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The Churg-Strauss Syndrome

1995; Elsevier BV; Volume: 107; Issue: 2 Linguagem: Inglês

10.1378/chest.107.2.578

1931-3543

Mark Kozak, Edward A. Gill, Larry S. Green,

Urticaria and Related Conditions

The Churg-Strauss syndrome (CSS) is an unusual disease that presents as a systemic vasculitis and peripheral eosinophilia in a patient with chronic atopic disease. Although often not prominent on initial presentation, cardiac involvement is a major cause of morbidity and mortality in patients with CSS. We report a case of a young woman with CSS who had a myocardial infarction. Coronary arteriography was performed for recurrent chest pain and demonstrated diffuse vasculopathy consistent with vasculitis in CSS. We have also included a review of the literature on cardiac involvement in CSS. The Churg-Strauss syndrome (CSS) is an unusual disease that presents as a systemic vasculitis and peripheral eosinophilia in a patient with chronic atopic disease. Although often not prominent on initial presentation, cardiac involvement is a major cause of morbidity and mortality in patients with CSS. We report a case of a young woman with CSS who had a myocardial infarction. Coronary arteriography was performed for recurrent chest pain and demonstrated diffuse vasculopathy consistent with vasculitis in CSS. We have also included a review of the literature on cardiac involvement in CSS. =Churg-Strauss syndrome The Churg-Strauss syndrome (CSS), also known as allergic granulomatous angitis, is a multisystem disease of unknown etiology. The 1990 American College of Rheumatology Criteria for CSS require that four of the following six criteria be met: (1) asthma (2) eosinophilia (>10% of leukocytes by differential cell count); (3) mononeuropathy or polyneuropathy; (4) migratory or transient pulmonary infiltrates; (5) paranasal sinus abnormality; and (6) extravascular eosinophils.1Masi AT Hunder GG Lie JT Michel BA Bloch DA Arend WP et al.The American College of Rheumatology 1990 Criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis).Arthritis Rheum. 1990; 33: 1094-1100Crossref PubMed Scopus (1778) Google Scholar While it is believed to be a rare disorder, recent reports of “limited forms” of the disorder2Lie JT Bayardo RJ Isolated eosinophilic coronary arteritis and eosinophilic myocarditis: a limited form of Churg-Strauss syndrome.Arch Pathol Lab Med. 1989; 113: 199-201PubMed Google Scholar, 3Nissim F Von der Valde J Czernobilsky B A limited form of Churg-Strauss syndrome: ocular and cutaneous manifestations.Arch Pathol Lab Med. 1982; 106: 305-307PubMed Google Scholar and the general difficulties in the diagnosis and classification of the vasculitides suggest that this entity may be underdiagnosed. Recognition of this syndrome is important because it responds well to proper therapy while the outcome is poor in untreated patients.4Lanham JG Elkon KB Pusey CD Hughes GR Systemic vasculitis with asthma and eosinophila: a clinical approach to the Churg-Strauss syndrome.Medicine. 1984; 63: 65-81Crossref PubMed Scopus (1040) Google Scholar, 5Chumbley LC Harrison EG DeRemee RA Allergic granulomatosis and angiitis (Churg-Strauss syndrome): report and analysis of 30 cases.Mayo Clin Proc. 1977; 52: 477-484PubMed Google Scholar, 6Guillevin L Du LTH Godeau P Jais P Wechsler B Clinical findings and prognosis of polyarteritis nodosa and Churg-Strauss angiitis: a study in 165 patients.Br J Rheumatol. 1988; 27: 258-264Crossref PubMed Scopus (246) Google Scholar, 7Churg J Strauss L Allergic granulomatosis, allergic angiitis, and periarteritis nodosa.Am J Pathol. 1951; 27: 277-301PubMed Google Scholar Also, involvement of one organ system may dominate the initial presentation, thus obscuring the diagnosis. Cardiovascular and pulmonary physicians in particular need to be aware of this entity because a large number of deaths in this disease are related to cardiac causes (vida infra). We report the case of a young woman with CSS who had a myocardial infarction. Coronary angiography was performed for recurrent chest pain and supported the diagnosis of coronary vasculitis. The patient is a 32-year-old woman who was previously healthy except for a 2-year history of asthma. The patient developed radiographically documented sinusitis 3 months before admission to our hospital. She was treated with antibiotics, during which time she developed bloody diarrhea. A colonic biopsy specimen showed a nonspecific vasculitis. The patient then developed weakness of all four extremities and became bedridden over a period of 3 to 4 weeks. Electromyography and nerve conduction studies showed sensorimotor axonal polyneuropathy. Laboratory studies were