American Journal of Emergency Medicine xxx (2014) xxx–xxx
Contents lists available at ScienceDirect
American Journal of Emergency Medicine journal homepage: www.elsevier.com/locate/ajem
Case Report
Acute heart failure as a form of relapse in a patient with adult-onset Still disease Abstract Adult-onset Still disease (AOSD) is a rare systemic disorder, affecting mostly young subjects, particularly women. Myocarditis is rare in adults, and it usually presents as myopericarditis. It may cause arrhythmias, heart failure, and sudden death, as other myocarditis. The definite diagnosis is only confirmed by endomyocardial biopsy. Our patient had a history of AOSD and consulted to the emergency department with acute heart failure. Ventricular tachycardia, severe left ventricular dysfunction, and the laboratory test made us think of myocardial compromise associated with AOSD, which was finally confirmed by endomyocardial biopsy. We used corticosteroids with favorable response. Adult-onset Still disease (AOSD) is a rare systemic disorder, affecting mostly young subjects, particularly women, with an incidence of about 0.22 to 0.34 cases per 100 000 persons per year [1]. Cardiac manifestations are rare. Serositis is present in 30% to 40% of cases. Pericarditis is the most common cardiac manifestation present in 30% of cases and is subclinical in up to 50% of cases [2]. Myocarditis is less common in adults, and it usually presents as myopericarditis. It may cause arrhythmias, heart failure, and sudden death, as other myocarditis. The definite diagnosis is only confirmed by the presence of monocyte infiltration and fibroblasts at endomyocardial biopsy [3–5]. We present a patient with recurrent AOSD with rheumatic and cardiac symptoms. The patient was a 48-year-old woman, a former smoker with a history of AOSD since 2007, who initially presented with joint involvement, prolonged febrile syndrome, and skin rash. She remained stable, medicated with prednisone 5 mg/d, hydroxychloroquine 200 mg twice a day, and methotrexate 25 mg/wk, until September 2012, when she presented with a disease flare with symptoms of arthritis and dyspnea in functional classification (FC) I-II. The dose of methotrexate was increased to 20 mg/wk. The symptoms of arthritis improved, but dyspnea progressed to FC II-III. One month later, she consulted the emergency department because of dyspnea in FC IV, asthenia, dizziness, tachycardia, and peripheral edema. She was admitted with tachypnea, tachycardia, decreased oxygen saturation, and hypotension but did not present with fever. The apex beat was shifted to left, a holosystolic murmur of mitral regurgitation and S4 were present at auscultation, and jugular venous pressure was elevated with a rise during inspiration. Vesicular breath sounds were decreased at both bases of the lungs, with bilateral wheezes and crackles over the lung bases and middle fields. Liver enlargement and lower extremities edema 3 + were also present.
The electrocardiogram showed sinus rhythm alternating with atrial tachycardia, with a heart rate of 100 beats/min, a PR interval of 160 milliseconds, delayed conduction in the right bundle branch, and Q waves in leads II, lead III, and aVF. The chest x-ray showed cardiac enlargement, bilateral pulmonary venous congestion, and blunting of both costophrenic angles suggestive of bilateral pleural effusion and kyphoscoliosis. The patient was admitted at our coronary care unit with a diagnosis of congestive heart failure and supraventricular arrhythmias. Laboratory tests at admission were as follows: hematocrit, 32%; hemoglobin, 10 g/dL; white blood cells count, 17 000/mm 3 (neutrophils, 86%; basophils, 0.1%; eosinophils, 1.2%; lymphocytes, 8.8%; monocytes, 4%); platelets, 301 000/mm 3; blood urea nitrogen, 40 mg/dL; creatinine, 0.8 mg/dL; plasma glucose, 92 mg/dL; sodium, 132 mEq/L; potassium, 4.2 mEq/L; magnessium, 1.9 mEq/ L; creatine kinase, 390; creatine kinase–MB, 30 U/L; troponin T, 66 ng/L; aspartate aminotransferase, 169 U/L; alanine transaminase, 330 U/L; total bilirubin, 0.91 mg/dL; total proteins, 5.5 g/dL; alkaline phosphatase, 159 U/L; Ca 2 +, 7.8 mg/dL; erythrocyte sedimentation rate, 82 mm; polymerase chain reaction, 19 U/L; ferritin, 1650 ng/mL; lactate dehydrogenase, 857 U/L; and thyroid hormones, within normal ranges. Color-Doppler echocardiography showed mild left ventricular dilation with severe ventricular dysfunction, ejection fraction of 30%, akinesia of the inferior, inferoseptal and inferolateral segments at the midlevel and basal level and hypokinesia of the remaining segments, moderate to severe mitral regurgitation due to mitral valve tenting, mild right ventricular dysfunction, and systolic pulmonary artery pressure of 50 mm Hg. Left ventricular filling pressures were increased, and there was mild tricuspid regurgitation. During the first night after hospitalization, the patient presented with episodes of sustained ventricular tachycardia with hemodynamic impairment, requiring electrical cardioversion, followed by intravenous infusion of amiodarone (Fig. 1). The arrhythmia relapsed three times. A diagnosis of autoimmune or viral myocarditis was suspected as cause of acute heart failure and arrhythmias owing to the history of autoimmune disease, clinical findings, and type of presentation and elevated cardiac enzymes. An endomyocardial biopsy was performed (Fig. 2). The patient underwent coronary angiography because of electrocardiogram changes and segmental wall motion abnormalities, and coronary artery disease was ruled out. The biopsy showed focal endocardial thickness due to thin fibrosis, lymphocyte infiltrate and scarce leukocytes, and mild to moderate diffuse myocardial hypertrophy. Tissue damage by leukocytes (neutrophils and eosinophils) was seen in a fragment. There were also irregular edema and focal perivascular infiltrates
0735-6757/$ – see front matter © 2014 Elsevier Inc. All rights reserved.
