Key Treatment

Prednisone is the treatment of choice in temporal arteritis (SOR: C).

with collagen vascular diseases (e.g., systemic scleroderma, SLE, SS). They have a bimodal distribution and are seen most often between age 10 to 15 and 45 to 60 years. Myo-sitis is most common after age 50. The cause of inflammatory myopathies is unknown, but evidence suggests a genetic predisposition (associated with certain HLA markers) combined with an environmental insult, such as viruses, thereby initiating an autoimmune process.

Patients usually experience progressive, symmetric, proximal muscle weakness with fatigue, malaise, and morning stiffness. Muscles often affected are those of the shoulder, neck, and pelvic girdle. Pulmonary (interstitial pneumonitis or fibrosis), cardiac (cardiomyopathy, congestive heart failure, arrhythmias), pharyngeal (dysphagia), and muscu-loskeletal (myalgias, arthralgias) symptoms might occur, although most patients do not experience synovitis. CK as well as aldolase, alanine transaminase (ALT),AST, and lactate dehydrogenase (LDH) levels might be elevated, although ESR is elevated only half the time. Muscle biopsy can also be helpful in diagnosis. Dermatomyositis is characterized by all these manifestations plus a scaly, erythematous, or violaceous rash on the face and neck, upper back (shawl sign), and upper anterior chest and neck (V sign). Other signs include dystrophic cuticles, mechanic's hands (darkened or dirty-appearing horizontal lines across palmar aspect of fingers), and Gottron's papules, seen on dorsal aspects of the PIP, DIP, or MCP joints, elbows, patellae, and medial mal-leoli (Olsen and Wortmann, 2004). Disease in patients with skin changes but without muscle inflammation is termed amyopathic dermatomyositis.

Inflammatory myopathies can result from malignancies. Screening for malignancies common for the patient's gender and age should be undertaken, but further exploration is not indicated. However, ovarian cancer appears to be associated with dermatomyositis, so the family physician should consider evaluation for this.

Before treatment, a thorough motor neurologic examination is done, with muscle enzyme levels (CK, aldolase, ALT, AST, LDH) and cancer screening tests appropriate for patient age and gender. Prednisone, 1 mg/kg/day for up to several months, is the drug of choice; the earlier started in the disease process, the more effective it normally is. If prednisone is not sufficient, methotrexate, azathioprine, or another immunosuppressant is added.

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