International Journal of Cardiology, 34 (1992) 57-62 0 1992 Elsevier Science Publishers B.V. All rights reserved
CARD10
57 0167-5273/92/$05.00
01371
Symptomatic cardiac involvement in juvenile rheumatoid
arthritis
Jo& Goldenberg,
Marcos B. Ferraz, Angela P. Pessoa, Antonio S.M. Fonseca, Antonio C. Carvalho, Maria 0. Hilario and Edgard Atra
Dirlision of Curdiofogy and Rheumatology, Department of Medicine and Diuision of Allergy, Immunology and Rheumatology, Department of Pediatrics, Escola Paul&a de Medicina, Sco Paula, Brazil (Received
2 April 1991; revision
accepted
22 July 1991)
Goldenberg J, Ferraz MB, Pessoa AP, Fonseca ASM, Carvalho AC, Hilario MO, Atra E. Symptomatic cardiac involvement in juvenile rheumatoid arthritis. Int J Cardiol 1992;34:57-62. In a retrospective study of 172 patients with juvenile rheumatoid arthritis, symptomatic cardiac involvement occurred in 13 (7.6%) patients (11 systemic and 2 polyarticular). There was predominance of the male sex and in most patients the involvement occurred in the initial years of the disease. Pericarditis occurred in seven patients; perimyocarditis in four and myocarditis in two patients. In the follow-up, one of the patients with pericarditis died of an arrhythmia during pericardiocentesis for cardiac tamponade. Among the patients with myocarditis, three died of septicemia during active disease. One of these three patients had myocarditis associated with cardiac tamponade. Among the 172 patients with juvenile rheumatoid arthritis, five children died; four belonged to the symptomatic cardiac involvement group (P < 0.001). Cardiac involvement, in particular myocarditis and cardiac tamponade, can he regarded as a factor of worse prognosis. Key words: Juvenile rheumatoid
arthritis; Myocarditis; Pericarditis; Cardiac involvement
Introduction The cardiac involvement in juvenile rheumatoid arthritis was first described by Still in 1897 [Il. Among the heart layers the pericardium is the most frequently affected one followed by myocardium, and less frequently parietal and valvar endocardium [2,3]. Cardiac involvement is cliniCorrespondence to: Dr. JosC Goldenberg, M.D., PhD., Escola Paulista de Medicina, Disciplina de Reumatologia, Rua Botucatti 740, Vila Clementino, CEP 04023, S%o Paula, Brazil.
cally evident in 3-9% of patients with juvenile rheumatoid arthritis, especially in its systemic form [2-51. Pericarditis is usually benign. In some cases, however, it evolves to constriction [6,7] and/or cardiac tamponade [8-141 and is occasionally associated with pleuritis as part of a polyserositis [3,4,151. Echocardiographic studies in juvenile rheumatoid arthritis patients detected pericarditis in as much as 36% of the cases [16]. This finding is consistent with the incidence reported in autopsy studies, which showed pericardial involvement in 45% of the cases [4].
58
cardiography could be performed in 10 of the 13 selected cases. Pericarditis was diagnosed when any two of the following were observed: pericardial friction rub; enlargement of cardiac silhouette; ventricular repolarization changes; decreased amplitude of QRS complexes; evidence of pericardial effusion or thickening. The following criteria were used to diagnose myocarditis: presence of third heart sound or evidence of congestive heart failure in the absence of pericardial effusion or constriction, sysor endocarditis; chamber temic hypertension, overloador evidence of myocardial damage detectedby electrocardiography; enlargement of cardiac chambers or reduced myocardial contractility detected by echocardiography.
Myocarditis, associated or not with pericarditis, produces more expressive clinical manifestations [17,18]. It can lead to congestive heart failure and arrhythmias, causing death in juvenile rheumatoid arthritis patients [ 19-211. Although not a frequent manifestation, mitral and aortic valves can be affected, especially in patients with a severe aggressive arthritis associated with high titers of rheumatoid factor [22,231 and in its pauciarticular form with HLA-B27 positivity [24]. The present study is a retrospective analysis of 172 patients with juvenile rheumatoid arthritis evaluated by us in a 16-year period.
