Морфологически верифицированный ANCA-позитивный панкардит Лёффлера: медикаментозное и хирургическое лечение

Благова О.В., Алиева И.Н., Недоступ А.В. и др. Морфологически верифицированный ANCA-позитивный панкардит Лёффлера: медикаментозное и хирургическое лечение. Терапевтический архив. 2019; 91 (4): 99–106. DOI: 10.26442/00403660.2019.04.000048

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Blagova O.V., Aliyeva I.N., Nedostup A.V., et al. Morphologically proved ANCA positive Loeffler’s pancarditis: medical and surgical treatment. Therapeutic Archive. 2019; 91 (4): 99–106. DOI: 10.26442/00403660.2019.04.000048

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Аннотация

Эндокардит Лёффлера остается очень редким заболеванием, развивается вследствие эозинофильного воспаления преимущественно эндокарда с исходом в фиброз и массивным тромбообразованием и в целом характеризуется неблагоприятным прогнозом.
Представлено клиническое наблюдение пациентки 42 лет с эндокардитом Лёффлера. Развитию заболевания предшествовали поливалентная аллергия, нетяжелый синдром сухого глаза и пансинусит с однократной эозинофилией крови до 16%. Поводом для госпитализации послужило появление и быстрое нарастание бивентрикулярной сердечной недостаточности. На момент обследования и в течение предшествующего года уровень эозинофилов крови оставался нормальным, однократно отмечено трехкратное повышение уровня эозинофильного катионного протеина. Выявлено 20-кратное повышение уровня рANCA, 2,5-кратное повышение уровня антител к ДНК, антитела к ядрам кардиомиоцитов 1:160. Диагноз поставлен на основании данных электрокардиографии (низкий вольтаж QRS, гипертрофия предсердий), эхокардиографии, мультиспиральной компьютерной томографии и магнитно-резонансной томографии сердца (утолщение и отсроченное контрастирование эндокарда, массивный тромбоз верхушки левого желудочка с облитерацией его полости, осумкованная жидкость в перикарде со сдавлением правого желудочка). Систолическая дисфункция, грубые признаки рестрикции и нарушения ритма отсутствовали. Выполнены тромбэктомия, пластика трикуспидального клапана, резекция перикарда, ушивание открытого овального окна. При морфологическом и иммуногистохимическом исследовании эндо-, мио-, перикарда – признаки активного воспаления с единичными эозинофилами, васкулит, перимускулярный склероз, склероз эндокарда. Вирусного генома не выявлено. Начата терапия метилпреднизолоном 24 мг/сут, азатиоприном 75 мг/сут. Через полгода после операции явления сердечной недостаточности полностью отсутствуют, тромбоз не рецидивировал.

Ключевые слова: эндокардит Лёффлера, миокардит, перикардит, панкардит, тромбоз левого желудочка, биопсия миокарда, антикардиальные антитела, антинейтрофильные цитоплазматические антитела, тромбэктомия, резекция перикарда, иммуносупрессивная терапия, рестриктивная кардиомиопатия.

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Loeffler's endocarditis remains is a very rare disease, develops due to eosinophilic inflammation predominantly of the endocardium with an outcome in fibrosis and massive thrombus formation and. He is generally characterized by an unfavorable prognosis. Clinical case of a 42-year-old patient with Loeffler endocarditis is presented. The development of the disease was preceded by a polyvalent allergy, mild dry eye syndrome and pansinusitis with a single eosinophilia of blood up to 16%. The reason for the hospitalization was the appearance of biventricular heart failure. During the previous year, the level of blood eosinophils remained normal, a threefold increase in the level of eosinophilic cationic protein was observed once. A 20-fold increase in the pANCA level, a 2.5-fold increase in the level of antibodies to DNA, an antibody to the nuclei of cardiomyocytes 1:160 were detected. The diagnosis was made on the basis of electrocardiography data (low QRS voltage, atrial hypertrophy), echocardiography, multispiral computed tomography and magnetic resonance imaging of the heart (thickening and delayed contrasting of the endocardium, massive thrombosis of the left ventricular apex with obliteration of its cavity, encapsulated fluid in the pericardium with compression of the right ventricle). Systolic dysfunction, severe signs of restriction and arrhythmias were absent. Trombectomy, tricuspid valve plasty, pericardial resection, suturing of an open oval window were performed. Signs of active inflammation with single eosinophils, vasculitis, perimuscular sclerosis, endocardial sclerosis were detected in morphological and immunohistochemical studies of endo-, myo-, pericardium. Viral genome was not found. The therapy with methylprednisolone 24 mg/day, azathioprine 75 mg/day was started. Six months after the operation, the symptoms of heart failure are completely absent, the thrombosis did not recur.