Please cite this article as: Acosta A, et al, Acute heart failure as a form of relapse in a patient with adult-onset Still disease, Am J Emerg Med (2014), http://dx.doi.org/10.1016/j.ajem.2014.02.021
2
A. Acosta et al. / American Journal of Emergency Medicine xxx (2014) xxx–xxx
Fig. 1. Electrocardiogram with sustained ventricular tachycardia.
Adriana Acosta MD Division of Cardiology Instituto Cardiovascular de Buenos Aires Buenos Aires, Argentina
of neutrophils with nuclear dust suggestive of myocarditis associated with AOSD. Solumedrol pulses of 1 g/d for 3 days were indicated, followed by deltisone 40 mg/d and methotrexate 20 mg/wk. The patient achieved a negative fluid balance with favorable response. An implantable cardioverter-defibrillator was implanted before discharge as secondary prevention because of high risk of arrhythmias. One month later, the device delivered a new shock. The patient is currently free of angina and dyspnea, and the arthritis is in remission. She did not present with further episodes of heart failure or arrhythmia. The last echocardiogram, performed in July 2013, demonstrated normal left ventricular dimension, with mild left ventricular dysfunction, an ejection fraction of 41%, mild mitral regurgitation, mild right ventricular dysfunction, systolic pulmonary artery pressure of 38 mm Hg, and mild tricuspid regurgitation. The laboratory tests showed an erythrocyte sedimentation rate of 52 mm, C-reactive protein of 0.5 U/L, and ferritin of 45 ng/mL. The corticosteroid was gradually reduced to 8 mg of meprednisone per day.
Jorge Thierer MD Division of Cardiology, CEMIC Buenos Aires, Argentina Diego Conde MD Division of Cardiology Instituto Cardiovascular de Buenos Aires Buenos Aires, Argentina E-mail address: [email protected] Cecilia Pisoni MD Division of Rheumatology, CEMIC Buenos Aires, Argentina Julio San Martino MD Laboratorio de Patologia Buenos Aires, Argentina Marcelo Trivi MD Mirta Diez MD Division of Cardiology Instituto Cardiovascular de Buenos Aires Buenos Aires, Argentina
http://dx.doi.org/10.1016/j.ajem.2014.02.021
References
Fig. 2. Endomyocardial biopsy. The biopsy showed focal endocardial thickness due to thin fibrosis, lymphocyte infiltrate and scarce leukocytes, and mild to moderate diffuse myocardial hypertrophy. Tissue damage by leukocytes (neutrophils and eosinophils) was seen in a fragment. There were also irregular edema and focal perivascular infiltrates of neutrophils with nuclear dust suggestive of myocarditis associated with AOSD.
[1] Yoo WH. Adult onset Still's disease flared with pericardial effusion. Rheumatol Int 2008;28(3):285–7. [2] Buss Sebastian J, Wolf David, Mereles Derliz, Blank Norbert, Katus Hugo A, Hardt Stefan E. A rare case of reversible constrictive pericarditis with severe pericardial thickening in a patient with adult onset Still's disease. Int J Cardiol 2010;144(2):e23–5. [3] Bank I, Marboe CC, Redberg RF, Jacobs J. Myocarditis in adult Still’s disease. Arthritis Rheum 1985;28:452–4. [4] Sachs RN, Talvard O, Lanfranchi J. Myocarditis in adult Still’s disease. Int J Cardiol 1990;27(3):377–80. [5] Jadhav P, Nanayakkara N. Myocarditis in adult onset Stills disease. Int J Rheum Dis 2009;12(3):272–4.
