Chest pain, weakness, and elevated cardiac enzymes: How would you treat?

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Case Reports

Chest pain, weakness, and elevated cardiac enzymes: How would you treat?

J Fam Pract. 2006 February;55(2):118-124

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References

1. Alpert JS, Thygesen K, Antman E, et al. Myocardial infarction redefined—a consensus document of the Joint European Society of Cardiology/American College of Cardiology Committee for the redefinition of myocardial infarction. J Am Coll Cardiol 2000;36:959-969.

2. Braunwald E, Antman EM, Beasley JW, et al. ACC/AHA 2002 guideline update for the management of patients with unstable angina and no-ST-segment elevation myocardial infarction—summary article: a report of the American College of Cardiology/American Heart Association task force on practice guidelines (Committee on the Management of Patients With Unstable Angina). J Am Coll Cardiol 2002;40:1366-1374.

3. Ng SM, Krishnaswamy P, Morrisey R, et al. Mitigation of the clinical significance of spurious elevations of cardiac troponin I in settings of coronary ischemia using serial testing of multiple cardiac markers. Am J Cardiol 2001;87:994-999.

4. Jeremias A, Gibson C. Narrative review: Alternative causes for elevated cardiac troponin levels when acute coronary syndromes are excluded. Ann Intern Med 2005;142:786-791.

5. Miller M. Muscle enzymes in the evaluation of neuromuscular disease. In UpToDate [database]. Available at www.uptodate.com. Accessed on January 10, 2005.

6. Dalakas C, Hohlfeld R. Polymyositis and dermatomyositis. Lancet 2003;362:971-982.

7. Rowland LP. Polymyositis, inclusion body myositis, and related myopathies. In: Merritt’s Neurology. 10th ed. Philadelphia, Pa: Lippincott, Williams & Wilkins; 2000:765-769.

8. Peterson LZR, Marfin AA. West Nile virus: a primer for the clinician. Ann Intern Med 2002;137:173-179.

9. van der Meulen MFG, Bronner I, et al. Polymyositis: An overdiagnosed entity. Neurology 2003;61:316-321.

10. Callen J. Dermatomyositis. Lancet 2000;355:53-57.

11. Marie I, Hachulla E, Cherin P, et al. Interstitial lung disease in polymyositis and dermatomyositis. Arthritis Rheum 2002;47:614-622.

12. Sparsa A, Liozon E, Herrmann F, et al. Routine vs extensive malignancy search for adult dermatomyositis and polymyositis. Arch Dermatol 2002;138:885-890.

13. Buchbinder R, Forbes A, Hall S, Dennett X, Giles G. Incidence of malignant disease in biopsy-proven inflammatory myopathy. Ann Intern Med 2001;134:1087-1095.

14. Hengstman GJD, Brouwer, Egberts WT, et al. Clinical and serological characteristics of 125 Dutch myositis patients: Myositis specific antibodies aid in the differential diagnosis of the idiopathic inflammatory myopathies. J Neurol 2002;249:69-75.

15. Schmidt WA, Wetzel W, Friedlander R, et al. Clinical and serological aspects of patients with anti-Jo-1 antibodies—an evolving spectrum of disease manifestations. Clin Rheumatol 2000;19:371-377.

16. Dalakis M, Illa I. A controlled trial of high-dose intravenous immune globulin infusions as treatment for dermatomyositis. N Engl J Med 1993;329:1993-2000.

17. Miller FW, Leitman SF, Cronin M, et al. Controlled trial of plasma exchange and leukapheresis in polymyositis and dermatomyositis. N Engl J Med 1992;326:1380-1384.

18. Marie I, Hachulla E, Hatron PY, et al. Polymyositis and dermatomyositis: Short term and longterm outcome, and predictive factors of prognosis. J Rheumatol 2001;28:2230-2237.

19. Danko K, Ponyi A, Constantin T, Borgulya G, Szegedi G. Long-term survival of patients with idiopathic inflammatory myopathies according to clinical features: A longitudinal study of 162 cases. Medicine (Baltimore) 2004;83:35-42.

CORRESPONDENCE: Edward Onusko, MD, 825 West Locust Street, Wilmington OH 45177. edonusko@cmhregional.com

Differentiate between the inflammatory myopathies based on characteristic pathological findings on muscle biopsy (previously discussed). Muscle biopsy is the definitive test for establishing the diagnosis. In our case presentation, the regional neuropathologist thought the biopsy result was most consistent with dermatomyositis despite the clinical paucity of skin abnormalities, though our consulting neurologist favored a diagnosis of polymyositis on clinical grounds.

Treatment recommendations

Corticosteroids are the most efficacious treatment for dermatomyositis (strength of recommendation [SOR]: B).¹⁰ One empirical regimen is to give prednisone 1 mg/kg/d as initial therapy; maintain this therapy for 1 month after symptoms and CK have normalized; then slowly taper (SOR: C).¹⁰ Twenty-five percent of patients will not respond to steroids; others will not tolerate the side effects of steroid therapy.¹⁰

FAST TRACK

Corticosteroids are the preferred treatment for dermatomyositis; immune system suppressing drugs may be useful as a second-line treatment

Immunosuppressive drugs such as azothioprine, methotrexate, cyclosporine, mycophenolate mofetil and cyclophosphamide may be used as second-line treatment (SOR: C).⁶ Intravenous immunoglobulin may have some efficacy (SOR: B).¹⁶ Plasmapheresis does not appear to be effective (SOR: B).¹⁷

Determinants of prognosis

Most patients will improve over several weeks or months with therapy, although a third or more are left with mild to severe muscle damage. Dermatomyositis responds better than polymyositis; inclusion-body myositis is the most difficult to treat.¹⁰ Poor prognostic factors include older age, association with cancer, pulmonary fibrosis, dysphagia with aspiration pneumonia, cardiac involvement, steroid-resistant disease, and calcinosis in dermatomyositis.^6,10 Studies have demonstrated 5-year survival rates between 77% and 92%.^18,19 The main causes of death were related to malignancy and cardiac or pulmonary complications.