Key words: Loeffler’s endocarditis, myocarditis, pericarditis, pancarditis, left ventricular thrombosis, myocardial biopsy, anti-heart antibodies, antineutrophil cytoplasm antibodies, thrombectomy, pericardial resection, immunosuppressive therapy, restrictive cardiomyopathy.

Список литературы

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14. Brambatti M, Matassini MV, Adler ED, Klingel K, Camici PG, Ammirati E. Eosinophilic Myocarditis: Characteristics, Treatment, and Outcomes. J Am Coll Cardiol. 2017;70(19):2363-75. doi: 10.1016/j.jacc.2017.09.023
15. Butterfield JH, Kane GC, Weiler CR. Hypereosinophilic syndrome: endomyocardial biopsy versus echocardiography to diagnose cardiac involvement. Postgrad Med. 2017;129(5):517-23. doi: 10.1080/00325481.2017.1317215
16. Kasamatsu Y, Kida T, Shigeru M, Tagashira T, Murai N, Takai E, Takaoka H. Clinically suspected acute myopericarditis with cardiac tamponade associated with peripheral blood eosinophilia presenting in early pregnancy: a case report. J Med Case Rep. 2013;7(1):129. doi: 10.1186/1752-1947-7-129
17. Tan SA, Duggal A. Pericardial involvement as a rare manifestation of hypereosinophilic syndrome. South Med J. 2009;102(7):751-3. doi: 10.1097/SMJ.0b013e3181a9429d
18. Lui CY, Makoui C. Severe constrictive pericarditis as an unsuspected cause of death in a patient with idiopathic hypereosinophilic syndrome and restrictive cardiomyopathy. Clin Cardiol. 1988;11(7):502-4.
19. Song T, Jones DM, Homsi Y. Therapeutic effect of anti-IL-5 on eosinophilic myocarditis with large pericardial effusion. BMJ Case Rep. 2017;2017. pii: bcr-2016-218992. doi: 10.1136/bcr-2016-218992
20. Inoue T, Watanabe C, Ayukawa H, Nadahama T, Hosokawa A, Beppu K, Oya H, Sasaki Y, Takeda S, Shirasaka A, Doi T, Hashimoto T, Kawai C. Biopsy-proven Loeffler endocarditis successfully treated with steroids. Circulation. 2015 Feb 24;131(8):e353-4. doi: 10.1161/CIRCULATIONAHA.114.012976
21. Rotoli B, Catalano L, Galderisi M, Luciano L, Pollio G, Guerriero A, D'Errico A, Mecucci C, La Starza R, Frigeri F, Di Francia R, Pinto A. Rapid reversion of Loeffler's endocarditis by imatinib in early stage clonal hypereosinophilic syndrome. Leuk Lymphoma. 2004;45(12):2503-7.
22. Mannelli L, Cherian V, Nayar A, Srichai-Parsia M. Loeffler's endocarditis in hypereosinophilic syndrome. Curr Probl Diagn Radiol. 2012;41(4):146-8. doi: 10.1067/j.cpradiol.2011.07.015
23. Fassnacht F, Roumier M, Fouret P, Levy V, Varnous S, Russel S, Lefevre G, Kahn JE. Successful Heart Transplantation for Unreversible Endomyocardial Fibrosis Related to FIP1L1-PDGFRA Chronic Eosinophilic Leukemia. Transplantation. 2015;99(11):e176-7. doi: 10.1097/TP.0000000000000939
24. Gatti G, Poli S, Benussi B, Bussani R, Iorio A, Confalonieri M, Milo M, Tavcar I, Pappalardo A, Sinagra G. Left ventricular thrombectomy in myocarditis: the epicardial scan & video-assisted transaortic approach. Minim Invasive Ther Allied Technol. 2018 Apr;27(2):101-4. doi: 10.1080/13645706.2017.1361448
25. Schneider U, Jenni R, Turina J, Turina M, Hess OM. Long-term follow up of patients with endomyocardial fibrosis: effects of surgery. Heart. 1998;79(4):362-7.
26. Moraes F, Lapa C, Hazin S, Tenorio E, Gomes C, Moraes CR. Surgery for endomyocardial fibrosis revisited. Eur J Cardiothorac Surg. 1999;15(3):309-12; discussion 312-3.