notable for a mild leukocytosis with 57% eosinophils. There was no history of tryptophan ingestion. After a 3-week stay at a rehabilitation center during which time she had no improvement in her neuromuscular status, she was transferred to our hospital for further evaluation. The patient developed chest pain and ST-segment elevation in leads V4-V5 on her electrocardiogram. She was transferred to the cardiac care unit where creatine kinase values were elevated with elevation of the MB fraction. She developed postinfarction angina and was taken to the catheterization laboratory. Results of hemodynamic studies were unremarkable. Left coronary angiography (Fig 1) revealed abrupt cutoffs of both divisions of a large obtuse marginal branch and other smaller branches. Right coronary arteriography (Fig 2) revealed marked changes in caliber of several small branches. There was also a paucity of septal branches off the posterior descending artery.Figure 2Arteriogram of the right coronary artery in the right anterior oblique projection shows dilatation and narrowing of right ventricular branches (arrows).View Large Image Figure ViewerDownload (PPT) Although involvement of all organ systems has been described, the vasculitis of CSS seems to involve the lungs, skin, heart, and peripheral nerves with the greatest frequency.4Lanham JG Elkon KB Pusey CD Hughes GR Systemic vasculitis with asthma and eosinophila: a clinical approach to the Churg-Strauss syndrome.Medicine. 1984; 63: 65-81Crossref PubMed Scopus (1040) Google Scholar, 5Chumbley LC Harrison EG DeRemee RA Allergic granulomatosis and angiitis (Churg-Strauss syndrome): report and analysis of 30 cases.Mayo Clin Proc. 1977; 52: 477-484PubMed Google Scholar, 7Churg J Strauss L Allergic granulomatosis, allergic angiitis, and periarteritis nodosa.Am J Pathol. 1951; 27: 277-301PubMed Google Scholar The original report by Churg and Strauss7Churg J Strauss L Allergic granulomatosis, allergic angiitis, and periarteritis nodosa.Am J Pathol. 1951; 27: 277-301PubMed Google Scholar noted cardiac failure as the cause of death in 3 of 11 (27%) patients. At postmortem examination, they found interstitial, eosinophilic inflammation of the myocardium in 7 of 11 (64%) autopsies and coronary vasculitis (acute or healed) in 6 of 10. Pericardial involvement was common, but the valves were essentially spared. The other large series of patients did not include enough autopsy information to supplement these data; however, 33%5Chumbley LC Harrison EG DeRemee RA Allergic granulomatosis and angiitis (Churg-Strauss syndrome): report and analysis of 30 cases.Mayo Clin Proc. 1977; 52: 477-484PubMed Google Scholar and 48%5Chumbley LC Harrison EG DeRemee RA Allergic granulomatosis and angiitis (Churg-Strauss syndrome): report and analysis of 30 cases.Mayo Clin Proc. 1977; 52: 477-484PubMed Google Scholar of the deaths in these series were attributed to cardiac causes. These percentages are striking in light of the relative youth of these patients; the average age of the patients in the above series was 47 and 38 years, respectively. In reviewing the literature, we could find only a single report of a patient with CSS surviving a clinically recognized myocardial infarction.8Suzuki N Arai Y Miyamoto Y Isokane N Fukushima N Sano Y Acute myocardial injury and repeated angina pectoris-like attacks in a young patient with Churg-Strauss syndrome.Jpn J Thorac Dis. 1991; 29: 1630-1637Google Scholar More common are case reports of death in which coronary vasculitis was detected at autopsy.2Lie JT Bayardo RJ Isolated eosinophilic coronary arteritis and eosinophilic myocarditis: a limited form of Churg-Strauss syndrome.Arch Pathol Lab Med. 1989; 113: 199-201PubMed Google Scholar,9Thomsen D Chamsi-Pasha H Hasleton P Heart transplantation for Churg-Strauss syndrome.Br Heart J. 1989; 62: 409-410Crossref PubMed Scopus (27) Google Scholar, 10Rosenberg TF Medsger TA DeCicco FA Fireman P Allergic granulomatous angiitis (Churg-Strauss syndrome).J Allergy Clin Immunol. 1975; 55: 56-67Abstract Full Text PDF PubMed Scopus (23) Google Scholar, 11Lee PC Gocke CD Harris ED Anderson ME Bergin CJ Price JM et al.Conferences and reviews: 47-year-old woman with 6-week history of lower extremity weakness and eosinophilia.West J Med. 1992; 156: 517-522PubMed Google Scholar Both large epicardial coronary arteries and smaller intramural coronary arteries have been implicated in this process. Cardiac decompensation was the terminal event in two cases;2Lie JT Bayardo RJ Isolated eosinophilic coronary arteritis and eosinophilic myocarditis: a limited form of Churg-Strauss syndrome.Arch Pathol Lab Med. 1989; 113: 199-201PubMed Google Scholar, 10Rosenberg TF Medsger TA DeCicco FA Fireman P Allergic granulomatous angiitis (Churg-Strauss syndrome).J Allergy Clin Immunol. 