Materials and Methods Results
The study included 172 patients with juvenile rheumatoid arthritis, classified according to American Rheumatism Association criteria (1986) [25], selected from the Pediatrics Rheumatology Division of Escola Paulista de Medicina from 1972 to 1988. The demographic characteristics of the patients are shown in Table 1. Thirteen cases presented clinical evidence of cardiac involvement during an exacerbation in the course of disease. The historical data, clinical features, radiological, electrocardiographic and echocardiographic findings were analysed. Echo-
The heart was affected during the course of juvenile rheumatoid arthritis in 13 patients (7.6%), of whom 11 presented with the systemic form, and two with the polyarticular form. In the 13 children with detectable cardiac involvement none had a recent infection. Male to female ratio was 8:5. Nine patients were Caucasian and 4 non-Caucasian. The age at the onset of disease ranged from 2 years and 3 months to 14 years and 1 month (mean: 5 years and 5 months); cardiac
TABLE 1 Demographic characteristics of juvenile rheumatoid arthritis (JRA) patients Type of JRA
Race
Sex M
F
C
NC
Systemic (n = 57)
28
29
47
10
Polyarticular (?I = 57)
16
41
45
12
Pauciarticular (n = 58)
27
31
47
11
Age at onset of disease range (mean)
Duration of the disease range
llmoto14yr and 11 mo (5 yr 4 mo) 6moto16yr and 5 mo (6 yr and 9 mo) 12 mo to 14 yr and 2 mo (7 yr and 1 mo)
2moto16yr
M = male; F = female; C = Caucasian; NC = non-Caucasian; yr = years; mo = months.
6 mo to 15 yr
9 mo to 18 yr
59
involvement was diagnosed in patients aged from 2 years and 3 months to 14 years and 8 months (mean: 5 years and 10 months). In one case the cardiac involvement preceded articular involvement. In the majority of the patients, heart involvement was diagnosed within the first year of the disease. A total of 17 acute episodes were observed; 10 patients presented only one acute episode, and 3 patients recurring episodes. Isolated pericarditis, the most common cardiac involvement, was found in 5 patients with systemic juvenile rheumatoid arthritis and in 2 with the polyarticular form. Myopericarditis was found in 4 patients and isolated myocarditis in 2 patients, all with systemic juvenile rheumatoid arthritis. Clinical manifestations, radiological and graphic findings are presented in Tables 2 and 3. One of the patients with isolated pericarditis died due to ventricular tachycardia during pericardiocentesis for acute cardiac tamponade. The other 6 patients presented a good resolution of their pericarditis. As for articular involvement, 3 patients were in remission, and 3 presented with polyarticular involvement. Among the patients with myocardial involvement, recurrent episodes of cardiac involvement occurred in three. Acute cardiac tamponade was treated successfully in one case by pericardiec-
TABLE 2 Clinical manifestations involvement
in patients with symptomatic cardiac
Signs and symptoms
Isolated pericarditis (n=7)
Isolated myocarditis (n = 2) or perimyocarditis (n = 4)
Tachycardia Friction rub Dull heart sounds Tachypnea Dyspnea Congestive heart failure Cardiac tamponade Third heart sound Systolic murmur
7 4 2 2 1 1 1
6 2 1
_
6 5 1 5 2
TABLE 3 Radiological, electrocardiographic, and echocardiographic findings observed in juvenile rheumatoid arthritis patients with symptomatic cardiac involvement (n = 13) Chest X-rays Enlarged cardiac silhouette Pleural effusion Evidence of pulmonary congestion Electrocardiography Repolarization abnormalities Chamber overload Subepicardial lesion Echocardiography * Pericardial effusion Enlargement of cardiac chambers Decreased myocardial contractility
9 6 5 8 3 1
8 4 2
* Three patients were not submitted to echocardiography.