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1. Loeffler W. Endocarditis parietalis fibroplastica mit Bluteosinophilie. Ein eigenartiges Krankheitsbild. Schweiz Med Wschr. 1936;66:817.
2. Cogan E, Roufosse F. Clinical management of the hypereosinophilic syndromes. Expert Rev Hematol. 2012;5(3):275-89; quiz 290. doi: 10.1586/ehm.12.14
3. Selivanenko VT, Martakov MA, Prokhorov AA, Dudakov VA, Shpak II. Surgical treatment of mitral valve failure due to Loеffler fibroplastic endocarditis. Kardiologiya i Serdechno-Sosudistaya Khirurgiya. 2009;2(1):82-3 (In Russ.).
4. Gao M, Zhang W, Zhao W, Qin L, Pei F, Zheng Y. Loeffler endocarditis as a rare cause of heart failure with preserved ejection fraction: A case report and review of literature. Medicine (Baltimore). 2018;97(11):e0079. doi: 10.1097/MD.0000000000010079
5. Salemi VM, Rochitte CE, Shiozaki AA, Andrade JM, Parga JR, de Ávila LF, Benvenuti LA, Cestari IN, Picard MH, Kim RJ, Mady C. Late gadolinium enhancement magnetic resonance imaging in the diagnosis and prognosis of endomyocardial fibrosis patients. Circ Cardiovasc Imaging. 2011;4(3):304-11. doi: 10.1161/CIRCIMAGING.110.950675
6. Strizhakov LA, Moiseev SV, Kogan EA, Pasha SP, Churganova LYu, Ditterle VE, Novikov PI, Semenkova EN, Mukhin NA. Cardiac involvement in systemic vasculitides: Pathogenetic mechanisms, the importance of risk factors for cardiovascular events, and diagnosis. Ther Archiv. 2014;86(12):35-42 (In Russ.).
7. Comarmond C, Pagnoux C, Khellaf M, Cordier JF, Hamidou M, Viallard JF, Maurier F, Jouneau S, Bienvenu B, Puéchal X, Aumaître O, Guenno GL, Quellec AL, Cevallos R, Fain O, Godeau B, Seror R, Dunogué B, Mahr A, Guilpain P, Cohen P, Aouba A, Mouthon L, Guillevin L; for the French Vasculitis Study Group (FVSG). Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) – clinical characteristics and long-term follow-up of the 383 patients enrolled in the FVSG cohort. Arthritis Rheum. 2013;65(1):270-81. doi: 10.1002/art.37721
8. Pugnet G, Gouya H, Puéchal X, Terrier B, Kahan A, Legmann P, Guillevin L, Vignaux O; French Vasculitis Study Group. Cardiac involvement in granulomatosis with polyangiitis: a magnetic resonance imaging study of 31 consecutive patients. Rheumatology (Oxford). 2017;56(6):947-56. doi: 10.1093/rheumatology/kew490
9. Qiao L, Gao D. A case report and literature review of Churg-Strauss syndrome presenting with myocarditis. Medicine (Baltimore). 2016;95(51):e5080. doi: 10.1097/MD.0000000000005080
10. Cereda AF, Pedrotti P, De Capitani L, Giannattasio C, Roghi A. Comprehensive evaluation of cardiac involvement in eosinophilic granulomatosis with polyangiitis (EGPA) with cardiac magnetic resonance. Eur J Intern Med. 2016 Oct 7. pii: S0953-6205(16)30315-6. doi: 10.1016/j.ejim.2016.09.014 [Epub ahead of print].
11. Groh M, Masciocco G, Kirchner E, Kristen A, Pellegrini C, Varnous S, Bortman G, Rosenberg M, Brucato A, Waterworth P, Bonacina E, Facchetti F, Calabrese L, Gregorini G, Scali JJ, Starling R, Frigerio M, D'Armini AM, Guillevin L. Heart transplantation in patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). J Heart Lung Transplant. 2014;33(8):842-50. doi: 10.1016/j.healun.2014.02.023
12. McGavin CR, Marshall AJ, Lewis CT. Churg-Strauss syndrome with critical endomyocardial fibrosis: 10 year survival after combined surgical and medical management. Heart. 2002;87(5):E5.
13. Androsova TV, Kozlovskaya LV, Taranova MV, Strizhakov LA, Gulyaev SV, Russkikh AV. Difficulties in the differential diagnosis of kidney injury in a patient with infective endocarditis associated with antineutrophil cytoplasmic antibodies. Ther Archive. 2017;89(6):84-8 (In Russ.). doi: 10.17116/terarkh201789684-88
14. Brambatti M, Matassini MV, Adler ED, Klingel K, Camici PG, Ammirati E. Eosinophilic Myocarditis: Characteristics, Treatment, and Outcomes. J Am Coll Cardiol. 2017;70(19):2363-75. doi: 10.1016/j.jacc.2017.09.023
