British Heart,Journal, I97I, 33, 416-4I9.

Cardiac involvement in chronic polymyositis

P. G. LynchFrom the Department of Neuropathology, University of Manchester

This is a report of a 30-year-old male patient with a six-year history of chronic polymyositis.There was no family history of muscular or cardiac disease. Three years after the onset of theillness the patient developed congestive cardiac failure with evidence of complete heart block.Treatment with steroids failed to arrest the course of the disease. Necropsy showed abnormalitiesin the proximal and distal limb muscles, in the sternomastoid muscles, and in the laryngeal muscles.There were extensivefibrotic lesions in the chambers of the heart and in the conducting system.

Severe cardiac involvement is a rare mani-festation of chronic polymyositis. Oppenheim(I899) appears to have been the first author todescribe cardiac lesions in this condition.Subsequently Sheard (195I) pointed out thatpolymyositis involving the pericardium occur-red in some cases. Radermecker and vanBogaert (I955) described cardiac lesions in 2cases of acute polymyositis, and Barnard,Rankin, and Robertson (I960) describedchanges in the heart of a i6-year-old WestAfrican girl who died during the acute phaseof polymyositis. Garcin et al. (I955) statedthat clinical evidence of pericarditis or ofmyo-cardial involvement was uncommon in poly-myositis, though electrocardiographic ab-normalities were sometimes found. Waltonand Adams (I958) found clinical and electro-cardiographic evidence of pericardial involve-ment in 4 out of 40 cases classified as poly-myositis.The case to be described in this paper

illustrates many ofthe clinical and pathologicalfeatues of chronic polymyositis.The biopsy and postmortem histological

findings in clinically affected and non-affectedvoluntary muscle will be described. Theresults of a detailed examination of the heartincluding the conducting system will also bepresented.

Clinical featuresThe patient, a 27-year-old warehouseman, wasadmitted to Manchester Royal Infirmary in AprilI965 complaining of weakness of the legs and ofslight difficulty inswallowing, of 3 years' duration.The symptoms began in December I962 when henoticed that his legs seemed heavy after walking a

short distance. He also found difficulty in standingerect and in rising from a recumbent position. Thepatient had also noticed occasional slight difficultyin swallowing, but had never suffered from doublevision nor from drooping of the eyelids. In I960he had an attack of unexplained haematuriaassociated with backache. There was no pasthistory ofrheumatic fever, venereal disease, nor ofexposure to toxic chemicals. There was no familyhistory of muscular disease.

Physical examination The patient weighed57 kg. There was slight uniform enlargement ofthe thyroid gland. Cardiovascular system: thepulse showed no abnormalities. Blood pressureI20/65 mm Hg. On auscultation, the first andsecond sounds at the apex and in the second rightinterspace were very quiet; in the third left inter-space, especially in the expiratory phase, therewas a short but loud systolic murmur followed bya clear second sound. No abnormalities werefound in the alimentary and respiratory systems.Examination of the central nervous system showedno cranial nerve abnormalities. The ankle-jerkswere absent. The plantar responses were flexor.Sensation was normal. Examination of themusculo-skeletal system showed no evidence ofmyotonia or fasciculation. There was slightwasting and weakness of the right and left supra-spinatus and infraspinatus muscles. There wasweakness of the right and left deltoid muscles andbilateral winging of the scapula. Muscle tone inthe upper and lower limbs was slightly diminishedon both sides. There was a diffuse wasting of bothlegs, almost equal above and below the knee. Theright and left gluteus maximus muscles werewasted. The hamstring muscles and the ileo-psoasmuscles showed much weakness. Skin lesions andmuscle tenderness were absent.

Investigations Haematology: Hb I5'2 g./IOOml.; leucocytes 9,300/cu. mm. (normal differen-tial); ESR (Wintrobe) 2 mm./hr.