tomy. Three patients died due to sepsis during a flare-up of the disease. Of the 172 juvenile rheumatoid arthritis cases studied, five patients died, of whom 4 presented with cardiac involvement which seems to be a factor of poor prognosis (P < 0.0001). Juvenile rheumatoid arthritis began earlier in deceased patients (mean age: 2 years and 7 months), as compared with survivors (mean age: 6 years and 8 months). Death occurred usually within the first year of disease (3 out of 4) especially in patients presenting with myocardial involvement and/or cardiac tamponade. The drug of choice for therapeutic regimen was prednisone (1 mg/kg/day) for at least 3 months. Low doses of digitalis were administered to 4 patients with congestive heart failure. For patients presenting with cardiac tamponade, pericardiocentesis was performed in one and pericardiectomy in the other. Discussion The incidence of clinically evident heart involvement in our patients was 7.6%, a finding similar to those reported in the literature [2-51. Eighty five percent of our patients with symptomatic cardiac involvement suffered from systemic juvenile rheumatoid arthritis, data similar to that reported in the literature [2,5,16,17]. Only
60
two of our patients presented with the polyarticular form. No significant difference was observed in the age of patients with or without cardiac involvement (5 years and 5 months and 5 years and 4 months respectively). The finding is consistent with the literature [3,4,16]. Our observations indicate that the most severe cases were among patients who presented with the disease before the age of four years since the disease started in all the fatal cases by this age. The interval between the diagnosis of articular manifestations and cardiac involvement was less than 3 years and 4 months and the cardiac involvement occurred within the first year of disease in the majority of cases. Isolated pericarditis was the most frequent cardiac manifestation and was found in 54% of symptomatic cases. A relatively high incidence of myocardial involvement, with or without pericarditis, was observed in 3.5% of all juvenile rheumatoid arthritis patients and 46% of symptomatic cases. The incidence was higher than that described in the literature, ranging from 0.5 to 1.3% of all juvenile rheumatoid arthritis patients
KU71. Clinical manifestations of pericarditis were mild. Tachycardia and friction rub were the most frequent findings, folIowed by dyspnea and cardiac tamponade in one patient. The findings are in agreement with those of other authors [3,4,26]. A careful clinical evaluation, as well as additional tests, are needed for early diagnosis of pericarditis. Differential diagnosis between rheumatoid pericarditis and viral pericarditis can be difficult. Viral pericarditis frequently follows an upper respiratory infection with a short latent period, and acute onset [27]. Other entities associated with pericarditis in children include rheumatic fever, bacterial infection, and, less frequently, benign idiopathic pericarditis [ 131. In contrast, myocardial involvement produced florid clinical manifestations, including tachypnea and dyspnea in all patients. A systolic murmur was found in 33% of our patients and has been described by others [18] as a clinical finding during the active phase of disease. It is due to
hyperkinesia and/or widening of the fibrous ring, secondary to enlargement of cardiac chambers, seeming to be indicative of organic valvar damage. This murmur tends to disappear with clinical management of myocarditis. Congestive heart failure was found in 5 cases (83%) and was indicative of severe cardiac involvement. Chest radiography and electrocardiography are useful for indicating cardiac involvement as reported by other authors [2-41. However, they have a limited value since cardiac enlargement and repolarization changes are nonspecific and found in the presence of pericarditis as well as myocarditis [3,4,17,18]. Echocardiography is a more accurate method for detecting cardiac lesions due to its high specificity and sensitivity. Echocardiograms may reveal abnormal findings in patients presenting with few symptoms and normal chest radiography or electrocardiography. Echocardiography associated with clinical findings plays an important role in the detection of pericardial, myocardial, and endocardial involvement. The course of isolated pericarditis was benign, except in one case who developed cardiac tamponade, and died during pericardiocentesis, due to ventricular tachycardia. The usually benign course of pericarditis has been reported in the Iiterature [3,4,26]. On the other hand, myocardial involvement had a more severe course, especially in those patients with congestive heart failure, three of whom died. The other three cases were in remission after a follow-up ranging from 4 months to 8 years, with one still on treatment with corticosteroids. Considering the influence of cardiac involvement, especially myocarditis and cardiac tamponade, on the mortality observed by us, we advocate that all juvenile rheumatoid arthritis patients, especially those with florid systemic manifestations, should be submitted to electrocardiography and echocardiogram, in order that the cardiac involvement can be detected early and treated, and more severe disease is prevented. The mortality and symptomatic cardiac involvement in our patients were higher than that reported earlier [3,4,16] due to a higher incidence
61
of severe pericarditis with cardiac tamponade and of myocarditis associated with congestive heart failure, both being conditions indicative of a poor prognosis. Use of corticosteroids for pericarditis is controversial. According to Lietman and Bywaters [4], patients with mild pericarditis could be successfully treated with nonsteroidal anti-inflammatory drugs in appropriate dosage. However, like Svantesson et al. [31 we believe that corticosteroids are the drug of choice even for patients with mild manifestations in order to prevent cardiac tamponade, constriction, or even myocarditis. Myocarditis requires appropriate doses of corticosteroids for at least 3 months, due to its high potential risk of death and for chronic cardiomyopathy [17]. Low doses of digitalis were employed in 4 patients presenting with congestive heart failure without toxicity. Nevertheless, others 117,281do not recommend the use of digitalis due to a greater susceptibility to toxic effects. Pericardiocentesis was performed in the majority of patients with cardiac tamponade reported [6-141, one of whom [12] required additional pericardiectomy. The intrapericardial injection of methylprednisolone was reported by Scharf et al. [8] with satisfactory results. Finally, the report suggests that cardiac involvement, particularly myocarditis and cardiac tamponade, should be regarded as factors of worse prognosis. References Still GF. On a form of chronic joint disease in children. Med Chir Tram 1897;80:87-90. Brewer EJ. Juvenile rheumatoid arthritis: cardiac involvement. Arthritis Rheum 1977; 20 (suppl 2): 231-236. Svantesson H. Bjorkhem G, Elborgh R. Cardiac involvement in juvenile rheumatoid arthritis. Acta Paediatr Stand 1983;72:345-350. Lietman PS, Bywaters EGL. Pericarditis in juvenile rheumatoid arthritis. Pediatrics 1963;32:855-860. Schaller J, Wedgewood RJ. Juvenile rheumatoid arthritis: a review. Pediatrics 1972;50;940-953. Saraiva LR, Lira Y, Vilachan R, Wanderley JE, Souza SG, Lima R. Artrite reumatoide juvenil e pericardite constrictiva corn derrame. Arq Bras Cardiol 1983;47:137-142.
