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Polymyositis with Cardiac Manifestations and Unexpected Immunology I Morrison, a Mcentegart, H Capell

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Polymyositis with Cardiac Manifestations and Unexpected Immunology I Morrison, a Mcentegart, H Capell 1110 Ann Rheum Dis: first published as 10.1136/ard.61.12.1113 on 1 December 2002. Downloaded from LETTERS Polymyositis with cardiac manifestations and unexpected immunology I Morrison, A McEntegart, H Capell ............................................................................................................................. Ann Rheum Dis 2002;61:1110–1111 olymyositis is an inflammatory myopathy of skeletal consistent with previous poliomyelitis, in addition to severe muscle. Cardiac involvement in the disease was first myositis. Magnetic resonance imaging could not be performed Pdescribed in 18991 but has only been studied in detail over because of the pacemaker. A diagnosis of polymyositis was the past 20 years. About 10–15% of all patients with poly- made and treatment was started with 60 mg/day prednisolone myositis have a cardiac abnormality as their initial presenting and intravenous immunoglobulin. A muscle biopsy, after 13 feature, while up to 70% of all those with polymyositis will days of prednisolone, showed an inflammatory infiltrate with have some cardiac involvement diagnosed non-invasively dur- myonecrosis consistent with inflammatory myopathy. ing the course of their illness.2 These can manifest either as Anti-acetylcholine receptor antibodies and anti-titin anti- cardiac failure; electrocardiographic changes, including non- bodies were also positive but CT of the thorax showed no evi- specific ST changes, and varying forms of AV block; myocardi- dence of thymoma and single fibre EMG studies were not tis; valve disease and myocardial ischaemia in the presence of supportive of a diagnosis of myasthenia gravis. normal coronary vasculature.3 The patient was discharged after 20 days on 55 mg/day Myasthenia gravis is thought to be an autoimmune disease prednisolone with repeat admissions organised for immuno- characterised by the presence of antibodies to the acetyl- globulin every six weeks, and neurological review for her pos- choline receptor of the motor end plate. Idiopathic forms are sible myasthenia gravis. Upon discharge, she had 4/5 muscle rarely associated with polymyositis,4 but a much stronger cor- power with a CK of 1113 U/l. relation exists in the presence of a thymoma, particularly in Subsequent follow up two months after her initial those with myocardial involvement, when anti-titin antibod- admission showed a CK of <200 U/l and complete restoration ies are usually present.5 However, thymoma is often less than of muscle power. She is currently receiving 35 mg/day 1 mm in size and therefore not visible on computed prednisolone, reducing by 2.5 mg/month. tomographic (CT) scanning. We describe a 56 year old woman with polymyositis, who DISCUSSION initially presented with myocardial involvement and was also Our case demonstrates several of the important cardiac found to have antibodies consistent with myasthenia gravis. features in polymyositis. The initial infarction, which occurred in the presence of only two significant cardiovascular risk fac- http://ard.bmj.com/ CASE REPORT tors (hypertension and hypercholesterolaemia), and the A 56 year old woman was admitted to this hospital for inser- subsequent normal coronary angiography suggest the pres- tion of a permanent pacemaker after an episode of dizziness ence of myocardial ischaemia with normal coronary vascula- and collapse, secondary to complete heart block. ture as described previously.6 It is thought that the ischaemia She had a past history of anterolateral myocardial may be related to small vessel myocardial disease, which infarction 10 months previously for which she received would not be evident on angiography.7 However, myocardial thrombolytic treatment in a district general hospital. Treat- infarction in the presence of normal coronary vasculature is ment was started with aspirin, atenolol, ramipril, and pravas- not uncommon, and alternative explanations in this example on September 23, 2021 by guest. Protected copyright. tatin after the infarction. Subsequent coronary angiography include infarction with clot lysis; infarction secondary to cor- was normal. At outpatient follow up five months later, she was onary artery spasm; or myocarditis. found to have a creatine kinase (CK) level of greater than 3000 Fibrosis of the conducting system of the heart, particularly U/l associated with significant leg weakness. Electromyo- the AV node, has been previously characterised8 and might graphic (EMG) analysis showed changes consistent with old have explained the patient’s subsequent presentation in com- polio, which had affected the patient as a child. Attempts at plete heart block. As there was no evidence