Please cite this article as: Acosta A, et al, Acute heart failure as a form of relapse in a patient with adult-onset Still disease, Am J Emerg Med (2014), http://dx.doi.org/10.1016/j.ajem.2014.02.021
Contents lists available at ScienceDirect
American Journal of Emergency Medicine journal homepage: www.elsevier.com/locate/ajem
Case Report
Acute heart failure as a form of relapse in a patient with adult-onset Still disease Abstract Adult-onset Still disease (AOSD) is a rare systemic disorder, affecting mostly young subjects, particularly women. Myocarditis is rare in adults, and it usually presents as myopericarditis. It may cause arrhythmias, heart failure, and sudden death, as other myocarditis. The definite diagnosis is only confirmed by endomyocardial biopsy. Our patient had a history of AOSD and consulted to the emergency department with acute heart failure. Ventricular tachycardia, severe left ventricular dysfunction, and the laboratory test made us think of myocardial compromise associated with AOSD, which was finally confirmed by endomyocardial biopsy. We used corticosteroids with favorable response. Adult-onset Still disease (AOSD) is a rare systemic disorder, affecting mostly young subjects, particularly women, with an incidence of about 0.22 to 0.34 cases per 100 000 persons per year [1]. Cardiac manifestations are rare. Serositis is present in 30% to 40% of cases. Pericarditis is the most common cardiac manifestation present in 30% of cases and is subclinical in up to 50% of cases [2]. Myocarditis is less common in adults, and it usually presents as myopericarditis. It may cause arrhythmias, heart failure, and sudden death, as other myocarditis. The definite diagnosis is only confirmed by the presence of monocyte infiltration and fibroblasts at endomyocardial biopsy [3–5]. We present a patient with recurrent AOSD with rheumatic and cardiac symptoms. The patient was a 48-year-old woman, a former smoker with a history of AOSD since 2007, who initially presented with joint involvement, prolonged febrile syndrome, and skin rash. She remained stable, medicated with prednisone 5 mg/d, hydroxychloroquine 200 mg twice a day, and methotrexate 25 mg/wk, until September 2012, when she presented with a disease flare with symptoms of arthritis and dyspnea in functional classification (FC) I-II. The dose of methotrexate was increased to 20 mg/wk. The symptoms of arthritis improved, but dyspnea progressed to FC II-III. One month later, she consulted the emergency department because of dyspnea in FC IV, asthenia, dizziness, tachycardia, and peripheral edema. She was admitted with tachypnea, tachycardia, decreased oxygen saturation, and hypotension but did not present with fever. The apex beat was shifted to left, a holosystolic murmur of mitral regurgitation and S4 were present at auscultation, and jugular venous pressure was elevated with a rise during inspiration. Vesicular breath sounds were decreased at both bases of the lungs, with bilateral wheezes and crackles over the lung bases and middle fields. Liver enlargement and lower extremities edema 3 + were also present.
The electrocardiogram showed sinus rhythm alternating with atrial tachycardia, with a heart rate of 100 beats/min, a PR interval of 160 milliseconds, delayed conduction in the right bundle branch, and Q waves in leads II, lead III, and aVF. The chest x-ray showed cardiac enlargement, bilateral pulmonary venous congestion, and blunting of both costophrenic angles suggestive of bilateral pleural effusion and kyphoscoliosis. The patient was admitted at our coronary care unit with a diagnosis of congestive heart failure and supraventricular arrhythmias. Laboratory tests at admission were as follows: hematocrit, 32%; hemoglobin, 10 g/dL; white blood cells count, 17 000/mm 3 (neutrophils, 86%; basophils, 0.1%; eosinophils, 1.2%; lymphocytes, 8.8%; monocytes, 4%); platelets, 301 000/mm 3; blood urea nitrogen, 40 mg/dL; creatinine, 0.8 mg/dL; plasma glucose, 92 mg/dL; sodium, 132 mEq/L; potassium, 4.2 mEq/L; magnessium, 1.9 mEq/ L; creatine kinase, 390; creatine kinase–MB, 30 U/L; troponin T, 66 ng/L; aspartate aminotransferase, 169 U/L; alanine transaminase, 330 U/L; total bilirubin, 0.91 mg/dL; total proteins, 5.5 g/dL; alkaline phosphatase, 159 U/L; Ca 2 +, 7.8 mg/dL; erythrocyte sedimentation rate, 82 mm; polymerase chain reaction, 19 U/L; ferritin, 1650 ng/mL; lactate dehydrogenase, 857 U/L; and thyroid hormones, within normal ranges. Color-Doppler echocardiography showed mild left ventricular dilation with severe ventricular dysfunction, ejection fraction of 30%, akinesia of the inferior, inferoseptal and inferolateral segments at the midlevel and basal level and hypokinesia of the remaining segments, moderate to severe mitral regurgitation due to mitral valve tenting, mild right ventricular dysfunction, and systolic pulmonary artery pressure of 50 mm Hg. Left ventricular filling pressures were increased, and there was mild tricuspid regurgitation. During the first night after hospitalization, the patient presented with episodes of sustained ventricular tachycardia with hemodynamic impairment, requiring electrical cardioversion, followed by intravenous infusion of amiodarone (Fig. 1). The arrhythmia relapsed three times. A diagnosis of autoimmune or viral myocarditis was suspected as cause of acute heart failure and arrhythmias owing to the history of autoimmune disease, clinical findings, and type of presentation and elevated cardiac enzymes. An endomyocardial biopsy was performed (Fig. 2). The patient underwent coronary angiography because of electrocardiogram changes and segmental wall motion abnormalities, and coronary artery disease was ruled out. The biopsy showed focal endocardial thickness due to thin fibrosis, lymphocyte infiltrate and scarce leukocytes, and mild to moderate diffuse myocardial hypertrophy. Tissue damage by leukocytes (neutrophils and eosinophils) was seen in a fragment. There were also irregular edema and focal perivascular infiltrates
0735-6757/$ – see front matter © 2014 Elsevier Inc. All rights reserved.