9 10
11
12
13 14
15
I6
17 18
19 20 21 22
23
24
25
Bauer-Vinassac D, Chapsal J, Duboc D, Menkes CJ, Guerin F, Simon, F. Insuffisance aortique et pericardite rapidement constrictive au tours d’une polyarthrite rhumatoid B debut juvenile. A propos d’une observation et revue de la litterature. Ann Med Interne (Paris) 1987;138: 141-142. Scharf J, Levy J, Benderly A, Nahir M. Pericardial tamponade in juvenile rheumatoid arthritis. Arthritis Rheum 1976;19:760-762. Majeed HA, Kvasnicka J. Juvenile rheumatoid arthrttis with cardiac tamponade. Ann Rheum Dis 197837273-276. Yancey CL, Doughty RA, Cohlan BA, Athreya BH. Pericarditis and cardiac tamponade in juvenile rheumatoid arthritis, Pediatrics 1981;68:369-373. Kahan A, Feldmann JL, Menkes CJ, Fontaine JL. Polyarthrite juvenile compliquee de tamponade recidivante: traitement par pericardotomie et corticoides a forte dose. Ann Med Interne (Paris) 1983;134:337-338. Alukal MK, Costello PB, Green FA. Cardiac tamponade in systemic juvenile rheumatoid arthritis requiring emergency pericardiectomy. J Rheumatol 1984;11:222-225. Nadas AS, Levy JM. Pericarditis in children. Am J Cardiol 1961;7:109-117. Goldenberg J, Pessoa AP, Roizemblatt S. Povoa RMS, Hilirio MO, Atra E, Ferraz MB. Cardiac tamponade in juvenile chronic arthritis: report of two cases and review of publications. Ann Rheum Dis 1990;49:549-553. Calabro, JJ. Other extraarticular manifestations of juvenile rheumatoid arthritis. Arthritis Rheum 1977;2o(suppl 2):253-255. Bernstein B, Takahashi M, Hanson V. Cardiac involvement in juvenile rheumatoid arthritis. J Pediatr 1974;85: 313-317. Miller III JJ, French JW. Myocarditis in juvenile rheumatoid arthritis. Am J Dis Child 1977;133:205-209. Cabazuelo Huerta G, Jimenez-Busselo MT, Fronteira Isquierdo P. Miocarditis en la artritis rheumatoidea juvenil. An Esp Pediatr 1986;24:134-136. Bywaters EGL. Death in juvenile chronic polyarthritis. Arthritis Rheum 1977;2O(suppl 2k256. Bernstein B. Death in juvenile rheumatoid arthritis. Arthritis Rheum 1977;2O(suppl 2):256-257. Baum J, Gutowska G. Death in juvenile rheumatoid arthritis. Arthritis Rheum 1977;2Otsuppl 2):253-255. Leak AM, Millar-Craig MW, Ansell BM. Aortic regurgitation in seropositive juvenile arthritis. Ann Rheum Dis 1981;40:229-234, Delgado EA, Petty RE, Malleson PN. Patterson MW, D’Orsogna L, Le Blanc J. Aortic valve insufficiency and coronary artery narrowing in a child with polyarticular juvenile rheumatoid arthritis. J Rheumatol 1988;15:144147. Kean WF, Anastassiades TP, Ford PM. Aortic incompetence in HLA B27-positive juvenile arthritis. Ann Rheum Dis 1980;39:294-295. Cassidy JT, Levison JE, Bass JC et al. A study of classifi-
62 cation criteria for a diagnosis of juvenile rheumatoid arthritis. Arthritis Rheum 1986;29:274-281. 26 Lindsley CB, Mattioli L. Pericarditis: a initial manifestation of juvenile rheumatoid arthritis. J Kans Med Sot 1979;80:119-121.
27 Clayer GG, Taybi H, Riley HD, Simon JL. Pericarditis with effusion in infants and children J. Pediatr 1970;63: 265-272. 28 Calabro JJ. Myocarditis in juvenile rheumatoid arthritis. Am J Dis Child 1977;131:1306.
