare form of small-vessel vasculitis. In the current report, we describe the case of a 17-year-old Czech girl predominantly characterized by peripheral neuropathy, the presence of cardiac and pulmonary involvement, hypereosinophilia, asthma, and sinusitis that led to the diagnosis of Churg-Strauss syndrome.

Cardiac Involvement in ANCA (+) and ANCA (-) Churg-Strauss Syndrome Evaluated by Cardiovascular Magnetic Resonance

The cardiovascular magnetic resonance (CMR) pattern of Churg-Strauss syndrome (CSS) includes myopericarditis, diffuse subendocardial vasculitis or myocardial infarction with or without cardiac symptoms and is usually associated with lack of antineutrophil cytoplasmic antibodies (ANCA). To correlate the CMR pattern with ANCA in CSS, compare it with healthy controls and systemic lupus erythematosus (SLE) patients and re-evaluate 2 yrs after the first CMR. 28 consecutive CSS, aged 42±7 yrs, were referred for CMR and 2 yrs re-evaluation. The CMR included left ventricular ejection fraction (LVEF), T2-weighted (T2-W), early (EGE) and late gadolinium enhanced (LGE) imaging. Their results were compared with 28 systemic lupus erythematosus (SLE) under remission and 28 controls with normal myocardial perfusion, assessed by scintigraphy. CMR revealed acute cardiac lesions in all ANCA (-) CSS with active disease and acute cardiac symptoms and only in one asymptomatic ANCA (+) CSS, with active disease. Diffuse subendocardial fibrosis (DSF) or past myocarditis was identified in both ANCA(+) and ANCA (-) CSS, but with higher incidence and fibrosis amount in ANCA (-) CSS (p<0.05). In comparison to SLE, both ANCA (+) and ANCA (-) CSS had higher incidence of DSF, lower incidence of myocarditis and no evidence of myocardial infarction, due to coronary artery disease (p<0.05). In 2 yrs CMR follow up, 1/3 of CSS with DSF presented LV function deterioration and one died, although immunosuppressive treatment was given early after CSS diagnosis. Cardiac involvement either as DSF or myocarditis, can be detected in both ANCA (+) and ANCA (-) CSS, although more clinically overt in ANCA (-). DSF carries an ominous prognosis for LV function. CMR, due to its capability to detect disease severity, before cardiac dysfunction takes place, is an excellent tool for CSS risk stratification and treatment individualization.

Churg–Strauss syndrome presenting with eosinophilic myocarditis: A diagnostic challenge

Churg–Strauss syndrome (CSS) is an unusual disease that presents as systemic vasculitis and peripheral eosinophilia in patients with an atopic constitution. Cardiac involvement is unusual and often not prominent on initial presentation, but is an important cause of morbidity and mortality in patients with CSS. We report the case of a young woman with severe acute myocarditis. Coronary arteriography demonstrated extensive focal vasculopathy, consistent with coronary vasculitis, and myocardial biopsy showed eosinophilic myocarditis. This presentation led to an initial diagnosis of CSS in this patient and appropriate therapy resulted in a spectacular remission of disease activity.

Churg–Strauss syndrome presenting with eosinophilic myocarditis: A diagnostic challenge

Churg–Strauss syndrome (CSS) is an unusual disease that presents as systemic vasculitis and peripheral eosinophilia in patients with an atopic constitution. Cardiac involvement is unusual and often not prominent on initial presentation, but is an important cause of morbidity and mortality in patients with CSS. We report the case of a young woman with severe acute myocarditis. Coronary arteriography demonstrated extensive focal vasculopathy, consistent with coronary vasculitis, and myocardial biopsy showed eosinophilic myocarditis. This presentation led to an initial diagnosis of CSS in this patient and appropriate therapy resulted in a spectacular remission of disease activity.

THU0205 The temporal appearance of symptoms and signs in churg strauss syndrome: From the prodromal phase to damage

Objectivesto characterise the clinical presentation of Churg Strauss syndrome (CSS) in a monocentric cohort of patients and to assess the response to treatment and the long-term outcome of the disease.MethodsWe...