1975; 55: 56-67Abstract Full Text PDF PubMed Scopus (23) Google Scholar however, one patient underwent orthotopic cardiac transplantation on an emergent basis and recovered.9Thomsen D Chamsi-Pasha H Hasleton P Heart transplantation for Churg-Strauss syndrome.Br Heart J. 1989; 62: 409-410Crossref PubMed Scopus (27) Google Scholar Other case reports confirm the occurrence of clinical pericardial disease, including tamponade12Hasley PB Follansbee WP Coulehan JL Cardiac manifestations of Churg-Strauss syndrome: report of a case and review of the literature.Am Heart J. 1990; 120: 996-999Abstract Full Text PDF PubMed Scopus (81) Google Scholar, 13Davidson AG Thompson PJ Davies J Corrin B Turner-War-wick M Prominent pericardial and myocardial lesions in the Churg-Strauss syndrome (allergic granulomatosis and angiitis).Thorax. 1983; 38: 793-795Crossref PubMed Scopus (36) Google Scholar and left ventricular dysfunction.9Thomsen D Chamsi-Pasha H Hasleton P Heart transplantation for Churg-Strauss syndrome.Br Heart J. 1989; 62: 409-410Crossref PubMed Scopus (27) Google Scholar,12Hasley PB Follansbee WP Coulehan JL Cardiac manifestations of Churg-Strauss syndrome: report of a case and review of the literature.Am Heart J. 1990; 120: 996-999Abstract Full Text PDF PubMed Scopus (81) Google Scholar, 13Davidson AG Thompson PJ Davies J Corrin B Turner-War-wick M Prominent pericardial and myocardial lesions in the Churg-Strauss syndrome (allergic granulomatosis and angiitis).Thorax. 1983; 38: 793-795Crossref PubMed Scopus (36) Google Scholar, 14Lanham JG Cooke S Davies J Hughes GR Endomyocardial complication of the Churg-Strauss syndrome.Postgrad Med J. 1985; 61: 341-344Crossref PubMed Scopus (41) Google Scholar, 15Leung WH Wong KK Lau CP Wong CK Cheng CH So KF Myocardial involvement in Churg-Strauss syndrome: the role of endomyocardial biopsy.J Rheumatol. 1989; 16: 828-831PubMed Google Scholar In these latter cases, the relative contribution of vasculitis and primary myopathic processes cannot be determined. One echocardiographic study of 12 patients with CSS showed left ventricular dilatation in 4 of 12, decreased fractional shortening in 3 of 12, and mitral regurgitation in 6 of 12. Although the mitral regurgitation was mild in most of these patients, two eventually required mitral valve replacement.16Morgan JM Raposo L Gibson DG Cardiac involvement in Churg-Strauss syndrome shown by echocardiography.Br Heart J. 1989; 62: 462-466Crossref PubMed Scopus (78) Google Scholar There are several reports of endomyocardial biopsy specimens being used to determine the cause of decreased ventricular function in CSS. Most have been nondiagnostic12Hasley PB Follansbee WP Coulehan JL Cardiac manifestations of Churg-Strauss syndrome: report of a case and review of the literature.Am Heart J. 1990; 120: 996-999Abstract Full Text PDF PubMed Scopus (81) Google Scholar, 13Davidson AG Thompson PJ Davies J Corrin B Turner-War-wick M Prominent pericardial and myocardial lesions in the Churg-Strauss syndrome (allergic granulomatosis and angiitis).Thorax. 1983; 38: 793-795Crossref PubMed Scopus (36) Google Scholar, 14Lanham JG Cooke S Davies J Hughes GR Endomyocardial complication of the Churg-Strauss syndrome.Postgrad Med J. 1985; 61: 341-344Crossref PubMed Scopus (41) Google Scholar; however, at least one case of myocarditis has been diagnosed by this method.9Thomsen D Chamsi-Pasha H Hasleton P Heart transplantation for Churg-Strauss syndrome.Br Heart J. 1989; 62: 409-410Crossref PubMed Scopus (27) Google Scholar The literature contains several reports of the use of coronary arteriography in patients with CSS. This has been done to evaluate the cause of myocardial infarction,8Suzuki N Arai Y Miyamoto Y Isokane N Fukushima N Sano Y Acute myocardial injury and repeated angina pectoris-like attacks in a young patient with Churg-Strauss syndrome.Jpn J Thorac Dis. 1991; 29: 1630-1637Google Scholar chest pain,15Leung WH Wong KK Lau CP Wong CK Cheng CH So KF Myocardial involvement in Churg-Strauss syndrome: the role of endomyocardial biopsy.J Rheumatol. 