CARD10
57 0167-5273/92/$05.00
01371
Symptomatic cardiac involvement in juvenile rheumatoid
arthritis
Jo& Goldenberg,
Marcos B. Ferraz, Angela P. Pessoa, Antonio S.M. Fonseca, Antonio C. Carvalho, Maria 0. Hilario and Edgard Atra
Dirlision of Curdiofogy and Rheumatology, Department of Medicine and Diuision of Allergy, Immunology and Rheumatology, Department of Pediatrics, Escola Paul&a de Medicina, Sco Paula, Brazil (Received
2 April 1991; revision
accepted
22 July 1991)
Goldenberg J, Ferraz MB, Pessoa AP, Fonseca ASM, Carvalho AC, Hilario MO, Atra E. Symptomatic cardiac involvement in juvenile rheumatoid arthritis. Int J Cardiol 1992;34:57-62. In a retrospective study of 172 patients with juvenile rheumatoid arthritis, symptomatic cardiac involvement occurred in 13 (7.6%) patients (11 systemic and 2 polyarticular). There was predominance of the male sex and in most patients the involvement occurred in the initial years of the disease. Pericarditis occurred in seven patients; perimyocarditis in four and myocarditis in two patients. In the follow-up, one of the patients with pericarditis died of an arrhythmia during pericardiocentesis for cardiac tamponade. Among the patients with myocarditis, three died of septicemia during active disease. One of these three patients had myocarditis associated with cardiac tamponade. Among the 172 patients with juvenile rheumatoid arthritis, five children died; four belonged to the symptomatic cardiac involvement group (P < 0.001). Cardiac involvement, in particular myocarditis and cardiac tamponade, can he regarded as a factor of worse prognosis. Key words: Juvenile rheumatoid
arthritis; Myocarditis; Pericarditis; Cardiac involvement
Introduction The cardiac involvement in juvenile rheumatoid arthritis was first described by Still in 1897 [Il. Among the heart layers the pericardium is the most frequently affected one followed by myocardium, and less frequently parietal and valvar endocardium [2,3]. Cardiac involvement is cliniCorrespondence to: Dr. JosC Goldenberg, M.D., PhD., Escola Paulista de Medicina, Disciplina de Reumatologia, Rua Botucatti 740, Vila Clementino, CEP 04023, S%o Paula, Brazil.
cally evident in 3-9% of patients with juvenile rheumatoid arthritis, especially in its systemic form [2-51. Pericarditis is usually benign. In some cases, however, it evolves to constriction [6,7] and/or cardiac tamponade [8-141 and is occasionally associated with pleuritis as part of a polyserositis [3,4,151. Echocardiographic studies in juvenile rheumatoid arthritis patients detected pericarditis in as much as 36% of the cases [16]. This finding is consistent with the incidence reported in autopsy studies, which showed pericardial involvement in 45% of the cases [4].
58
cardiography could be performed in 10 of the 13 selected cases. Pericarditis was diagnosed when any two of the following were observed: pericardial friction rub; enlargement of cardiac silhouette; ventricular repolarization changes; decreased amplitude of QRS complexes; evidence of pericardial effusion or thickening. The following criteria were used to diagnose myocarditis: presence of third heart sound or evidence of congestive heart failure in the absence of pericardial effusion or constriction, sysor endocarditis; chamber temic hypertension, overloador evidence of myocardial damage detectedby electrocardiography; enlargement of cardiac chambers or reduced myocardial contractility detected by echocardiography.
Myocarditis, associated or not with pericarditis, produces more expressive clinical manifestations [17,18]. It can lead to congestive heart failure and arrhythmias, causing death in juvenile rheumatoid arthritis patients [ 19-211. Although not a frequent manifestation, mitral and aortic valves can be affected, especially in patients with a severe aggressive arthritis associated with high titers of rheumatoid factor [22,231 and in its pauciarticular form with HLA-B27 positivity [24]. The present study is a retrospective analysis of 172 patients with juvenile rheumatoid arthritis evaluated by us in a 16-year period.
