Scand J Rheumatology 7: 203-208, 1978
CARDIAC INVOLVEMENT IN POLYMYOSITIS Martti Oka and Tapio Raasakka
Scand J Rheumatol Downloaded from informahealthcare.com by University of British Columbia on 11/18/14 For personal use only.
From the Deparimeni of Medicine, Ceniral Nospiial, Jyvaskyla, Finland
ABSTRACT. Sixteen cases of polymyositis (PM) were treated at the Central Hospital, Jyvaskyla. Features of cardiac involvement were observed in 11 cases (69%). The heart diseases which developed or worsened after the onset of PM were grouped as follows: (1) Sole disturbance of conduction--one case, (2) congestive heart failurefour cases, (3) coronary heart disease with or without congestive heart failur-six cases. Three patients suffered an acute myocardial infarction. Electrocardiography revealed arrhythmias in eight cases and disturbances of conduction in three. In one patient complete atrioventricular block and congestive heart failure necessitated installation of an intracardiac pacemaker. In four patients congestive heart failure progressed to death. In two autopsied cases changes suggestive of PM were found in the heart muscle. Involvement of the heart in PM was prognostically a bad sign.
Polymyositis is said rarely to involve the myocardium. Isolated cases of heart disease associated with PM have been reported by several authors (1-5). Walton & Adams (6) described T-wave abnormality of the electrocardiogram (ECG) in four cases out of 40. Sharratt and coworkers (7) found ECG abnormalities in five out of 13 cases of PM. The ECG usually showed arrhythmias and disturbances of conduction. One of the patients had severe left ventricular disease. Reza and co-workers (8) found four patients in whom cardiac disease was a major feature. They also reviewed the records of 110 other patients and found abnormal ECG in 82; three of their patients died of cardiac disease. We report the cardiac findings in our PM patients and the effects of the heart disease on the prognosis of PM.
PATIENTS AND METHODS The series comprised 16 patients--seven males and nine females. At the onset of the disease their ages had ranged from 13 to 72 years (av. 49). The duration of the disease varied from one month to 15 years (av. 4.7). The patients reflect the incidence of PM in a population of 240 OOO
during the 12-year period 1966-77. The incidence of PM was thus 0.55 per 100 000 inhabitants The series included 5 patients with dermatomyositis. In two patients, rheumatoid arthritis had been diagnosed many years before the onset of PM. One patient in the terminal stage of PM had signs fulfilling the criteria of systemic lupus erythematosus (SLE). In three patients Raynaud's syndrome was observed. In one case Pm was associated with bronchial cancer metastasizing to various organs. The diagnostic criteria of Peter & Bohan (9-10) were used. They were as follows: (1) Symmetrical weakness of the limb-girdle muscles and anterior neck flexors, (2) typical muscle biopsy finding, (3) elevation of skeletal-muscle enzymes in serum, (4) typical electromyographic (EMG) finding, (5) typical dermatological features. The diagnosis was considered according to the number of criteria fulfilled, as definite (4) in l l , probable (3) in three and possible (2) in two patients (Table I). A11 the patients showed proximal muscle weakness. In 15 cases, muscle biopsy findings were positive, and in one case the muscle was normal. EMG revealed typical changes in eight out of 11 cases; the study was not performed in five cases. Serum skeletal-muscle enzymes were elevated in 13 cases. In six patients the onset of the disease was acute and in the others chronic. The disease started with flu-like symptoms in two cases, with a massive eosinophilic reaction in one case, with Raynaud's syndrome in two cases, and after parturition in one case. Apart from involvement of muscles, skin and heart, the following symptoms were recorded: synovitis or arthralgia in six cases; pulmonary fibrosis in two cases; hepatomegaly, mild toxic hepatitis, lymphadenopathy, proteinuria and decreased renal function, papillary stasis, and paresis of the peroneus nerve, in one case each. The erythrocyte sedimentation rate ranged from 10 to 131 mm/h (av. 54). Mild anaemia was found in four patients; leukocytosis was observed in nine. The rheumatoid factor test (Waaler-Rose titre) was positive in six cases and the antinuclear antibody test (ANA) in four. In one case serum C3 concentration was decreased and in another case cryoprecipitation and the direct Coombs test were positive. As regards functional capacity, the patients were graded as follows: Grade I-normal, Grade 11-slight weakness, sufficient for normal activities in spite of handicaps, Grade 111-moderate to marked weakness allowing only light activities, Grade IV-profound weakness, patient bedridden. Scand J Rheurnotology 7
Table 1. P(itirt7r.c PM=nolvnlvo\iti\. DM =dermatomyositis. SLE= hystemic luptis eruthematous. RA=rheumatoid arthritih Age at
Case
( 1 .t
-
37 F 48 M
10
3
44 F
3
52 F 48 M 49 M h6 M MF
1112 4
1
7
Scand J Rheumatol Downloaded from informahealthcare.com by University of British Columbia on 11/18/14 For personal use only.