Unrecognized tamponade diagnosed pre-induction by focused echocardiography

We report a case of unrecognized cardiac tamponade diagnosed pre-induction by focused transthoracic echocardiography (TTE). The value of focused perioperative TTE, the anesthetic implications of Churg-Strauss syndrome, and the diagnosis of cardiac tamponade are discussed. A 58-yr-old man with a history of severe asymptomatic aortic stenosis presented for elective endoscopic sinus surgery for intractable nasal polyps with recurrent sinusitis. His cardiologist and cardiac surgeon had recommended proceeding with surgery, as aortic valve replacement was not indicated because he was asymptomatic. Prior to induction, a focused TTE was performed by anesthesia in order to document the degree of aortic stenosis, baseline ventricular function, and baseline volume status. This provided a baseline for comparison in case the patient's hemodynamic status should deteriorate intraoperatively. Unexpectedly, the TTE examination revealed cardiac tamponade. After confirmation of the diagnosis by cardiology, urgent pericardiocentesis was performed. A diagnosis of Churg-Strauss syndrome was ultimately made, and the patient was treated with high-dose prednisone therapy. Focused TTE has significant clinical utility for the diagnosis and assessment of hemodynamically significant cardiac conditions, particularly in the complex patient where clinical examination is challenging and echocardiographic findings can have immediate management implications.

Churg-Strauss syndrome presenting with focal segmental glomerulosclerosis without proteinuria: response to unconventional therapy

Churg-Strauss syndrome (CSS), or allergic granulomatosis, is a rare disease manifested by tissue infiltration, hypereosinophilia and vasculitis. Renal involvement may be seen in up to 50% of cases. We report the case of a 25-year old man who presented with a history of fever for two months, tingling, numbness, and paraesthesia of the upper limbs and left lower limb, along with diarrhoea for one month and an inability to walk for the past seven days. Serial laboratory investigations helped to reach the final diagnosis of CSS with mononeuritis multiplex, and skin, pulmonary and gastrointestinal involvement with hypertension. This is due to renal involvement in the form of focal segmental glomerulosclerosis without any nephrotic range proteinuria, which is a very rare clinical entity. The patient's symptoms were relieved after the administration of an unconventional mode of therapy.

The Churg-Strauss Syndrome: Diagnosed for the First Time in a Gastrectomy Specimen

The Churg-Strauss syndrome (CSS), first described in 1951 and characterised by eosinophilic inflammation and necrotising vasculitis in patients with asthma can in principle affect any organ. We report on a 47-year-old male patient, in whom the diagnosis of CSS was finally established for the first time on the basis of the histological work-up of a gastrectomy specimen. Endoscopic inspection had revealed a rigid gastric wall and a large irregularly shaped ulcer in the prepyloric antrum. Despite the fact that no carcinoma was demonstrable in the biopsy material, a gastrectomy was nevertheless performed since the endoscopic appearance was strongly suspicious for a carcinoma. The gastrectomy specimen revealed massive eosinophilic gastritis in combination with granulomas and necrotising vasculitis--localised mainly in the muscularis propria. In view of the subsequent information that the patient also suffered from asthma, the diagnosis of CSS (previously unrecognised) was established--for the first time--in the gastrectomy specimen.

Churg-Strauss Syndrome Revealed by Acute Coronary Artery Disease: A Case Report*

Churg-Strauss syndrome (CSS) is a very rare small-vessel vasculitis. Clinical features include asthma, rhinitis and/or sinusitis, and peripheral eosinophilia. Although cardiac findings are observed in 50% of cases, coronary artery disease is rarely reported and even more rarely suggestive. The value of cardiac MRI for these patients is discussed here. A 52-year-old non-smoker male without family antecedents of cardiovascular disease presented with worsening of atypical asthma that developed 10 months earlier. A month before, he had been admitted to the ICU because of respiratory distress and cardiogenic shock with chest pain. The angiogram revealed stenosis of the three main coronary arteries requiring the placement of several stents. Follow-up cardiac assessments showed good results. General impairment, unstable asthma associated with rhinitis, and eosinophilia suggested a systemic disease. The diagnosis of CSS was established considering that five criteria of the American College of Rheumatology were found. Prednisolone was prescribed at 1 mg/kg/day, which completely suppressed all symptoms and eosinophilia. Cardiac MRI was performed two months later and revealed a good control of myocardial lesions characterized by fibrosis beneath the anterior endocardium and the median septum. Immunosuppressive treatment was then administered together with corticosteroid therapy. These results suggest that acute coronary artery disease can reveal CSS in some cases. Here, the patient’s cardiac assessment was normal apart from the acute episode, and cardiac MRI helped detect signs of myocarditis and establish a prognosis of CSS.

