0521779407-17 CUNY1086/Karliner 0 521 77940 7 June 13, 2007 7:57
1192 Polymyositis and Related Disorders
General Measures
■Check baseline labs used to screen side effects of meds
■CXR & pulmonary function tests if concerned for interstitial lung
disease
■Swallowing study if there is dysphagia or hoarseness
■Physical therapy to prevent contracture & maintain strength of unaf-
fected muscles
■Screen for malignancy as appropriate for pt’s age & gender
specific therapy
Treatment Options
■High-dose oral prednisone
■In severe disease, add methotrexate or azathioprine
■In DM, topical steroids or hydroxychloroquine may help rash but
not muscle weakness
■In refractory cases, IVIg may lead to improvement
■Cylcosporine, tacrolimus & alkylating agents such as cyclophos-
phamide & chlorambucil have also been reported as effective
■Case reports also describe the efficacy of tumor necrosis factor
inhibitors such as infliximab
Side Effects & Contraindications
■Prednisone: hypertension, glucose intolerance, sodium retention,
hypokalemia, rash, cushingoid appearance, osteoporosis, aseptic
necrosis, cataracts, infection
■Methotrexate: mucositis, GI distress, cirrhosis, bone marrow
depression, pneumonitis, pseudolymphoma, infection. Avoid use
w/alcohol use or liver or renal dysfunction.
■Azathioprine: GI distress, bone marrow depression, serious infec-
tion. Increased risk of neoplasia if previously used alkylating agent.
Avoid concomitant use of allopurinol.
follow-up
■See every 4 to 6 weeks, checking muscle strength & CPK & for poten-
tial drug toxicities. Frequency can be decreased if remission is seen.
■Meds are gradually tapered over a 6-month period once remission
has been achieved for 6 to 12 weeks.
complications & prognosis
■Refractory disease may result in severe muscle weakness w/ atrophy,
joint contractures, aspiration pneumonia