onset/ Sex
Duration
5 6
7 8 9 I0 11 I?
13 14
is
16
72 F 63M 67 F 14 F 78 F 39 M 13 F 57 M
I113
3 6 1/13 4
3 7 I/? 5 15 112 I4
4 4
Class of polymyositis
Cardiac involvement
Primary PM DM + SLE
I1 IV
Primary PM Primary DM Primary DM Primary PM PM + Neoplasm Phl + RA
IV IV
Primary PM Primary DM Primary PM Primary PM Primary PM Primary DM Primary PM PM + RA
RESULTS Feature\ ot cdidiac inkolvement were obqerved i n I 1 patient< (h9,qRS in lead V,. LAHB, VE. Firstdegree A-V block Pathological Q-wave in leads 11, 111, aVF. LVH, LAHB, VE. Paroxysmal atrial fibrillation
LVH=left ventricular hypertrophy. LAHB=left antenor haemiblock. LBBB=left bundle branch block. VE=ventncular extraryctole5
based on typical chest pain. abrupt elevation of serum enzymes and QRS changes in ECG. The infarction affected the anterior wall of the myocardium in one case (no. 10) and the inferior wall in two cases (nos. 6. 11). The other ECG abnormalities observed in this group were S-T segment depression, left ventricular hypertrophy pattern, left anterior haemiblock, first-degree atrioventricular block, extrasystoles and tachyarrhythmias. Prednisolone was used for the treatment of PM. In addition, azathioprine was tried in one case. The disease is in remission in one case
but active in three. As stated above. two fatalities occurred. Prognosis (Table V )
In three cases, remission was verified by muscle biopsy and EMG (nos. 9, 12, 14). The two patients in partial remission were clinically symptomless but control biopsy showed active changes in one case (no. 13) and EMG pointed to chronic myositis in the other (no. 15). The response to steroid treatment was striking in case 13 with normalization of muscle power and serum enzyme levels and improvement
Cardiuc involvement in polyrnyositis Table IV. ECG,findings
Conduction disturbances First-degree AV block Complete AV block Arrhythmias
Scand J Rheumatol Downloaded from informahealthcare.com by University of British Columbia on 11/18/14 For personal use only.