Materials and Methods Results
The study included 172 patients with juvenile rheumatoid arthritis, classified according to American Rheumatism Association criteria (1986) [25], selected from the Pediatrics Rheumatology Division of Escola Paulista de Medicina from 1972 to 1988. The demographic characteristics of the patients are shown in Table 1. Thirteen cases presented clinical evidence of cardiac involvement during an exacerbation in the course of disease. The historical data, clinical features, radiological, electrocardiographic and echocardiographic findings were analysed. Echo-
The heart was affected during the course of juvenile rheumatoid arthritis in 13 patients (7.6%), of whom 11 presented with the systemic form, and two with the polyarticular form. In the 13 children with detectable cardiac involvement none had a recent infection. Male to female ratio was 8:5. Nine patients were Caucasian and 4 non-Caucasian. The age at the onset of disease ranged from 2 years and 3 months to 14 years and 1 month (mean: 5 years and 5 months); cardiac
TABLE 1 Demographic characteristics of juvenile rheumatoid arthritis (JRA) patients Type of JRA
Race
Sex M
F
C
NC
Systemic (n = 57)
28
29
47
10
Polyarticular (?I = 57)
16
41
45
12
Pauciarticular (n = 58)
27
31
47
11
Age at onset of disease range (mean)
Duration of the disease range
llmoto14yr and 11 mo (5 yr 4 mo) 6moto16yr and 5 mo (6 yr and 9 mo) 12 mo to 14 yr and 2 mo (7 yr and 1 mo)
2moto16yr
M = male; F = female; C = Caucasian; NC = non-Caucasian; yr = years; mo = months.
6 mo to 15 yr
9 mo to 18 yr
59
involvement was diagnosed in patients aged from 2 years and 3 months to 14 years and 8 months (mean: 5 years and 10 months). In one case the cardiac involvement preceded articular involvement. In the majority of the patients, heart involvement was diagnosed within the first year of the disease. A total of 17 acute episodes were observed; 10 patients presented only one acute episode, and 3 patients recurring episodes. Isolated pericarditis, the most common cardiac involvement, was found in 5 patients with systemic juvenile rheumatoid arthritis and in 2 with the polyarticular form. Myopericarditis was found in 4 patients and isolated myocarditis in 2 patients, all with systemic juvenile rheumatoid arthritis. Clinical manifestations, radiological and graphic findings are presented in Tables 2 and 3. One of the patients with isolated pericarditis died due to ventricular tachycardia during pericardiocentesis for acute cardiac tamponade. The other 6 patients presented a good resolution of their pericarditis. As for articular involvement, 3 patients were in remission, and 3 presented with polyarticular involvement. Among the patients with myocardial involvement, recurrent episodes of cardiac involvement occurred in three. Acute cardiac tamponade was treated successfully in one case by pericardiec-
TABLE 2 Clinical manifestations involvement
in patients with symptomatic cardiac
Signs and symptoms
Isolated pericarditis (n=7)
Isolated myocarditis (n = 2) or perimyocarditis (n = 4)
Tachycardia Friction rub Dull heart sounds Tachypnea Dyspnea Congestive heart failure Cardiac tamponade Third heart sound Systolic murmur
7 4 2 2 1 1 1
6 2 1
_
6 5 1 5 2
TABLE 3 Radiological, electrocardiographic, and echocardiographic findings observed in juvenile rheumatoid arthritis patients with symptomatic cardiac involvement (n = 13) Chest X-rays Enlarged cardiac silhouette Pleural effusion Evidence of pulmonary congestion Electrocardiography Repolarization abnormalities Chamber overload Subepicardial lesion Echocardiography * Pericardial effusion Enlargement of cardiac chambers Decreased myocardial contractility
9 6 5 8 3 1
8 4 2
* Three patients were not submitted to echocardiography.
tomy. Three patients died due to sepsis during a flare-up of the disease. Of the 172 juvenile rheumatoid arthritis cases studied, five patients died, of whom 4 presented with cardiac involvement which seems to be a factor of poor prognosis (P < 0.0001). Juvenile rheumatoid arthritis began earlier in deceased patients (mean age: 2 years and 7 months), as compared with survivors (mean age: 6 years and 8 months). Death occurred usually within the first year of disease (3 out of 4) especially in patients presenting with myocardial involvement and/or cardiac tamponade. The drug of choice for therapeutic regimen was prednisone (1 mg/kg/day) for at least 3 months. Low doses of digitalis were administered to 4 patients with congestive heart failure. For patients presenting with cardiac tamponade, pericardiocentesis was performed in one and pericardiectomy in the other. Discussion The incidence of clinically evident heart involvement in our patients was 7.6%, a finding similar to those reported in the literature [2-51. Eighty five percent of our patients with symptomatic cardiac involvement suffered from systemic juvenile rheumatoid arthritis, data similar to that reported in the literature [2,5,16,17]. Only
60
two of our patients presented with the polyarticular form. No significant difference was observed in the age of patients with or without cardiac involvement (5 years and 5 months and 5 years and 4 months respectively). The finding is consistent with the literature [3,4,16]. Our observations indicate that the most severe cases were among patients who presented with the disease before the age of four years since the disease started in all the fatal cases by this age. The interval between the diagnosis of articular manifestations and cardiac involvement was less than 3 years and 4 months and the cardiac involvement occurred within the first year of disease in the majority of cases. Isolated pericarditis was the most frequent cardiac manifestation and was found in 54% of symptomatic cases. A relatively high incidence of myocardial involvement, with or without pericarditis, was observed in 3.5% of all juvenile rheumatoid arthritis patients and 46% of symptomatic cases. The incidence was higher than that described in the literature, ranging from 0.5 to 1.3% of all juvenile rheumatoid arthritis patients