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Cardiac involvement in chronic polymyositis 417

Biochemistry: Glucose tolerance test normal;serum protein bound iodine 5-4 ±g.f100 ml.;radio-iodine test uptake in thyroid at 2 hours - 8per cent (normal); serum cholesterol i85 mg./Ioo ml.; blood urea 25 mg./Ioo ml.; serum Na+I39 mEq/l.; serum K+ 4-8 mEq/l.; serumaldolase 12-2 Bruns units/l. (normal range 3-10Bruns units/l.); serum creatine phosphokinase 5-3International units/l. (normal range up toi International unit/l.); 24-hour urine creatine430 mg.; 24-hour urine creatinine 773 mg. (normalrange 1-2 g./24 hr.). Volume of 24-hour urinespecimen 1330 ml. SGOT 3I units/ml. (normalrange 6-40 units/ml.); SGPT ii units/ml.(normal range 5-35 units/ml.). Serum albumin4'2 g./Ioo ml. Serum globulin 3-2 g./IOO ml.

Chest x-ray: No abnormalities detected.

Nerve conduction studies: No abnormalitiesdetected.

Electromyography: Most muscles examinedshowed considerable spontaneous fibrillationpotentials and positive sharp waves. Short dura-tion polyphasic volitional action potentials werealso noted.

Muscle biopsy (left deltoid muscle): Histologicalexamination showed myopathic changes (Fig. i).

Treatment The patient was given prednisoneand neomercazole.

Progress In November I965 the patientdeveloped congestive cardiac failure which re-sponded initially to treatment with digoxin anddiuretics. In February i966 he was found to havea pulse rate of 40/min. and an electrocardiogramshowed evidence of complete heart block. Treat-ment was started with frusemide 'lasix' andisoprenaline ('saventrine'). There was an initialfavourable response to this regimen, but attacks ofheart block recurred later. In June i966 cardiaccatheterization studies gave findings consistentwith a constrictive type of cardiomyopathy.Electrocardiography showed right bundle-branchblock. A biopsy specimen was taken from the leftsupraspinatus muscle. Histological examinationshowed myopathic features, and there was noevidence of immation. No antibody againsthuman heart muscle could be found in thepatient's serum using the fluorescent antibodytechnique.

After discharge from hospital the patient'scondition deteriorated. An internal cardiac pace-maker was inserted on 17 October 1968, but thepatient suddenly collapsed and died on i9 OctoberI968.

Pathological featuresMacroscopical Necropsy was performed 48hours after death. The body weighed 37 kg.,height I80 cm. There was symmetrical wasting ofthe limb muscles. Skeletal muscle tissue in theaffected regions was pale fawn and of a soft con-sistency. There was uniform enlargement of the

FIG. i Deltoid muscle, showing myopathicchanges. (Masson's trichrome. x ixo.)

F I G. 2 Heart. Sino-atrial node showingfibrosis. The artery to the node can be seen onthe left in the lower part of the field.(Anderson's Victoria Blue and Van Gieson.x iIo.)

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4I8 P. G. Lynch

thyroid gland. The lungs showed areas of collapsein their lower lobes and there was atrophy of thecortex of both suprarenal glands. The heartweighed 44o g. There was a fibrinous pericarditis.Cardiac pacemaker leads were stitched in themyocardium of the left ventricle. The averagethickness of the left ventricular myocardium wasi8-o mm. (normal being I2 mm.). The cutsurfaces of the left ventricle revealed severalsmall foci of greyish-white tissue in the myo-cardium of the left ventricle. The heart valves,coronary ostia, and coronary arteries showed noabnormalities. The aorta, pulmonary arteries,superior vena cava, inferior vena cava, and pul-monary veins showed no abnormalities. Thebrain, spinal cord, and peripheral nerves showedno naked-eye abnormalities.

Microscopical Heart: There was extensive re-placement fibrosis of cardiac muscle fibres in theright atrium and right ventricle. The left atriumshowed mild epicarditis only. The left ventricleshowed scattered areas of replacement fibrosis ofcardiac muscle fibres. The cardiac valves andcoronary arteries showed no abnormalities.