Table V. Prognosis No. of cases
Abnormality
Ventricular extrasystoles Supraventricular extrasystoles Paroxysmal supraventricular tachycardia Paroxysmal atrial fibrillation Left ventricular hypertrophy pattern S-T segment depression (normal R voltage) Left anterior haemiblock Left bundle branch block Acute infarction pattern (QRS changes)
207
3 3 1
Remission Partial remission Persistent activity Fatal outcome
Cardiac
Patients without cardiac
patients
involvement
1 1
2 2 1 0
4 5
I 7 3 4
I 3
in EMG. After 7 months of treatment the patient has returned to work. Partial, periodical remissions were also observed in five cardiac patients (nos. 1, 3, 5, 6, 8) but all had relapses. Prednisolone was administered to all of them, azathioprine to two. One patient (no. 1) is still in clinical remission with normal findings in the control biopsy and EMG but at the last check-up the serum creatine phosphokinase level showed a sharp rise, indicating impending relapse. Therapeutically, azathioprine has been a failure in our PM patients but steroids seemed to induce partial or sometimes full remission in some of them. The prognosis was especially poor in the second group of cardiac patients with congestive heart failure. The prognosis for the patients without cardiac involvement (nos. 12-16) seemed to be better, and they were clearly younger than those with cardiac involvement. No fatalities occurred in this group. DISCUSSION
In the county of Keski-Suomi (Central Finland) the incidence of PM was 0.55 per 100000 inhabitants. This figure is reliable, as all patients with PM are remitted to the Central Hospital, Jyvaskyla. Of the 16 patients five were classified as dermatomyositis. An associated connective tissue disease was diagnosed in three cases: rheumatoid arthritis in two and SLE in one case. Rheumatoid arthritis was in a chronic, inactive stage at the onset of PM but the symptoms of SLE appeared in the terminal stage. Most of the patients (69%) showed features of
cardiac involvement. They were grouped according to the types of heart disease complicating PM. The first group comprised one patient with a disturbance of conduction. The second group consisted of four middle-aged patients with previously normal hearts and normal blood pressure in whom congestive heart failure developed. In three, the course was fatal with gross cardiomegaly, mitral insufficiency and signs of fluid retention. In one case, progressive heart failure and complete atrioventricular block necessitated installation of an intracardiac pacemaker. As far as we know this is the first reported case of polymyositic heart disease treated with an intracardiac pacemaker. The left heart was particularly involved in this group of patients which represented the pure type of “polymyositic congestive cardiomyopathy” . The third group consisted of six patients with symptoms and signs of coronary insufficiency. In five of them the symptoms only appeared after the onset of PM. Three patients suffered an acute myocardial infarction. Symptoms of congestive heart failure were observed in four cases. The ECG abnormalities observed in this group were ischaemic S-T segment changes, pathognomonic QRS changes, left ventricular hypertrophy pattern, disturbances of conduction and arrhythmias. Two fatalities occurred. Autopsy revealed-in addition to coronary sclerosis and scars of infarction-changes suggesting involvement of the heart muscle by PM. At microscopy, sections of heart muscle showed hyalinized scar tissue, but also disintegration of muscle fibres and infiltrates of lymphoid and plasma cells. In patients with manifest or latent coronary heart disease PM obviously entails a serious risk. The patients of the third group were all aged, which makes it probable that they had latent coronary insufficiency which was aggravated by PM. Prognosis was poor in the patients with cardiac
208
M . Oku and T . Raasukka
involvement as compared with those without cardiac disease. Steroid therapy was beneficial in several cases, but a therapeutic trial with azathioprine proved t o be a failure.
Scand J Rheumatol Downloaded from informahealthcare.com by University of British Columbia on 11/18/14 For personal use only.
REFERENCES I . Goldfischer, J. & Rubin, E. H.: Dermatomyositis with pulmonary lesions. Ann Intern Med 50: 194. 1959. 2. Concha, E.. Jimenez, E., Silva, L.. & Losada, M.: Derrnatornyositis-Report of 9 cases. Arch lnteramer Rheum5:234, I%?. 3. Hill, D. L. & Barrows, H. S.: Identical skeletal and cardiac muscle involvement in a case of Fatal polymyositis. Arch Neurol 19: 545, 1968. 4. Lynch, P. (3.: Cardiac involvement in chronic polymyositis. Br Heart J33:416, 1971. 5. Schaumburg, H. H., Nielsen, S . L. & Yurchak, P. M.: Heart block in polymyositis. New Engl J Med 284:480. 1971.
6. Walton, J. N. & Adams. R. D.: Polymyositis. p. 157. E. & S. Livingstone, London, 1958. 7. Sharratt, G. P., Danta, G . & Carson, P. H. M.: Cardiac abnormality in polymyositis. Ann Rheum Dis 36: 575. 1977. 8. Reza, M. J., Goldberg, L. S . & Pearson, C. M.: Cardiac involvement in polymyositis. Abstracts. XIV International Congresb of Rheumatology, San Francisco, 1977, No. 86, p. 40. 9. Bohan, A. & Peter, J. B.: Polymyositis and dermatomyositis (First of two parts). New Engl J Med 292: 344. 1975. 10. Bohan, A. & Peter, J . B.: Polymyositis and dermatornyositis (Second of two parts). New Engl J Med 292: 403, 1975. Submitted for publication May 5 . 1978
Martti Oka Department of Medicine Central Hospital 40620 Jyvaskyla 62 Finland
CARDIAC INVOLVEMENT IN POLYMYOSITIS Martti Oka and Tapio Raasakka
Scand J Rheumatol Downloaded from informahealthcare.com by University of British Columbia on 11/18/14 For personal use only.