15. Butterfield JH, Kane GC, Weiler CR. Hypereosinophilic syndrome: endomyocardial biopsy versus echocardiography to diagnose cardiac involvement. Postgrad Med. 2017;129(5):517-23. doi: 10.1080/00325481.2017.1317215
16. Kasamatsu Y, Kida T, Shigeru M, Tagashira T, Murai N, Takai E, Takaoka H. Clinically suspected acute myopericarditis with cardiac tamponade associated with peripheral blood eosinophilia presenting in early pregnancy: a case report. J Med Case Rep. 2013;7(1):129. doi: 10.1186/1752-1947-7-129
17. Tan SA, Duggal A. Pericardial involvement as a rare manifestation of hypereosinophilic syndrome. South Med J. 2009;102(7):751-3. doi: 10.1097/SMJ.0b013e3181a9429d
18. Lui CY, Makoui C. Severe constrictive pericarditis as an unsuspected cause of death in a patient with idiopathic hypereosinophilic syndrome and restrictive cardiomyopathy. Clin Cardiol. 1988;11(7):502-4.
19. Song T, Jones DM, Homsi Y. Therapeutic effect of anti-IL-5 on eosinophilic myocarditis with large pericardial effusion. BMJ Case Rep. 2017;2017. pii: bcr-2016-218992. doi: 10.1136/bcr-2016-218992
20. Inoue T, Watanabe C, Ayukawa H, Nadahama T, Hosokawa A, Beppu K, Oya H, Sasaki Y, Takeda S, Shirasaka A, Doi T, Hashimoto T, Kawai C. Biopsy-proven Loeffler endocarditis successfully treated with steroids. Circulation. 2015 Feb 24;131(8):e353-4. doi: 10.1161/CIRCULATIONAHA.114.012976
21. Rotoli B, Catalano L, Galderisi M, Luciano L, Pollio G, Guerriero A, D'Errico A, Mecucci C, La Starza R, Frigeri F, Di Francia R, Pinto A. Rapid reversion of Loeffler's endocarditis by imatinib in early stage clonal hypereosinophilic syndrome. Leuk Lymphoma. 2004;45(12):2503-7.
22. Mannelli L, Cherian V, Nayar A, Srichai-Parsia M. Loeffler's endocarditis in hypereosinophilic syndrome. Curr Probl Diagn Radiol. 2012;41(4):146-8. doi: 10.1067/j.cpradiol.2011.07.015
23. Fassnacht F, Roumier M, Fouret P, Levy V, Varnous S, Russel S, Lefevre G, Kahn JE. Successful Heart Transplantation for Unreversible Endomyocardial Fibrosis Related to FIP1L1-PDGFRA Chronic Eosinophilic Leukemia. Transplantation. 2015;99(11):e176-7. doi: 10.1097/TP.0000000000000939
24. Gatti G, Poli S, Benussi B, Bussani R, Iorio A, Confalonieri M, Milo M, Tavcar I, Pappalardo A, Sinagra G. Left ventricular thrombectomy in myocarditis: the epicardial scan & video-assisted transaortic approach. Minim Invasive Ther Allied Technol. 2018 Apr;27(2):101-4. doi: 10.1080/13645706.2017.1361448
25. Schneider U, Jenni R, Turina J, Turina M, Hess OM. Long-term follow up of patients with endomyocardial fibrosis: effects of surgery. Heart. 1998;79(4):362-7.
26. Moraes F, Lapa C, Hazin S, Tenorio E, Gomes C, Moraes CR. Surgery for endomyocardial fibrosis revisited. Eur J Cardiothorac Surg. 1999;15(3):309-12; discussion 312-3.

Авторы

О.В. Благова 1, И.Н. Алиева 1, А.В. Недоступ 1, Е.А. Коган 1, Р.Н. Комаров 1, С.В. Чернявский 1, Т.В. Сеславинская 1, П.А. Шелуха 1, В.П. Седов 1, Н.В. Гагарина 1, Е.А. Мершина 2, В.Е. Синицын 2, В.В. Фомин 1

1 ФГАОУ ВО «Первый Московский государственный медицинский университет им. И.М. Сеченова» Минздрава России (Сеченовский Университет), Москва, Россия;
2 ФГАУ «Лечебно-реабилитационный центр» Минздрава России, Москва, Россия

________________________________________________

O.V. Blagova 1, I.N. Aliyeva 1, A.V. Nedostup 1, E.A. Kogan 1, R.N. Komarov 1, S.V. Chernyavsky 1, V.V. Seslavinskaya 1, P.A. Shelukha 1, V.P. Sedov 1, N.V. Gagarina 1, E.A. Mershina 2, V.E. Sinitsyn 2, V.V. Fomin 1

1 I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University), Moscow, Russia;
2 Federal Center of Treatment and Rehabilitation of the Ministry of Health of the Russian Federation, Moscow, Russia

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