Immunosuppressant and immunomodulatory treatment for dermatomyositis and polymyositis

Academic Department of Rheumatology, GKT School of Medicine, King's College Hospital, Denmark Hill, London, UK, SE5 9RS.
Cochrane database of systematic reviews (Online) (Impact Factor: 6.03). 02/2005; 3(3):CD003643. DOI: 10.1002/14651858.CD003643.pub2
Source: PubMed


Polymyositis and dermatomyositis are long-term inflammatory muscle diseases, causing muscle weakness and disability. For some reason, the body's immune system turns against its own muscles in an autoimmune response. Corticosteroids are the principle treatment but due to side effects, there is a need for additional treatment from drugs that suppress the immune system (immunosuppressants) or modify it (immunomodulatory therapies) to improve disease response. The small number of randomised trials are inadequate to decide whether these agents are beneficial in dermatomyositis and polymyositis. One small trial of intravenous immunoglobulin in dermatomyositis suggests that it is beneficial. More randomised controlled trials are needed.

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Available from: John B Winer, Jan 07, 2015
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    • "Biological agents, in particular TNF-α blocking agents, have been proposed as potential steroid-sparing agents and as long-term therapies in addition or substitution to corticosteroid therapy [10–12]. According to Martin et al., anti-TNF-α therapy has become the most commonly considered second- or third-line therapy for patients with refractory juvenile DM in the UK even in the absence of prospective randomized control trials (RCTs) to support such use [13]. "
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    ABSTRACT: We performed a systematic search of databases from 1990 to 2013 to identify articles concerning the new onset of dermatomyositis/polymyositis (DM/PM) in patients treated with anti-TNF- α therapy. We retrieved 13 publications describing 20 patients where the new onset of DM/PM after anti-TNF- α therapy was recorded. 17 patients were affected by rheumatoid arthritis (RA), one by Crohn's disease, one by ankylosing spondilytis, and one by seronegative arthritis. In 91% of the cases antinuclear autoantibodies were detected after the introduction of anti-TNF- α therapy. In 6 patients antisynthetase antibodies were detected and other clinical findings as interstitial lung disease (ILD) were recorded. Improvement of DM/PM after anti-TNF suspension (with the concomitant use of other immunosuppressors) was recorded in 94% of cases. The emergence of DM/PM and antisynthetase syndrome seem to be associated with the use of anti-TNF- α agents, especially in patients with chronic inflammatory diseases (mainly RA) with positive autoantibodies before therapy initiation. In particular, physicians should pay attention to patients affected by RA with positive antisynthetase antibodies and/or history of ILD. In those cases, the use of the TNF- α blocking agents may trigger the onset of PM/DM or antisynthetase syndrome or may aggravate/trigger the lung disease.
    Full-text · Article · Jan 2014 · The Scientific World Journal
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    • "The therapeutic management of myositis is challenging. Conventional approaches include baseline measurements of muscle involvement followed by prednisone for 6 to 8 weeks [9]. Although corticosteroids often remain the initial therapeutic choice, side effects and limited efficacy require additional steroid-sparing agents. "
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    ABSTRACT: The idiopathic inflammatory myopathies are rare diseases for which data regarding the natural history, response to therapies and factors affecting mortality are needed. We performed this study to examine the effects of treatment and clinical features on survival in polymyositis and dermatomyositis patients. A total of 160 consecutive patients (77 with polymyositis and 83 with dermatomyositis) seen at the University of Michigan from 1997 to 2003 were included. Medical records were abstracted for clinical, laboratory and therapeutic data, including initial steroid regimen and immunosuppressive use. State vital records were utilized to derive mortality and cause of death data. Survival was modeled by left-truncated Kaplan-Meier estimation and Cox regression. The 5- and 10-year survival estimates were 77% (95% CI = 66 to 85), and 62% (95% CI = 48 to 73), respectively, and the rates were similar for polymyositis and dermatomyositis. Survival between the sexes was similar through 5 years and significantly lower thereafter for males (10-year survival: 18% male, 73% female; P = 0.002 for 5- to 10-year interval). The sex disparity was restricted to the polymyositis group. Increased age at diagnosis and non-Caucasian race were associated with lower survival. Intravenous versus oral corticosteroid use was associated with a higher risk of death among Caucasians (HR = 10.6, 95% CI = 2.1 to 52.8). Early survival between patients treated with methotrexate versus azathioprine was similar, but survival at 10 years was higher for the methotrexate-treated group (76% vs 52%, P = 0.046 for 5- to 10-year interval). Patients treated initially with intravenous corticosteroids had higher mortality, which was likely related to disease severity. Both methotrexate and azathioprine showed similar early survival benefits as first-line immunosuppressive drugs. Survival was higher between 5 and 10 years in the methotrexate-treated group, but could not be confirmed in multivariable modeling for the full follow-up period. Other important predictors of long-term survival included younger age, female sex and Caucasian race.
    Full-text · Article · Jan 2012 · Arthritis research & therapy
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    • "To perform a placebo-controlled trial would nowadays be considered unethical. Other immunosuppressive drugs can be used as add-on medication but are usually not as effective, stressing the need for alternative first-line treatment strategies with fewer side effects [3]. Oral dexamethasone is seven times more potent than prednisone and pulsed schemes have been successfully used in other autoimmune diseases as an alternative to prednisone [4– 11]. "
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    ABSTRACT: To determine if high-dose pulsed dexamethasone is more effective and safer than daily high-dose prednisolone in treatment-naive adult patients with inflammatory myopathies (sporadic inclusion body myositis excluded) we performed a multicenter, double-blind randomised controlled clinical trial with 18 months follow-up. Sixty-two patients were randomised into 28-day cycles of oral high-dose dexamethasone or daily high-dose prednisolone. Primary outcome measures included (1) seven point composite score of six clinically relevant outcomes and (2) (time-to) remission and (time-to) relapse. No difference between both treatment groups on the composite score was found. Side-effects occurred significantly less frequently in the dexamethasone group. Median time to relapse was 60 (2.9) weeks in the prednisolone and 44 (4.7) weeks in the dexamethasone group (log-rank test p=0.03). In conclusion, pulsed high-dose oral dexamethasone is not superior to daily prednisolone as first-line treatment of idiopathic inflammatory myopathies, but is a good alternative by causing substantially fewer side-effects.
    Full-text · Article · Jun 2010 · Neuromuscular Disorders
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