From the Deparimeni of Medicine, Ceniral Nospiial, Jyvaskyla, Finland
ABSTRACT. Sixteen cases of polymyositis (PM) were treated at the Central Hospital, Jyvaskyla. Features of cardiac involvement were observed in 11 cases (69%). The heart diseases which developed or worsened after the onset of PM were grouped as follows: (1) Sole disturbance of conduction--one case, (2) congestive heart failurefour cases, (3) coronary heart disease with or without congestive heart failur-six cases. Three patients suffered an acute myocardial infarction. Electrocardiography revealed arrhythmias in eight cases and disturbances of conduction in three. In one patient complete atrioventricular block and congestive heart failure necessitated installation of an intracardiac pacemaker. In four patients congestive heart failure progressed to death. In two autopsied cases changes suggestive of PM were found in the heart muscle. Involvement of the heart in PM was prognostically a bad sign.
Polymyositis is said rarely to involve the myocardium. Isolated cases of heart disease associated with PM have been reported by several authors (1-5). Walton & Adams (6) described T-wave abnormality of the electrocardiogram (ECG) in four cases out of 40. Sharratt and coworkers (7) found ECG abnormalities in five out of 13 cases of PM. The ECG usually showed arrhythmias and disturbances of conduction. One of the patients had severe left ventricular disease. Reza and co-workers (8) found four patients in whom cardiac disease was a major feature. They also reviewed the records of 110 other patients and found abnormal ECG in 82; three of their patients died of cardiac disease. We report the cardiac findings in our PM patients and the effects of the heart disease on the prognosis of PM.
PATIENTS AND METHODS The series comprised 16 patients--seven males and nine females. At the onset of the disease their ages had ranged from 13 to 72 years (av. 49). The duration of the disease varied from one month to 15 years (av. 4.7). The patients reflect the incidence of PM in a population of 240 OOO
during the 12-year period 1966-77. The incidence of PM was thus 0.55 per 100 000 inhabitants The series included 5 patients with dermatomyositis. In two patients, rheumatoid arthritis had been diagnosed many years before the onset of PM. One patient in the terminal stage of PM had signs fulfilling the criteria of systemic lupus erythematosus (SLE). In three patients Raynaud's syndrome was observed. In one case Pm was associated with bronchial cancer metastasizing to various organs. The diagnostic criteria of Peter & Bohan (9-10) were used. They were as follows: (1) Symmetrical weakness of the limb-girdle muscles and anterior neck flexors, (2) typical muscle biopsy finding, (3) elevation of skeletal-muscle enzymes in serum, (4) typical electromyographic (EMG) finding, (5) typical dermatological features. The diagnosis was considered according to the number of criteria fulfilled, as definite (4) in l l , probable (3) in three and possible (2) in two patients (Table I). A11 the patients showed proximal muscle weakness. In 15 cases, muscle biopsy findings were positive, and in one case the muscle was normal. EMG revealed typical changes in eight out of 11 cases; the study was not performed in five cases. Serum skeletal-muscle enzymes were elevated in 13 cases. In six patients the onset of the disease was acute and in the others chronic. The disease started with flu-like symptoms in two cases, with a massive eosinophilic reaction in one case, with Raynaud's syndrome in two cases, and after parturition in one case. Apart from involvement of muscles, skin and heart, the following symptoms were recorded: synovitis or arthralgia in six cases; pulmonary fibrosis in two cases; hepatomegaly, mild toxic hepatitis, lymphadenopathy, proteinuria and decreased renal function, papillary stasis, and paresis of the peroneus nerve, in one case each. The erythrocyte sedimentation rate ranged from 10 to 131 mm/h (av. 54). Mild anaemia was found in four patients; leukocytosis was observed in nine. The rheumatoid factor test (Waaler-Rose titre) was positive in six cases and the antinuclear antibody test (ANA) in four. In one case serum C3 concentration was decreased and in another case cryoprecipitation and the direct Coombs test were positive. As regards functional capacity, the patients were graded as follows: Grade I-normal, Grade 11-slight weakness, sufficient for normal activities in spite of handicaps, Grade 111-moderate to marked weakness allowing only light activities, Grade IV-profound weakness, patient bedridden. Scand J Rheurnotology 7
Table 1. P(itirt7r.c PM=nolvnlvo\iti\. DM =dermatomyositis. SLE= hystemic luptis eruthematous. RA=rheumatoid arthritih Age at
Case
( 1 .t
-
37 F 48 M
10
3
44 F
3
52 F 48 M 49 M h6 M MF
1112 4
1
7
Scand J Rheumatol Downloaded from informahealthcare.com by University of British Columbia on 11/18/14 For personal use only.
