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We obtain a chest X-ray and a PPD skin test with controls on patients prior to initiating immunosuppressive medications Patients with prior history of tuberculosis or a positive PPD may need to be treated
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prophylactically with isoniazid If patients have ILD and are to be placed on prednisone plus another immunosuppressive agent, we also start Bactrim for pneumocystis prophylaxis We measure bone density with dual-energy X-ray absorptiometry at baseline and every 6 months while patients are receiving corticosteroids A bone density score <25 standard deviations below normal is considered positive for osteoporosis Calcium supplementation (1 g/d) and vitamin D (400 800 IU/d) are started for prophylaxis against steroid-induced osteoporosis Postmenopausal women are also started on a bisphosphonate for prevention and treatment of osteoporosis We prescribe alendronate 35 mg/week (or another bisphosphonate) as prophylaxis against steroid-induced osteoporosis or 70 mg/week in those with osteoporosis Because the long-term side effects of bisphosphonates are not known, particularly in men and young premenopausal women, we prophylactically treat (alendronate 35 mg/week) these individuals only if the dual-energy X-ray absorptiometry scan demonstrates a density between 1 and 25 standard deviations below normal at baseline or if signi cant bone loss occurs on follow-up scans If bone densities are in the osteoporosis range, these are treated with alendronate 70 mg/week Alendronate can cause severe esophagitis, and absorption is impaired if taken with meals Therefore, patients must be instructed to remain upright and not to eat for at least 30 minutes following the dose of alendronate in the morning Antihistamine-H2 blockers are not started unless the patient develops gastrointestinal discomfort or has a history of peptic ulcer disease We instruct patients to start a low-sodium, low-carbohydrate, high-protein diet to prevent excessive weight gain Physical therapy and an aerobic exercise program are helpful in fending off side affects of prednisone (eg, weight gain) and preventing contractures and calcinosis that may result from immobility Blood pressure is measured at each visit as accelerated hypertension and renal failure may occur, particularly in patients with scleroderma or MCTD83 In addition, periodic eye examinations for cataracts and glaucoma should be performed We periodically check fasting blood glucose and serum potassium levels while they are on high doses of prednisone Potassium supplementation may be required, if the patient becomes hypokalemic
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tients with severe weakness or associated comorbidity (ie, ILD and myocarditis) and diabetes mellitus (for possible steroid-sparing effect) or in elderly or those with known osteoporosis (again for possible steroid-sparing effect)
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IVIG has become increasing popular in the treatment of refractory myositis Small, uncontrolled studies have reported bene cial response in DM and PM with IVIG9,41,87,103,141,205,212,213,258 A mild improvement in muscle strength was reported in three of four patients with IBM treated with IVIG214 However, we were unable to document any signi cant clinical improvement in nine patients with IBM treated with IVIG209 Subsequently two prospective, double-blind, placebo-controlled studies of IVIG in IBM revealed no signi cant improvement203,204 A prospective, double-blind, placebo-controlled study of IVIG in 15 patients with DM demonstrated signi cant clinical improvement with IVIG201 In support of the clinical observations, repeat biopsies in ve of the responsive patients revealed an increase in muscle ber diameter, increase in the number and decrease in the diameter of capillaries, resolution of complement on capillaries, and a reduction in the expression of intercellular adhesion molecule 1 (ICAM-1) and MHC-1 antigens We initiate IVIG (2 g/kg) slowly over 2 5 days and repeat infusions at monthly intervals for at least 3 months15,82 Subsequently, we try to decrease or spread out the dose: 2 g/kg every 2 months or 1 g/kg per month Treatment needs to be individualized Our own anecdotal experience suggests that IVIG is effective for DM and necrotizing myopathies but is less to for PM and not at all for IBM We generally give IVIG in combination with prednisone There is little evidence that it is effective as a monotherapy Prior to treatment, patients should have an IgA level checked Patients with low IgA levels may have anti-IgA antibodies in their sera, which predispose them to anaphylactic reactions to IVIG, because IVIG contains small amounts of IgA Patients should also have renal function checked, especially those with diabetes mellitus, because of a risk of IVIG-induced renal failure Flu-like symptoms headaches, myalgias, fever, chills, nausea, and vomiting are common and occur in as many as half the patients Rash, aseptic meningitis, and stroke can also occur
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