KU71. Clinical manifestations of pericarditis were mild. Tachycardia and friction rub were the most frequent findings, folIowed by dyspnea and cardiac tamponade in one patient. The findings are in agreement with those of other authors [3,4,26]. A careful clinical evaluation, as well as additional tests, are needed for early diagnosis of pericarditis. Differential diagnosis between rheumatoid pericarditis and viral pericarditis can be difficult. Viral pericarditis frequently follows an upper respiratory infection with a short latent period, and acute onset [27]. Other entities associated with pericarditis in children include rheumatic fever, bacterial infection, and, less frequently, benign idiopathic pericarditis [ 131. In contrast, myocardial involvement produced florid clinical manifestations, including tachypnea and dyspnea in all patients. A systolic murmur was found in 33% of our patients and has been described by others [18] as a clinical finding during the active phase of disease. It is due to
hyperkinesia and/or widening of the fibrous ring, secondary to enlargement of cardiac chambers, seeming to be indicative of organic valvar damage. This murmur tends to disappear with clinical management of myocarditis. Congestive heart failure was found in 5 cases (83%) and was indicative of severe cardiac involvement. Chest radiography and electrocardiography are useful for indicating cardiac involvement as reported by other authors [2-41. However, they have a limited value since cardiac enlargement and repolarization changes are nonspecific and found in the presence of pericarditis as well as myocarditis [3,4,17,18]. Echocardiography is a more accurate method for detecting cardiac lesions due to its high specificity and sensitivity. Echocardiograms may reveal abnormal findings in patients presenting with few symptoms and normal chest radiography or electrocardiography. Echocardiography associated with clinical findings plays an important role in the detection of pericardial, myocardial, and endocardial involvement. The course of isolated pericarditis was benign, except in one case who developed cardiac tamponade, and died during pericardiocentesis, due to ventricular tachycardia. The usually benign course of pericarditis has been reported in the Iiterature [3,4,26]. On the other hand, myocardial involvement had a more severe course, especially in those patients with congestive heart failure, three of whom died. The other three cases were in remission after a follow-up ranging from 4 months to 8 years, with one still on treatment with corticosteroids. Considering the influence of cardiac involvement, especially myocarditis and cardiac tamponade, on the mortality observed by us, we advocate that all juvenile rheumatoid arthritis patients, especially those with florid systemic manifestations, should be submitted to electrocardiography and echocardiogram, in order that the cardiac involvement can be detected early and treated, and more severe disease is prevented. The mortality and symptomatic cardiac involvement in our patients were higher than that reported earlier [3,4,16] due to a higher incidence
61
of severe pericarditis with cardiac tamponade and of myocarditis associated with congestive heart failure, both being conditions indicative of a poor prognosis. Use of corticosteroids for pericarditis is controversial. According to Lietman and Bywaters [4], patients with mild pericarditis could be successfully treated with nonsteroidal anti-inflammatory drugs in appropriate dosage. However, like Svantesson et al. [31 we believe that corticosteroids are the drug of choice even for patients with mild manifestations in order to prevent cardiac tamponade, constriction, or even myocarditis. Myocarditis requires appropriate doses of corticosteroids for at least 3 months, due to its high potential risk of death and for chronic cardiomyopathy [17]. Low doses of digitalis were employed in 4 patients presenting with congestive heart failure without toxicity. Nevertheless, others 117,281do not recommend the use of digitalis due to a greater susceptibility to toxic effects. Pericardiocentesis was performed in the majority of patients with cardiac tamponade reported [6-141, one of whom [12] required additional pericardiectomy. The intrapericardial injection of methylprednisolone was reported by Scharf et al. [8] with satisfactory results. Finally, the report suggests that cardiac involvement, particularly myocarditis and cardiac tamponade, should be regarded as factors of worse prognosis. References Still GF. On a form of chronic joint disease in children. Med Chir Tram 1897;80:87-90. Brewer EJ. Juvenile rheumatoid arthritis: cardiac involvement. Arthritis Rheum 1977; 20 (suppl 2): 231-236. Svantesson H. Bjorkhem G, Elborgh R. Cardiac involvement in juvenile rheumatoid arthritis. Acta Paediatr Stand 1983;72:345-350. Lietman PS, Bywaters EGL. Pericarditis in juvenile rheumatoid arthritis. Pediatrics 1963;32:855-860. Schaller J, Wedgewood RJ. Juvenile rheumatoid arthritis: a review. Pediatrics 1972;50;940-953. Saraiva LR, Lira Y, Vilachan R, Wanderley JE, Souza SG, Lima R. Artrite reumatoide juvenil e pericardite constrictiva corn derrame. Arq Bras Cardiol 1983;47:137-142.
