The heart in polymyositis and dermatomyositis
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Overview
In addition to skeletal muscle involvement, up to 40% of patients may have cardiac abnormalities. A small study of 16 autopsied patients with Polymyositis / Dermatomyositis suggests a poor correlation between the degree of skeletal involvement and myocarditis.
In a study of 55 patients with polymyositis reported that mild diffuse myocarditis, severe inflammation, or fibrosis of the cardiac conduction system have been found in 70% of the patients. Also, anti-SSA (anti-Ro) antibody, which is classically associated with an increased risk of infant cardiac conduction abnormalities in the neonatal lupus syndrome, was present in 69% of patients with evidence of cardiovascular involvement.
The role of gadolinium-DTPA enhanced MRI appears promising in diagnosing myocarditis in polymyositis. Myocarditis leading to congestive heart failure is an uncommon but severe manifestation of Polymyositis/Dermatomyositis.
Although coronary arteritis has been reported in few case reports, there are no controlled studies showing evidence of increased incidence of CAD in Polymyositis / Dermatomyositis.
Glucocorticoids represent the mainstay of therapy. Most patients do very well on oral prednisone therapy, whereas others fail to respond to any agents. The usual practice is to begin treatment with 40 to 80 mg/d of oral prednisone or its equivalent.
Methylprednisolone boluses of 500 to 1000 mg/day for 3 days is reserved for severe and acute cases. Azathioprine (Imuran) and methotrexate are used mostly as steroid-sparing agents.
Intravenous immunoglobulin given in monthly boluses is an expensive therapy that is reserved for patients with severe disease and poor response to conventional immunosuppressive therapy. Response can be seen as early as 2 weeks, but typically best effects are seen only after 3 months. [1]
Classification of Polymyositis and Dermatomyositis into two forms according to manifestations of the diseases as clinical and subclinical will help to better understand their clinical course.
Clinically Manifest Heart Involvement
Both diseases affect the heart. The most frequently reported clinically manifest cardiac problem is congestive heart failure, observed in between 3 and 45% of myositis patients. Whether this is more than in a matched control population is uncertain due to the lack of controlled studies. However, left ventricular diastolic dysfunction (LVDD) was reported in 12–42% of myositis patients compared with the estimated 30% in the general population, supporting an over risk of at least this type of cardiac manifestation in myositis patients.
Pericarditis is not common, but can occur when polymyositis occurs as part of an overlap syndrome with other autoimmune diseases, such as Systemic Lupus Erythematosus or Progressive Systemic Sclerosis.
Coronary arteritis and ischemic Coronary Artery Diseases with angina pectoris are rarely part (frequency is uncertain) of these overlap syndromes.
Localized or generalized myocardial dysfunction is common by echocardiographic assessment, but infrequently causes clinical failure.
The cardiomyopathy may be steroid responsive. Corticosteroid myopathy, a complication of treatment that can mimic Polymyositis, although with a normal Creatine Kinase, generally affects skeletal but not respiratory or cardiac muscle.
Polymyositis and dermatomyositis frequently affect the conduction system. In an electrocardiographic study of 77 patients, 23% had conduction block, which can occur in the absence of cardiomyopathy, usually in the absence of symptoms.
Pulmonary hypertension can occur, but is usually secondary to interstitial lung disease. Acute alveolitis can at times mimic acute congestive heart failure.
Subclinical cardiac involvement
Subclinical cardiac involvement is much more common than manifest heart problems and the frequency varies depending on the methods used. ECG changes are most common and abnormalities on ECG were observed in 32.5–72%.
Observed ECG abnormalities are as follow: atrial and ventricular arrhythmias, bundle branch block, A-V blocks, high-grade heart block, prolongation of PR-intervals, ventricular premature beats, left atrial abnormality, abnormal Q-waves as well as non-specific ST-T wave changes. [1] [1]
References
Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
