J Cardiovasc Med (Hagerstown). 2007 Dec;8(12):1076-9.
Myocardial late gadolinium enhancement in specific cardiomyopathies by cardiovascular magnetic resonance: a preliminary experience.
Silva C, Moon JC, Elkington AG, John AS, Mohiaddin RH, Pennell DJ.
Centre for Advanced Magnetic Resonance in Cardiology (CAMRIC), Royal Brompton Hospital, London SW3 6NP, UK. caterina.silva@unipd.it
Late gadolinium enhancement cardiovascular magnetic resonance (CMR) can visualize myocardial interstitial abnormalities. The aim of this study was to assess whether regions of abnormal myocardium can also be visualized by late enhancement gadolinium CMR in the specific cardiomyopathies. A retrospective review of all referrals for gadolinium CMR with specific cardiomyopathy over 20 months. Nine patients with different specific cardiomyopathies were identified. Late enhancement was demonstrated in all patients, with a mean signal intensity of 390 +/- 220% compared with normal regions. The distribution pattern of late enhancement was unlike the subendocardial late enhancement related to coronary territories found in myocardial infarction. The affected areas included papillary muscles (sarcoid), the mid-myocardium (Anderson-Fabry disease, glycogen storage disease, myocarditis, Becker muscular dystrophy) and the global sub-endocardium (systemic sclerosis, Loeffler's endocarditis, amyloid, Churg-Strauss). Focal myocardial late gadolinium enhancement is found in the specific cardiomyopathies, and the pattern is distinct from that seen in infarction. Further systematic studies are warranted to assess whether the pattern and extent of late enhancement may aid diagnosis and prognostic assessment.
Vasc Health Risk Manag. 2007;3(5):775-9.
Acute coronary syndrome associated with Churg-Strauss syndrome.
Wagner AD, Meyer GP, Rihl M, Rathmann A, Wittkop U, Zeidler H, Haller H, Lotz J.
Department Internal Medicine, Division of Nephrology, Medizinische Hochschule Hannover, Germany. A.D.Wagner@gmx.net
A 41 -year old female patient was admitted with acute onset of dyspnea and chest pain. Previous history revealed asthma, chronic sinusitis and eosinophilic proctitis. Electrocardiogram showed anterior ST-segment elevations and inferior ST-segment depression. Immediate heart catheterization revealed a distally occluded left anterior descending coronary artery, the occlusion being reversible after nitroglycerine. Cardiac magnetic resonance imaging was consistent with perimyocarditis. Hypereosinophilia and IgE elevation were present and Churg-strauss syndrome was diagnosed.
Presse Med. 2007 May;36(5 Pt 2):875-89. Epub 2007 Apr 3.
Churg-Strauss syndrome
Lhote F.
Service de médecine interne, Centre hospitalier, Saint-Denis, France. francois.lhote@ch-stdenis.fr
Churg-Strauss syndrome is a systemic and pulmonary vasculitis, defined by its association with severe asthma and with hypereosinophilia of the blood and tissues. The systemic vasculitis is a small-vessel vasculitis frequently associated with purpura, mononeuritis multiplex, and, more rarely, with rapidly progressive glomerulonephritis or diffuse alveolar hemorrhage. Its prevalence of 7 to 13 per million population makes it one of the rarest of the systemic vasculitides. Anti-MPO (antimyeloperoxidase) pANCA (ANCA with a perinuclear fluorescence pattern) is present in 35-40% of cases and appears to determine a subgroup of patients with a higher frequency of renal damage, alveolar hemorrhage, and central nervous system damage. Cardiac involvement is an important cause of morbidity and the leading cause of mortality in Churg-Strauss syndrome. Treatment is based on corticosteroid therapy and immunosuppressive drugs (cyclophosphamide and azathioprine) and is determined according to validated prognostic criteria (Five-Factor Score). Complete remission occurs in almost 90% of cases, and the 10-year survival rate has reached 79.4%. Relapses are frequent (25% of cases) and even after recovery from vasculitis, most patients (90%) still have asthma requiring corticosteroid treatment.
Heart Vessels. 2007 Mar;22(2):128-30. Epub 2007 Mar 23.
Churg-Strauss syndrome presenting with massive pericardial effusion.
Matsuo S, Sato Y, Matsumoto T, Naiki N, Horie M.