onset/ Sex
Duration
5 6
7 8 9 I0 11 I?
13 14
is
16
72 F 63M 67 F 14 F 78 F 39 M 13 F 57 M
I113
3 6 1/13 4
3 7 I/? 5 15 112 I4
4 4
Class of polymyositis
Cardiac involvement
Primary PM DM + SLE
I1 IV
Primary PM Primary DM Primary DM Primary PM PM + Neoplasm Phl + RA
IV IV
Primary PM Primary DM Primary PM Primary PM Primary PM Primary DM Primary PM PM + RA
RESULTS Feature\ ot cdidiac inkolvement were obqerved i n I 1 patient< (h9,qRS in lead V,. LAHB, VE. Firstdegree A-V block Pathological Q-wave in leads 11, 111, aVF. LVH, LAHB, VE. Paroxysmal atrial fibrillation
LVH=left ventricular hypertrophy. LAHB=left antenor haemiblock. LBBB=left bundle branch block. VE=ventncular extraryctole5
based on typical chest pain. abrupt elevation of serum enzymes and QRS changes in ECG. The infarction affected the anterior wall of the myocardium in one case (no. 10) and the inferior wall in two cases (nos. 6. 11). The other ECG abnormalities observed in this group were S-T segment depression, left ventricular hypertrophy pattern, left anterior haemiblock, first-degree atrioventricular block, extrasystoles and tachyarrhythmias. Prednisolone was used for the treatment of PM. In addition, azathioprine was tried in one case. The disease is in remission in one case
but active in three. As stated above. two fatalities occurred. Prognosis (Table V )
In three cases, remission was verified by muscle biopsy and EMG (nos. 9, 12, 14). The two patients in partial remission were clinically symptomless but control biopsy showed active changes in one case (no. 13) and EMG pointed to chronic myositis in the other (no. 15). The response to steroid treatment was striking in case 13 with normalization of muscle power and serum enzyme levels and improvement
Cardiuc involvement in polyrnyositis Table IV. ECG,findings
Conduction disturbances First-degree AV block Complete AV block Arrhythmias
Scand J Rheumatol Downloaded from informahealthcare.com by University of British Columbia on 11/18/14 For personal use only.
Table V. Prognosis No. of cases
Abnormality
Ventricular extrasystoles Supraventricular extrasystoles Paroxysmal supraventricular tachycardia Paroxysmal atrial fibrillation Left ventricular hypertrophy pattern S-T segment depression (normal R voltage) Left anterior haemiblock Left bundle branch block Acute infarction pattern (QRS changes)
207
3 3 1
Remission Partial remission Persistent activity Fatal outcome
Cardiac
Patients without cardiac
patients
involvement
1 1
2 2 1 0
4 5
I 7 3 4
I 3
in EMG. After 7 months of treatment the patient has returned to work. Partial, periodical remissions were also observed in five cardiac patients (nos. 1, 3, 5, 6, 8) but all had relapses. Prednisolone was administered to all of them, azathioprine to two. One patient (no. 1) is still in clinical remission with normal findings in the control biopsy and EMG but at the last check-up the serum creatine phosphokinase level showed a sharp rise, indicating impending relapse. Therapeutically, azathioprine has been a failure in our PM patients but steroids seemed to induce partial or sometimes full remission in some of them. The prognosis was especially poor in the second group of cardiac patients with congestive heart failure. The prognosis for the patients without cardiac involvement (nos. 12-16) seemed to be better, and they were clearly younger than those with cardiac involvement. No fatalities occurred in this group. DISCUSSION
In the county of Keski-Suomi (Central Finland) the incidence of PM was 0.55 per 100000 inhabitants. This figure is reliable, as all patients with PM are remitted to the Central Hospital, Jyvaskyla. Of the 16 patients five were classified as dermatomyositis. An associated connective tissue disease was diagnosed in three cases: rheumatoid arthritis in two and SLE in one case. Rheumatoid arthritis was in a chronic, inactive stage at the onset of PM but the symptoms of SLE appeared in the terminal stage. Most of the patients (69%) showed features of