Conducting system: The sino-atrial node arterieswere thick-walled but distinct and had goodlumens. The entire node showed conspicuousfibrosis and was virtually devoid of specializedmyocardium (Fig. 2). Adjacent myocardium ofthe right atrium showed replacement fibrosis. TheAV node showed no abnormalities. The nodeartery branches were greatly thickened by fibroustissue. The thickening appeared to affect thetunica media. The bundle of His was prominentand showed some fibrosis towards the bifurcation.The left bundles were seen only proximally nearto their origin. More distally the fascicles ran intosubendocardial fibrosis of the interventricularseptum (Fig. 3), in which scattered Purkinje cellscould be identified. The right bundle was readilyfollowed, but in places it was completely fibrousand contained no muscle fibres (Fig. 4). Theinteratrial and interventricular septa showedmoderate replacement fibrosis without cell reac-tion. The intramuscular vessels appeared normal.No histological abnormalities were found in the

brain, spinal cord, or dorsal root ganglia. The skinshowed no abnormalities. The thyroid glandshowed the pattern of a diffuse colloid goitre.

Voluntary muscle: Myopathic changes were seenin the sternomastoid, sartorius, peroneus brevis,and posterior cricoarytenoid muscles. No abnor-mal cellular infiltrates and no blood vesselchanges were noted in these muscles.

DiscussionDespite the fact that there was no evidence ofinflammation in any of the muscles examinedat necropsy, nor in any of the three musclebiopsy specimens taken from the patientduring his illness (one of these biopsy speci-mens was taken at another hospital in I964),there were many clinical similarities to poly-

F IG . 3 Heart. Left bundle showing fasciclesrunning into an area of subendocardialfibrosis. (Haematoxylin and eosin. x I90.)

FIG. 4 Heart. Right bundle showing almosttotal replacement fibrosis of the conductingtissue. (Haematoxylin and eosin. x izo.)

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Cardiac involvement in chronic polymyositis 419

myositis. These features were the rapid pro-gress of the disease, the symmetrical distribu-tion of muscular weakness in the upper andlower limbs, and the presence of dysphagiaand dysarthria.The relation between the changes found in

the myocardium and voluntary muscles isobscure. It is tempting to postulate a commonaetiological agent affecting both tissues. Theabsence of lymphocytes in both tissues andthe lack of serum antihuman heart antibodyseems to exclude a disorder of immunemechanisms. The poor response to steroidtherapy is also in keeping with this view.

It is worth remembering the importance ofexaminig the voluntary muscles in all casesof cardiomyopathy. The converse is also true.

I am indebted to Professor P. 0. Yates for hisadvice and helpful criticism, to Professor R. E. B.Hudson for his detailed mination of the con-ducting system of the heart, to Dr. L. A. Liver-

sedge for supplying clinical details of the patient,to Dr. G. Behr and Dr. J. Ball for allowing me tostudy muscle biopsy specimens from the patient;to Mrs. J. Cornwell for typing the manuscript, toMrs. C. M. Worsley for technical assistance, andto Mr. G. Humberstone for preparing the photo-micrographs.

ReferencesBarnard, B. G., Rankin, A. M., and Robertson, J. H.

(I960). Polymyositis. Report on 3 cases from WestAfrica. British Medical journal, I, I473.

Garcin, R., Lapresle, J., Gruner, J., and Scherrer, J.(1955). Les polymyosites. Revue Neurologique, 92,465.

Oppenheim, H. (I 899). Zur Dermatomyositis. Berlinerklinische Wochenschrift, 36, 805.

Radermecker, M. A., and van Bogaert, L. (i955). Deuxobservations de polymyosite aigue gen6ralise(Wagner-Hepp). Revue Neurologique, 92, 182.

Sheard, C. (i95i). Dermatomyositis. Archives ofInternal Medicine, 88, 640.

Walton, J. N., and Adams, R. D. (1958). Polymyositis.Livingstone, Edinburgh.

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