Department of Cardiovascular and Respiratory Medicine, Shiga University of Medical Science, Seta Otsu, Shiga 520-2192, Japan. smatsuo@belle.shiga-med.ac.jp
We describe a case of Churg-Strauss syndrome (CSS) presenting with a massive pericardial effusion without overt myocardial dysfunction. A 60-year-old man was referred to our hospital because of exertional dyspnea and fever. Initial chest multidetector-row computed tomography showed a massive pericardial effusion. The presence of eosinophilia, infiltrates of both lungs, pathological evidence of necrotizing vasculitis associated with eosinophilic infiltration, and history of asthma fulfilled the criteria of CSS. Massive pericardial effusion can be the first manifestation of cardiac involvement in CSS.
Semin Arthritis Rheum. 2007 Jun;36(6):386-91. Epub 2007 Feb 14.
Churg-Strauss syndrome revealed by granulomatous acute pericarditis: two case reports and a review of the literature.
Agard C, Rendu E, Leguern V, Ponge T, Masseau A, Barrier JH, Trochu JN, Hamidou MA, Guillevin L.
Internal Medicine, Hôtel-Dieu Hospital, CHU Nantes, Place Alexis Ricordeau, 44093 Nantes Cedex 01, France. christian.agard@chu-nantes.fr
BACKGROUND: Churg-Strauss syndrome (CSS) is a necrotizing systemic vasculitis with extravascular granulomas and eosinophilic infiltrates of small vessels. CSS is usually revealed by nonspecific signs of necrotizing vasculitis in a context of late-onset asthma and blood eosinophilia. It is considered a systemic vasculitis with the highest prevalence of cardiac involvement and can lead to rapid-onset heart failure due to specific cardiomyopathy. Pericardial effusion may also occur during CSS and is usually well tolerated. OBJECTIVE: The objective of these case reports was to indicate that CSS may present as tamponade, with or without other visceral involvement. METHODS: Among CSS patients treated during the past 10 years at 2 French university hospitals, we have identified and described 2 cases revealed by tamponade with pericardial biopsy-proven granulomatous vasculitis. We have also reviewed the international medical literature in PubMed on cardiac involvement in CSS. RESULTS: The first case report describes a 66-year-old man who had an isolated cardiac tamponade with both inflammatory syndrome and eosinophilia. Long-term remission was obtained with corticosteroids. The second case report describes a 46-year-old woman whose CSS presented with tamponade and associated central nervous system and myocardial involvement. Remission was obtained with corticosteroids and cyclophosphamide. In both cases, CSS was assessed by histological analysis of a pericardial sample. CONCLUSIONS: CSS may present as isolated cardiac tamponade. Whereas pericarditis with myocardial injury warrants immunosuppressive therapy, isolated pericarditis without other visceral involvement of poor prognosis only requires corticosteroid therapy.
Clin Res Cardiol. 2006 May;95(5):289-94. Epub 2006 Feb 27.
Rapid progressive eosinophilic cardiomyopathy in a patient with Churg-Strauss syndrome (CSS).
Rosenberg M, Lorenz HM, Gassler N, Katus HA, Frey N.
Department of Cardiology, Internal Medicine III, University of Heidelberg, INF 410, 69120, Heidelberg, Germany.
Churg-Strauss syndrome (CSS) is a rare necrotizing, systemic vasculitis that is almost invariably associated with bronchial asthma. Although overall prognosis is good and treatment with corticosteroids alone or in combination with other immunosuppressive agents is typically successful, there are reports of patients that do not show signs of clinical improvement under the usual pharmacotherapy. Small clinical studies suggested that cardiac or gastrointestinal involvement is associated with an adverse prognosis.We here report the case of a 38 year old male patient with a history of bronchial asthma who was admitted to our hospital for further evaluation of progressive dyspnea. Blood eosinophilia, infiltrates of both lungs, signs of necrosis and eosinophil deposits on myocardial biopsy combined with a history of bronchial asthma established the diagnosis of CSS with cardiac involvement. We initiated an immunosuppressive therapy with prednisone and methotrexate. Upon tapering of the dosage of prednisone, we noticed worsening of symptoms and further deterioration of cardiac function. Despite the addition of cyclophosphamide and adjustment of heart failure medication, we were not able to stabilize the cardiac situation. Due to rapid progressive eosinophilic cardiomyopathy associated with CSS refractory to medical therapy, our patient was placed on the urgent heart transplantation waiting list and, in the meantime, has undergone successful cardiac transplantation.