cardiac involvement. They were grouped according to the types of heart disease complicating PM. The first group comprised one patient with a disturbance of conduction. The second group consisted of four middle-aged patients with previously normal hearts and normal blood pressure in whom congestive heart failure developed. In three, the course was fatal with gross cardiomegaly, mitral insufficiency and signs of fluid retention. In one case, progressive heart failure and complete atrioventricular block necessitated installation of an intracardiac pacemaker. As far as we know this is the first reported case of polymyositic heart disease treated with an intracardiac pacemaker. The left heart was particularly involved in this group of patients which represented the pure type of “polymyositic congestive cardiomyopathy” . The third group consisted of six patients with symptoms and signs of coronary insufficiency. In five of them the symptoms only appeared after the onset of PM. Three patients suffered an acute myocardial infarction. Symptoms of congestive heart failure were observed in four cases. The ECG abnormalities observed in this group were ischaemic S-T segment changes, pathognomonic QRS changes, left ventricular hypertrophy pattern, disturbances of conduction and arrhythmias. Two fatalities occurred. Autopsy revealed-in addition to coronary sclerosis and scars of infarction-changes suggesting involvement of the heart muscle by PM. At microscopy, sections of heart muscle showed hyalinized scar tissue, but also disintegration of muscle fibres and infiltrates of lymphoid and plasma cells. In patients with manifest or latent coronary heart disease PM obviously entails a serious risk. The patients of the third group were all aged, which makes it probable that they had latent coronary insufficiency which was aggravated by PM. Prognosis was poor in the patients with cardiac
208
M . Oku and T . Raasukka
involvement as compared with those without cardiac disease. Steroid therapy was beneficial in several cases, but a therapeutic trial with azathioprine proved t o be a failure.
Scand J Rheumatol Downloaded from informahealthcare.com by University of British Columbia on 11/18/14 For personal use only.
REFERENCES I . Goldfischer, J. & Rubin, E. H.: Dermatomyositis with pulmonary lesions. Ann Intern Med 50: 194. 1959. 2. Concha, E.. Jimenez, E., Silva, L.. & Losada, M.: Derrnatornyositis-Report of 9 cases. Arch lnteramer Rheum5:234, I%?. 3. Hill, D. L. & Barrows, H. S.: Identical skeletal and cardiac muscle involvement in a case of Fatal polymyositis. Arch Neurol 19: 545, 1968. 4. Lynch, P. (3.: Cardiac involvement in chronic polymyositis. Br Heart J33:416, 1971. 5. Schaumburg, H. H., Nielsen, S . L. & Yurchak, P. M.: Heart block in polymyositis. New Engl J Med 284:480. 1971.
6. Walton, J. N. & Adams. R. D.: Polymyositis. p. 157. E. & S. Livingstone, London, 1958. 7. Sharratt, G. P., Danta, G . & Carson, P. H. M.: Cardiac abnormality in polymyositis. Ann Rheum Dis 36: 575. 1977. 8. Reza, M. J., Goldberg, L. S . & Pearson, C. M.: Cardiac involvement in polymyositis. Abstracts. XIV International Congresb of Rheumatology, San Francisco, 1977, No. 86, p. 40. 9. Bohan, A. & Peter, J. B.: Polymyositis and dermatomyositis (First of two parts). New Engl J Med 292: 344. 1975. 10. Bohan, A. & Peter, J . B.: Polymyositis and dermatornyositis (Second of two parts). New Engl J Med 292: 403, 1975. Submitted for publication May 5 . 1978
Martti Oka Department of Medicine Central Hospital 40620 Jyvaskyla 62 Finland