Please cite this article as: Acosta A, et al, Acute heart failure as a form of relapse in a patient with adult-onset Still disease, Am J Emerg Med (2014), http://dx.doi.org/10.1016/j.ajem.2014.02.021
2
A. Acosta et al. / American Journal of Emergency Medicine xxx (2014) xxx–xxx
Fig. 1. Electrocardiogram with sustained ventricular tachycardia.
Adriana Acosta MD Division of Cardiology Instituto Cardiovascular de Buenos Aires Buenos Aires, Argentina
of neutrophils with nuclear dust suggestive of myocarditis associated with AOSD. Solumedrol pulses of 1 g/d for 3 days were indicated, followed by deltisone 40 mg/d and methotrexate 20 mg/wk. The patient achieved a negative fluid balance with favorable response. An implantable cardioverter-defibrillator was implanted before discharge as secondary prevention because of high risk of arrhythmias. One month later, the device delivered a new shock. The patient is currently free of angina and dyspnea, and the arthritis is in remission. She did not present with further episodes of heart failure or arrhythmia. The last echocardiogram, performed in July 2013, demonstrated normal left ventricular dimension, with mild left ventricular dysfunction, an ejection fraction of 41%, mild mitral regurgitation, mild right ventricular dysfunction, systolic pulmonary artery pressure of 38 mm Hg, and mild tricuspid regurgitation. The laboratory tests showed an erythrocyte sedimentation rate of 52 mm, C-reactive protein of 0.5 U/L, and ferritin of 45 ng/mL. The corticosteroid was gradually reduced to 8 mg of meprednisone per day.
Jorge Thierer MD Division of Cardiology, CEMIC Buenos Aires, Argentina Diego Conde MD Division of Cardiology Instituto Cardiovascular de Buenos Aires Buenos Aires, Argentina E-mail address: [email protected] Cecilia Pisoni MD Division of Rheumatology, CEMIC Buenos Aires, Argentina Julio San Martino MD Laboratorio de Patologia Buenos Aires, Argentina Marcelo Trivi MD Mirta Diez MD Division of Cardiology Instituto Cardiovascular de Buenos Aires Buenos Aires, Argentina
http://dx.doi.org/10.1016/j.ajem.2014.02.021
References
Fig. 2. Endomyocardial biopsy. The biopsy showed focal endocardial thickness due to thin fibrosis, lymphocyte infiltrate and scarce leukocytes, and mild to moderate diffuse myocardial hypertrophy. Tissue damage by leukocytes (neutrophils and eosinophils) was seen in a fragment. There were also irregular edema and focal perivascular infiltrates of neutrophils with nuclear dust suggestive of myocarditis associated with AOSD.
[1] Yoo WH. Adult onset Still's disease flared with pericardial effusion. Rheumatol Int 2008;28(3):285–7. [2] Buss Sebastian J, Wolf David, Mereles Derliz, Blank Norbert, Katus Hugo A, Hardt Stefan E. A rare case of reversible constrictive pericarditis with severe pericardial thickening in a patient with adult onset Still's disease. Int J Cardiol 2010;144(2):e23–5. [3] Bank I, Marboe CC, Redberg RF, Jacobs J. Myocarditis in adult Still’s disease. Arthritis Rheum 1985;28:452–4. [4] Sachs RN, Talvard O, Lanfranchi J. Myocarditis in adult Still’s disease. Int J Cardiol 1990;27(3):377–80. [5] Jadhav P, Nanayakkara N. Myocarditis in adult onset Stills disease. Int J Rheum Dis 2009;12(3):272–4.
Please cite this article as: Acosta A, et al, Acute heart failure as a form of relapse in a patient with adult-onset Still disease, Am J Emerg Med (2014), http://dx.doi.org/10.1016/j.ajem.2014.02.021