Am J Cardiol. 2006 May 15;97(10):1519-24. Epub 2006 Mar 31.
Cardiac involvement in the Churg-Strauss syndrome.
Pelà G, Tirabassi G, Pattoneri P, Pavone L, Garini G, Bruschi G.
Department of Internal Medicine, Nephrology and Prevention Sciences, University of Parma, Parma, Italy. giovanna.pela@unipr.it
Churg-Strauss syndrome (CSS) is a rare systemic disease characterized by necrotizing vasculitis and peripheral eosinophilia. Cardiac involvement is considered common and is given a high rank among the causes of morbidity and mortality. The aim of this study was an update on the cardiac manifestations of this syndrome using a noninvasive approach. Sixteen patients with CSS were compared with a gender- and age-matched group of 20 healthy subjects. All patients but 1 were receiving treatment (steroids and/or immunosuppressive drugs). According to the Birmingham vasculitis activity score, 12 patients were in an active phase, and 4 were in drug-induced remission. All subjects underwent M-B-mode echocardiography and Doppler tissue echocardiography. Heart failure, life-threatening arrhythmias, and other prominent manifestations of heart disease were not observed. No differences were found in left ventricular diameter, volume, mass, or ejection fraction. The 2 groups did not differ in right ventricular diameter and pulmonary pressure. Few and nonspecific changes were detected by 2-dimensional echocardiography, including subclinical pericardial effusion and mitral regurgitation, in fewer than half the subjects. Subjects with CSS showed an impairment of ventricular relaxation. Changes were more prominent in the right ventricle. The peak velocity (PV) of early diastolic tricuspid inflow (E) was about 8% less than in controls, and the velocity of late diastolic inflow (A) was 35% greater. The E/A(PV) ratio was, on average, 33% less. In the left ventricle, E(PV) was 11% less and A(PV) 11% greater. The E/A ratio was decreased by 22%. Doppler analysis of tissue kinetics confirmed these indications. In the right ventricle, E(PV) was decreased by 10% and A(PV) was increased by 20% in the patient group. The E/A(PV) ratio was decreased by 29%. In the left ventricle, in which different sites were sampled, the average changes were -15%, +1%, and -23%, respectively. In the left ventricle, the velocity of systolic contraction was also decreased by 12%. Because of the small group size, only some of these differences were statistically significant. In conclusion, these moderate changes, devoid of clinical correlates, contrast with early reports emphasizing cardiac morbidity and poor prognosis in this syndrome.
Rev Med Suisse. 2006 Apr 19;2(62):1048-51.
Diagnosis and follow-up of vasculitis: usefulness of imaging
Vanini G, Albrecht S, Chizzolini C.
Service d'immunologie et d'allergologie, Département de médecine interne, HUG, 1211 Gèneve 14. Gianluca.Vanini@hcuge.ch
Vasculitides are due to inflammation of the vessel wall. There is a definite advantage in visualizing the inflammatory process within blood vessels without resorting to invasive procedures. A variety of non-invasive imaging techniques is now becoming available to investigate patients with vasculitis. These include ultrasonography, MRI coupled to angiographic sequences, PET, single photon emission computed tomography (SPECT). Their role is being evaluated and their characteristics exploited to address issues specific to each vasculitis. Thus, cardiac IRM should be considered in patients with ANCA-negative Churg-Strauss syndrome. Moreover, PET could be useful from investigate a subgroup of patients suffering from giant-cell arteritis. However, to validate
Int J Cardiol. 2006 Mar 22;108(1):112-3.
Endomyocardial fibrosis in Churg-Strauss syndrome assessed by cardiac magnetic resonance imaging.
Alter P, Maisch B.
Cardiac involvement frequently occurs in hypereosinophilic syndrome. The endocardium seems to be most susceptible to disturbances deriving from eosinophilia as seen in Loeffler's endocarditis. Hypereosinophilia and cardiac involvement are also seen in Churg-Strauss syndrome. The present report deals with a patient with Churg-Strauss syndrome who exhibited marked endomyocardial fibrosis that was visualized by cardiac magnetic resonance imaging. Loeffler's endocarditis is the classical endocardial manifestation of hypereosinophilic syndrome. Similarities between Loeffler and Churg-Strauss syndromes had been described previously. Potentially, endocardial involvement is more common even in Churg-Strauss syndrome than expected so far. The significance of these findings remains to be shown.