1989; 16: 828-831PubMed Google Scholar and heart failure.12Hasley PB Follansbee WP Coulehan JL Cardiac manifestations of Churg-Strauss syndrome: report of a case and review of the literature.Am Heart J. 1990; 120: 996-999Abstract Full Text PDF PubMed Scopus (81) Google Scholar None of these previous investigators reported any arteriographic abnormalities. Nevertheless, given the clinical occurrence of myocardial ischemia and the previously reported autopsy data, it is likely that small-vessel vasculitis was present in these patients. To our knowledge, the present case represents the first abnormal coronary arteriogram reported in a patient with CSS. It seems likely that our patient had vasculitis of the coronary arteries since she had no risks for atherosclerosis, and the abnormalities of her coronary arteriograms did not suggest atherosclerosis. The most obvious abnormality was the truncation of both divisions of the obtuse marginal branch; other smaller branches were similarly affected. Although still photographs do not adequately capture it, there was slow flow with “pruning” of the vessels, particularly in the distribution of the posterior descending branch. This resulted in loss of the posterior septal “blush.” Finally, there were alternating areas of narrowing and dilatation in the smaller branches. Unlike the large visceral aneurysms seen in CSS and polyarteritis nodosa, these findings were more subtle. In fact, they resembled the arteriopathy that we and others have seen in recipients of heart transplants, a process that is believed to be immunologically mediated.17Hammond EH Yowell RL Price GD Menlove RL Olsen SL O’Connell JB et al.Vascular rejection and its relationship to allograft coronary artery diseases.J Heart Lung Transplant. 1992; 11: S111-9PubMed Google Scholar Many other entities have been associated with vasculitis of the coronary arteries.18Parrillo JE Fauci AS Necrotizing vasculitis, coronary angiitis, and the cardiologist.Am Heart J. 1980; 99: 547-551Abstract Full Text PDF PubMed Scopus (26) Google Scholar Some relatively common diseases, such as rheumatoid arthritis19Kozak M, Green LS. Cardiac involvement. In: Ward J, Cannon G, eds. Systemic manifestations of rheumatoid arthritis. New York: Marcel Dekker (in press)Google Scholar and systemic lupus erythematosus,20Homcy CJ Liberthson RR Fallon JT Gross S Miller LM Ischemic heart disease in systemic lupus erythematosus in the young patient: report of six cases.Am J Cardiol. 1982; 49: 478-484Abstract Full Text PDF PubMed Scopus (100) Google Scholar have been associated with coronary arteritis in a fairly large percentage of autopsied cases. However, the clinical significance of these findings is unknown, and antemortem recognition of coronary vasculitis is rare in these diseases. Other rare arteritides such as polyarteritis nodosa21Schrader ML Hochman JS Bulkley BH The heart in polyarteritis nodosa: a clinicopathologic study.Am Heart J. 1985; 109: 1353-1359Abstract Full Text PDF PubMed Scopus (72) Google Scholar and mucocutaneous lymph node syndrome (Kawasaki’s disease)22Chung K Brandt L Fulton DR Kreidberg MB Cardiac and coronary arterial involvement in infants and children with mucocutaneous lymph node syndrome.Am J Cardiol. 1982; 50: 136-142Abstract Full Text PDF PubMed Scopus (42) Google Scholar frequently involve the coronary arteries and are important causes of clinical morbidity and mortality. Our patient developed cardiac symptoms fairly late in the course of her illness, although the cardiac involvement seems to have progressed at a time when other manifestations of the syndrome were relatively quiet. After coronary angiography, cyclophosphamide was added to her corticosteroid therapy. Unfortunately, our patient did not survive. She died of cytomegolovirus and Pneumocystis carinii pneumonia related to immunosuppressive therapy. Autopsy showed bilateral ventricular dilatation. Sectioning of the myocardium showed numerous foci of irregularly bordered fibrosis with regions of subacute hemorrhagic infarction. Sectioning of the coronary arteries revealed no areas of atherosclerosis but did reveal, in medium and small arteries, extensive areas of intimal thickening and mild residual vasculitis evidenced by presence of inflammatory cells. No eosinophils were reported. Churg-Strauss syndrome is an uncommon entity that may not be known to many cardiologists. Pulmonary physicians are aware of CSS as a cause of pulmonary vasculitis. However, CSS commonly involves the heart and often responds to appropriate therapy with immunosuppressives. We believe that CSS should be considered in a young patient with cardiac signs or symptoms if there is a history of asthma and the peripheral eosinophil count is >10% of leukocytes.1Masi AT Hunder GG Lie JT Michel BA Bloch DA Arend WP et al.The American College of Rheumatology 1990 Criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis).Arthritis Rheum. 1990; 33: 1094-1100Crossref PubMed Scopus (1778) Google Scholar It is also well to remember that intercurrent steroid therapy can lower the eosinophil count. Finally, if there are clinical indications for coronary arteriography, we suggest that filming be prolonged and allow for better visualization of smaller vessels, as the findings may be subtle.