A Case of Churg–Strauss Syndrome: Flow Cytometric Analysis of the Surface Activation Markers of Peripheral Eosinophils

© 2013 The Authors. doi: 10.2340/00015555-1384 Journal Compilation © 2013 Acta Dermato-Venereologica. ISSN 0001-5555 Churg–Strauss syndrome (CSS) is a complex multiple organ disease (1). The American College of Rheumatology has proposed 6 criteria for CSS classification, with the fulfilment of 4 criteria making a diagnosis of CSS (2). These criteria include asthma, eosinophilia, mononeuropathy or polyneuropathy, pulmonary infiltrates, paranasal sinus abnormality and extravascular eosinophils. Cutaneous manifestations are frequently observed in 40% of CSS patients. We report here a case of CSS presenting atypical cutaneous and subcutaneous manifestations of CSS with flow cytometric analysis of the peripheral eosinophils.

Effect of delayed diagnosis on disease course and management of Churg–Strauss syndrome: a retrospective study

Delayed diagnosis in patients with Churg-Strauss syndrome (CSS) is largely attributed to the variable and nonspecific presentation of the disease's initial symptoms. The aim of the study was to evaluate the effect of delayed diagnosis on the course of CSS. We conducted a retrospective study of 30 CSS patients followed up in our department. In each patient, we assessed the delay in CSS diagnosis (the time when patients already fulfilled four out of six of the American College of Rheumatology criteria and the diagnosis was not yet established), the disease activity at the time of diagnosis, and organ involvement during CSS course. A median value of 2 weeks was chosen as the cutoff point after which the diagnosis was considered as delayed. Sixteen patients were diagnosed before (group 1) and 14 patients after this cutoff point (group 2). In group 2, we found a higher Birmingham Vasculitis Activity Score at the moment of diagnosis (20.4 vs 25.1, p < 0.05) and a more severe disease course, resulting in more frequent hospitalization rates (0.64 vs 2.26/year, p < 0.00001), higher corticosteroids dose requirements (5.87 vs 11.57 mg/day converted to methylprednisolone, p < 0.0001), and additional immunosuppressive therapy administration (56.2 vs 92.8 %, p < 0.05) to maintain disease remission. All six perinuclear pattern of antineutrophil cytoplasmic antibobodies (pANCA)-positive patients (20 %) were found in group 1. Concluding, the delay in diagnosis of CSS of more than 2 weeks was found to be associated with a disease course that was more severe. The presence of the pANCA antibodies may occasionally facilitate establishment of the diagnosis.

Occam’s razor reveals a hidden Churg-Strauss syndrome

Abstract A 28 year-old caucasian lady, with nine years of uncontrolled bronchial asthma, rhinosinusitis and mild upper limb paresthesia, came to our attention to be followed for coeliac disease (CD). She had a biopsy performed elsewhere which proved the diagnosis five years before. Since there was no clinical improvement on a strict gluten-free diet, we re-evaluated the slides of her duodenal biopsies and we found an overestimation of the duodenal lesions due to the wrong orientation of the specimens. Moreover, she had never had positive CD-related antibodies and she was negative for DQ2/DQ8 MHC Class II heterodimers. Months later, she referred she was suffering from diffuse joint pain, epistaxis and a substantial weight loss. A few days later she was hospitalized because of a sudden onset of dyspnea, peripheral edema and pleural effusion. Her echocardiogram showed global left ventricular hypokenesia with an ejection fraction of 24%. The patient was discharged with a diagnosis of dilated cardiomyopathy and NYHA Class II. After a large spectrum of haematological exams, the diagnosis of Churg Strauss Syndrome (CSS), a rare multisystemic small-vessel necrotizing vasculitis, was confirmed by the presence of four/five out of six diagnostic American College of Rheumatology classification criteria (Asthma, Eosinophilia &gt;10%, Neuropathy, Non-fixed pulmonary infiltrates, Paranasal sinus abnormality and Biopsy containing a blood vessel with extravascular eosinophils). Our patient had been under-diagnosed by pulmonologist and by gastroenterologists although she presented the criteria required for CSS diagnosis. Our case report emphasizes that often seemingly unrelated symptoms can be caused by a single rare clinical complex.