Rev Neurol (Paris). 2006 Feb;162(2):229-32.
The association of cardiac involvement and ischemic stroke in Churg Strauss syndrome
Sonneville R, Lagrange M, Guidoux C, Michel M, Khellaf M, Russel S, Hosseini H.
Service de Neurologie, CHU Henri Mondor, Créteil, France.
In Churg and Strauss syndrome (CSS), three patterns of neurological involvement can be found, including mono or polyneuropathy, encephalopathy and stroke. We report two cases of stroke associated with major hypereosinophilia and cardiac involvement, leading to a diagnosis of CSS. Neurological and general outcome were good under treatment with steroids in combination with cyclophosphamide in one case. Churg and Strauss syndrome must be considered when a stroke is associated with a cardiac involvement and hypereosinophilia.
Chest. 2005 Aug;128(2):1047-50.
Erratum in:
Chest. 2005 Nov;128(5):3779.
A case of sudden cardiac death due to isolated eosinophilic coronary arteritis.
Lepper PM, Koenig W, Möller P, Perner S.
Department of Internal Medicine II, University of Ulm, Robert-Koch-Str 8, 89081 Ulm, Germany. philipp.lepper@medizin.uni-ulm.de
Spontaneous coronary artery dissection is a very rare event and occurs most often in young women following childbirth. It is also known as a rare focal complication in Churg-Strauss syndrome. Here, we present the case of a 43-year-old woman who died after spontaneous dissection of all three coronary arteries. The microscopic examination of coronary vessels showed severe eosinophilic infiltrations, whereas all extracardiac (medium-vessel and large-vessel) arteries were intact and free of inflammatory cells. Her history did not reveal allergy, asthma, or eosinophilia. To the best of our knowledge, this is the first case of spontaneous coronary dissection involving all coronary arteries without a history of Churg-Strauss syndrome or hypereosinophilic syndrome.
J Cardiovasc Pharmacol Ther. 2005 Jun;10(2):131-6.
Elevated troponins and the Churg-Strauss syndrome: a case report.
Zaky J, Caraang C, Yu R, El-Bialy A.
Department of Medicine, Sylmar, California 91342-1495, USA.
In a patient with persistently elevated troponin levels but normal ischemic work-up, a diagnostic dilemma can ensue. This is the case of a 65-year-old woman whose only cardiac risk factor was age. She presented repeatedly with chest pain, elevated troponins, and consistently elevated eosinophil levels until the fourth admission when she presented with multi-organ abnormalities including asthma and vasculitis that led to the diagnosis of Churg-Strauss syndrome (CSS). Initiation of corticosteroids immediately resolved all of her presenting symptoms; troponin and eosinophil levels quickly normalized. Eosinophilia from CSS can lead to multi-organ damage including the heart. Therefore, one must consider CSS in the differential of eosinophilia as early detection and treatment may be critical in decreasing morbidity and mortality.
Semin Respir Crit Care Med. 2004 Oct;25(5):535-45.
Churg-strauss syndrome.
Guillevin L, Pagnoux C, Mouthon L.
Department of Internal Medicine, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris V, Paris, France. loic.guillevin@cch.ap-hop-paris.fr
First described in 1951 as an allergic and granulomatous angiitis, Churg-Strauss syndrome (CSS) is a small-vessel vasculitis. Mean age at the time of diagnosis is approximately 50 years, with a sex ratio around 1. Asthma is the central feature of CSS and precedes the systemic manifestations in almost all cases, whereas 70% of the patients have maxillary sinusitis, allergic rhinitis, and/or sinus polyposis. General symptoms are frequent, and associated with pulmonary infiltrates in 38 to 77% of the patients; peripheral neuropathy, usually mononeuritis multiplex, in 64 to 75%; skin involvement in 40 to 70%; and gastrointestinal tract symptoms in 37 to 62%. Cardiac involvement is common, with pericarditis in 23% of the patients and myocarditis in 13%, and represents the primary cause of mortality. Hypereosinophilia is the main biological feature of CSS, whereas antineutrophil cytoplasm antibodies (ANCA), especially anti-myeloperoxidase (MPO), are found in one third to one half of the patients. Triggering factors, such as vaccination, desensitization, or exposure to leukotriene-receptor antagonists, have been suspected as contributing to the development of CSS, but its etiology has not yet been fully elucidated. T-helper type 2 (Th2) lymphocytes, by analogy with the pathogenesis of asthma, eosinophils infiltrating tissues, and anti-MPO ANCA are probably implicated in the pathogenesis of vasculitic lesions. CSS usually responds rapidly to corticosteroids. Adjunction of cyclophosphamide is indicated when at least one factor of poor prognosis is present. With treatment, remission is obtained in more than 80% of the patients, but it is often impossible to withdraw corticosteroids completely because of residual asthma. Relapses occur in 25% of the patients, half during the first year. The 10-year survival rate was 79% for our patients, with 73% of them requiring low-dose prednisone maintenance therapy for persistent asthma.