of significant muscle biopsy were unsuccessful. A statin-induced myositis myocardial ischaemia in this patient (CK MB fraction <0.1 was thought likely, and pravastatin was discontinued. and only mildly raised increase in troponin), fibrosis was felt Six months later she presented in complete heart block and to be the most likely pathological mechanism in this instance. at the time of transfer to cardiology in this hospital for pacing, Arriving at a diagnosis of myasthenia gravis in a patient examination showed significant proximal muscle weakness with muscle inflammation is difficult, as standard investiga- with 3/5 power in both arms, and 2/5 power in the legs; distal tions such as tensilon tests are inaccurate in the presence of muscle power was normal. Neurological examination demon- pre-existing muscle weakness, and a negative EMG analysis strated normal sensation but absent knee, bicep and ankle does not necessarily dismiss the diagnosis.9 The presence of jerks, with bilaterally downgoing plantars. old polio in this patient was a further confounding factor. Investigations showed a CK of 22 800 U/l (CK MB fraction Equally, symptoms may be disguised by both the pre-existing <0.1). The erythrocyte sedimentation rate was mildly raised at polymyositis and its treatment, as immunosuppression is a 35 mm/1st h; thyroid function was normal; and rheumatoid well recognised treatment for both. However, anti- factor, antinuclear antibodies, antineutrophil cytoplasmic anti- acetylcholine and anti-titin antibodies are highly specific,10 bodies, and viral screens were negative. Echocardiography and the association between myasthenia gravis, polymyositis, showed good LV function with trivial MR; ultrasound of the and cardiac disease, particularly with microscopic thymoma, is abdomen was normal; and EMG analysis again showed features well characterised. Although we do not feel we have enough www.annrheumdis.com Letters 1111 Ann Rheum Dis: first published as 10.1136/ard.61.12.1113 on 1 December 2002. Downloaded from evidence to support a diagnosis of myasthenia gravis at 2 Gottdiener JS, Sherber HS, Hawley RJ, Engle WK. Cardiac present, we have referred this patient for neurological follow manifestations of polymyositis. Am J Cardiol 1978;41:1131–9. up and review. 3 Lie JT. Cardiac manifestations in polymyositis/dermatomyositis: how to get to the heart of the matter. J Rheumatol 1995;22:809–10. This case demonstrates several of the cardiac manifesta- 4 Miller FM. Current concepts in the idiopathic inflammatory myopathies: tions of polymyositis. It also highlights the possible neurologi- polymyositis, dermatomyositis, and related disorders. Pathogenesis. Ann cal associations with the disease, and the difficulties in subse- Intern Med 1989;111:150–3. quent diagnosis. 5 Aarli, Johan A. Inflammatory myopathy in myasthenia gravis. Current Opin Neurol 1998;11:233–4. 6 Lynch PG. Cardiac involvement in chronic polymyositis. Br Heart J ..................... 1971;33:416–19. Authors’ affiliations 7 Denbow CE, Lie JT, Trancredi RG, Bunch TW. Cardiac involvement in I Morrison, A McEntegart, H Capell, Centre for Rheumatic Disease, polymyositis: a clinicopathologic study of 20 autopsied patients. Arthritis Glasgow Royal Infirmary, Scotland, UK Rhem 1979;22:1088–92. 8 Kehoe RF, Bauernfeind R, Tommaso C, Wyndham C, Rosen KM. Correspondence to: Dr H Capell, Centre for Rheumatic Disease, Glasgow Cardiac conduction defects in polymyositis. Electrophysiologic studies in Royal Infirmary, 84 Castle Street, Glasgow G4 0SF, UK; four patients. Ann Intern Med 1981;94:41–3. [email protected] 9 Oh SJ, Kim DE, Kuruoglu R, Bradley RJ, Dwyer D. Diagnostic sensitivity of the laboratory tests in myasthenia gravis. Muscle Nerve Accepted 11 April 2002 1992;15:720–4. 10 Vincent A, Newsom-Davis J. Acetylcholine receptor antibody as a REFERENCES diagnostic test for myasthenia gravis: results in 153 validated cases and 1 Oppenheim H. Zur dermatomyositis. Ber Kil Wochenschr 2967 diagnostic assays. J Neurol Neurosurg Psychiatry 1899;38:805–7. 1985;48:1246–52. Eosinophilic fasciitis with multiple myeloma: a new haematological association D Khanna, A Verity, J M Grossman ............................................................................................................................. Ann Rheum Dis 2002;61:1111–1112 osinophilic fasciitis (EF) is characterised by skin indura- osteoporosis included serum and urine electrophoresis, which tion, hypergammaglobulinaemia, and peripheral eosi- showed an IgGλ monoclonal protein on immunofixation. Enophilia. There have been numerous reports of an associ- Serum IgG was 36.00 g/l (normal 6.87–16.30) with other ation of EF with haematological disorders, including immunoglobulins being low normal. A bone marrow biopsy monoclonal gammopathy. To our knowledge, we describe the and aspiration was performed which disclosed 25% plasma http://ard.bmj.com/
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