9 10
11
12
13 14
15
I6
17 18
19 20 21 22
23
24
25
Bauer-Vinassac D, Chapsal J, Duboc D, Menkes CJ, Guerin F, Simon, F. Insuffisance aortique et pericardite rapidement constrictive au tours d’une polyarthrite rhumatoid B debut juvenile. A propos d’une observation et revue de la litterature. Ann Med Interne (Paris) 1987;138: 141-142. Scharf J, Levy J, Benderly A, Nahir M. Pericardial tamponade in juvenile rheumatoid arthritis. Arthritis Rheum 1976;19:760-762. Majeed HA, Kvasnicka J. Juvenile rheumatoid arthrttis with cardiac tamponade. Ann Rheum Dis 197837273-276. Yancey CL, Doughty RA, Cohlan BA, Athreya BH. Pericarditis and cardiac tamponade in juvenile rheumatoid arthritis, Pediatrics 1981;68:369-373. Kahan A, Feldmann JL, Menkes CJ, Fontaine JL. Polyarthrite juvenile compliquee de tamponade recidivante: traitement par pericardotomie et corticoides a forte dose. Ann Med Interne (Paris) 1983;134:337-338. Alukal MK, Costello PB, Green FA. Cardiac tamponade in systemic juvenile rheumatoid arthritis requiring emergency pericardiectomy. J Rheumatol 1984;11:222-225. Nadas AS, Levy JM. Pericarditis in children. Am J Cardiol 1961;7:109-117. Goldenberg J, Pessoa AP, Roizemblatt S. Povoa RMS, Hilirio MO, Atra E, Ferraz MB. Cardiac tamponade in juvenile chronic arthritis: report of two cases and review of publications. Ann Rheum Dis 1990;49:549-553. Calabro, JJ. Other extraarticular manifestations of juvenile rheumatoid arthritis. Arthritis Rheum 1977;2o(suppl 2):253-255. Bernstein B, Takahashi M, Hanson V. Cardiac involvement in juvenile rheumatoid arthritis. J Pediatr 1974;85: 313-317. Miller III JJ, French JW. Myocarditis in juvenile rheumatoid arthritis. Am J Dis Child 1977;133:205-209. Cabazuelo Huerta G, Jimenez-Busselo MT, Fronteira Isquierdo P. Miocarditis en la artritis rheumatoidea juvenil. An Esp Pediatr 1986;24:134-136. Bywaters EGL. Death in juvenile chronic polyarthritis. Arthritis Rheum 1977;2O(suppl 2k256. Bernstein B. Death in juvenile rheumatoid arthritis. Arthritis Rheum 1977;2O(suppl 2):256-257. Baum J, Gutowska G. Death in juvenile rheumatoid arthritis. Arthritis Rheum 1977;2Otsuppl 2):253-255. Leak AM, Millar-Craig MW, Ansell BM. Aortic regurgitation in seropositive juvenile arthritis. Ann Rheum Dis 1981;40:229-234, Delgado EA, Petty RE, Malleson PN. Patterson MW, D’Orsogna L, Le Blanc J. Aortic valve insufficiency and coronary artery narrowing in a child with polyarticular juvenile rheumatoid arthritis. J Rheumatol 1988;15:144147. Kean WF, Anastassiades TP, Ford PM. Aortic incompetence in HLA B27-positive juvenile arthritis. Ann Rheum Dis 1980;39:294-295. Cassidy JT, Levison JE, Bass JC et al. A study of classifi-
62 cation criteria for a diagnosis of juvenile rheumatoid arthritis. Arthritis Rheum 1986;29:274-281. 26 Lindsley CB, Mattioli L. Pericarditis: a initial manifestation of juvenile rheumatoid arthritis. J Kans Med Sot 1979;80:119-121.
27 Clayer GG, Taybi H, Riley HD, Simon JL. Pericarditis with effusion in infants and children J. Pediatr 1970;63: 265-272. 28 Calabro JJ. Myocarditis in juvenile rheumatoid arthritis. Am J Dis Child 1977;131:1306.