Dtsch Med Wochenschr. 2004 Oct 8;129(41):2173-6.
Comment in:
Dtsch Med Wochenschr. 2005 Jan 28;130(4):176; author reply 176.
Complicated course of Churg-Strauss syndrome with eosinophilic perimyocarditis and pericardial tamponade
Lamparter S, Pankuweit S, Kölsch S, Maisch B.
Abteilung für Innere Medizin, Diakonie-Krankenhaus Wehrda, Marburg. CS.Lamparter@t-online.de
HISTORY: A 30-year-old patient suffered from a dry cough and increasing dyspnea since two years; he further complained about non radiating chest pain and weight loss of 15 kg in the past 8 weeks. EXAMINATIONS: Physical examination revealed pulsus paradoxus and distended neck veins. On chest x-ray, signs of cardiomegaly without infiltrations were found. Echocardiographic studies demonstrated a large pericardial effusion with signs of pericardial tamponade. Pericardiocentesis and pericardioscopy was performed and pericardial as well as epimyocardial biopsy samples were taken. Serum studies revealed increased markers of myocardial infarction and hypereosinophilia without clinical evidence of parasitic, myeloproliferative, or neoplastic diseases. Diagnosis of acute eosinophilic myocarditis was established in the epimyocardial biopsy samples. DIAGNOSIS, TREATMENT AND COURSE: Based on the clinicopathologic findings, we diagnosed Churg Strauss syndrome with cardiac involvement. We instilled 500 mg triamcinolone intrapericardially and initiated systemic treatment with corticosteroids which resulted in normalization of the blood eosinophil count. During a follow up of 18 months, no recurrence of pericardial effusion was detected. However, while trying to reduce the steroids below 15 mg prednisolone equivalent per day, eosinophil numbers raised and wheezing increased. We suggested an immunosuppressive therapy including cyclophosphamide according to the Fauci protocol, which was denied by the patient due to potential adverse side effects. CONCLUSION: We suggest a detailed invasive strategy including endomyocardial biopsy to rule out viral myocarditis before immunosuppressive therapy with steroids is initiated in patients with suspected cardiac involvement in Churg Strauss syndrome.
Circ J. 2004 Jun;68(6):595-8.
Assessment of myocardial perfusion and fatty acid metabolism in a patient with Churg-Strauss syndrome associated with eosinophilic heart disease.
Shikama N, Nakagawa T, Takiguchi Y, Aotsuka N, Kuwabara Y, Komiyama N, Terano T, Hirai A.
Department of Internal Medicine, Chiba Municipal Hospital, Japan. ichi-hs@atlas.plala.or.jp
Churg-Strauss syndrome is characterized by asthma, eosinophilia and systemic necrotizing vasculitis; cardiac involvement (ie, eosinophilic heart disease) is the major cause of morbidity and mortality, although there are no reports of an association between left ventricular dysfunction because of eosinophilic heart disease and myocardial blood flow or myocardial fatty acid metabolism. A patient presented with Churg-Strauss syndrome associated with eosinophilic heart disease that had progressed to dilated cardiomyopathy. Coronary angiography, thallium-201 ((201)Tl) and iodine-123 beta-methyl-iodophenyl pentadecanoic acid ((123)I BMIPP) myocardial single photon emission computed tomography (SPECT) were performed to evaluate left ventricular dysfunction. Although coronary angiography was normal and (201)Tl SPECT showed no apparent image defect, (123)I BMIPP SPECT showed diffuse decreased accumulation, excepting the apex. The left ventricular dysfunction in patients with eosinophilic heart disease is associated with impaired myocardial fatty acid metabolism rather than with impaired myocardial blood flow.
Can J Cardiol. 2003 Sep;19(10):1184-8.
Churg-Strauss syndrome with myocarditis manifesting as acute myocardial infarction with cardiogenic shock: case report and review of the literature.
Shanks M, Ignaszewski AP, Chan SY, Allard MF.
miriamshanks@yahoo.com
A patient with a two-year history of worsening asthma presented with chest pain and shortness of breath. She developed cardiogenic shock. Analysis of blood chemistry detected increased troponin I concentration. Her electrocardiographic changes were consistent with a diagnosis of anteroseptal myocardial infarction. However, angiography showed normal coronary arteries. Left ventriculography showed severe mitral regurgitation and global hypokinesis. Peripheral eosinophilia was detected. Subsequent endomyocardial biopsy showed myocarditis with prominent eosinophil and plasma cell components. Churg-Strauss syndrome was diagnosed based on her history of asthma, evidence of peripheral eosinophilia and results of endomycardial biopsy. Treatment with a high dose of corticosteroids was initiated. As symptoms of heart failure improved - without recurrence of cardiac and respiratory symptoms - the dose of corticosteroids was gradually reduced. Eight months after her original presentation, she developed urticarial lesions on her abdomen and legs, with muscle soreness but no other associated symptoms. She was treated with a combination of prednisone and dapsone. After the diagnosis of Churg-Strauss syndrome, the patient remained symptom free with a normal ejection fraction for 15 months while taking prednisone.
Z Kardiol. 2003 Aug;92(8):677-81.
Left ventricular dysfunction in Churg-Strauss syndrome
Heger M, Bergler-Klein J, Zehetgruber M, Parschalk B, Thalhammer F, Maurer G, Binder T.
Universitätsklinik für Innere Medizin II, Abteilung für Kardiologie, Wien, Austria. maria.heger@univie.ac.at
Churg-Strauss syndrome is a rare disorder characterized by hypereosinophilia and a systemic vasculitis occurring inpatients with asthma and allergic rhinitis. Vasculitis commonly affects the lungs, the heart, the skin, and the peripheral nervous system. Cardiac involvement is characterized by acute and constrictive pericarditis, myocarditis and endocarditis, as well as ischemic cardiomyopathy. Endomyocardial fibrosis similar to Loeffler's syndrome has been rarely described. In the presented case, a 43 year old man with a history of allergy and asthma suffered from increasing dyspnea, fever, pulmonary infiltates and cardiomyopathy. Laboratory studies were notable for marked hypereosinophilia. In a bronchoscopic lavage and transbronchial biopsy eosinophilic infiltrates accompanied by vasculitis were found, Churg-Strauss syndrome was diagnosed. Echocardiogram showed endomyocardial deposits in the apex of the right ventricle, right ventricular function was normal particular in the basal segments. The left ventricle was slightly enlarged and left ventricular function was impaired. The diastolic mitral in-flow showed a restrictive pattern. Additionally, a pericardial effusion was observed without signs of tamponade. The patient received corticosteroids, cyclophosphamide and cardiomyopathy-specific therapy and showed a marked improvement after 4 months.
Cardiovasc Pathol. 2003 Mar-Apr;12(2):94-7.
Churg-Strauss syndrome and sudden cardiac death.
Val-Bernal JF, Mayorga M, García-Alberdi E, Pozueta JA.
Department of Anatomical Pathology, Marqués de Valdecilla University Hospital, Medical Faculty, University of Cantabria, Santander, Spain.
apavbj@humv.es
Churg-Strauss syndrome is a rare disorder characterized by necrotizing vasculitis, granulomas with eosinophilic necrosis, and tissue infiltration by eosinophils. Sudden cardiac death is rarely described in Churg-Strauss syndrome. In this article, we describe a case of Churg-Strauss syndrome with multiorgan involvement manifested as sudden cardiac death. To the best of our knowledge, this form of presentation has not been reported. A 49-year-old woman was found dead in her room. No premonitory complaints had been noted during the days preceding her death. Past medical history did not reveal any relevant illness. At autopsy, multiorganic Churg-Strauss syndrome with prominent cardiac involvement was found. Therefore, this syndrome in the active vasculitic phase may be asymptomatic and may involve predominantly the heart. This variant of the syndrome may be fulminant and present as sudden cardiac death. This form can only be elucidated by autopsy study. Copyright 2003 Elsevier Inc.
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