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Year : 2010  |  Volume : 58  |  Issue : 1  |  Page : 3-5

Drug treatment of polymyositis and dermatomyositis

Department of Neurology, The Institute of Neurological Sciences, CARE Hospital, Exhibition Road, Nampally, Hyderabad - 500 001, India

Date of Acceptance25-Jan-2010
Date of Web Publication8-Mar-2010

Correspondence Address:
J.M.K Murthy
Department of Neurology, The Institute of Neurological Sciences, CARE Hospital, Exhibition Road, Nampally, Hyderabad - 500 001
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.60386

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How to cite this article:
Murthy J. Drug treatment of polymyositis and dermatomyositis. Neurol India 2010;58:3-5

How to cite this URL:
Murthy J. Drug treatment of polymyositis and dermatomyositis. Neurol India [serial online] 2010 [cited 2023 Jun 10];58:3-5. Available from:

Idiopathic immune-mediated myopathies, notably polymyositis (PM) and dermatomyositis (DM) are relatively uncommon diseases and are associated with considerable morbidity and mortality if not recognized early and treated, in some cases as high as 50%, primarily related to life-threatening muscle weakness, cardiac and lung complications. [1],[2],[3],[4] The morbidity and mortality associated with PM and DM can be reduced to some extent by early diagnosis and institution of appropriate treatment. [2],[5],[6],[7],[8],[9] The importance of early drug therapy in PM and DM has once again been emphasized by Peyman et al., [10] in the article published in this issue of the journal. In their study early institution of drug therapy was associated with early and higher remission rates.

The evolving evidence suggests that the underlying immunopathogenesis of inflammatory myopathies may determine the response to drug treatment and also long-term prognosis. In DM a complement-dependent humoral process thought to be initiated by antibodies to endothelial cells results in microangiopathy with secondary ischemic changes in muscles, while in PM there is a T-cell response with invasion of muscle fibers by CD8 + lymphocytes and perforin-mediated cytotoxic necrosis. [11] The PM/DM complex is associated with several autoantibodies: Antisynthetase antibodies (Jo1, Pl7, PL12), anti-Mi2 antibody, anti-SRP antibody, anti-Ku antibody, anti-Ro, anti-U1-RNP antibodies and, notably, anti-PM-Scl antibody. [12] Presence of anti-PM-Scl antibody is not a good prognostic factor in patients with PM/DM, as there appears to be an association with lung (interstitial lung disease, ILD) and esophageal involvement as well as with malignancy. [13] The most common of the antisynthetases is the Jo-1 antibody which is associated with ILD and Raynaud phenomena (antisynthetase syndrome). [14],[15] ILD associated with PM/ DM is recognized as an important condition because it frequently causes death. [2],[4],[16],[17],[18],[19],[20]

PM and DM are highly treatable diseases and may achieve a complete or worthwhile functional remission. Treatment in PM and DM is very similar, with drugs that suppress the immune system the mainstay of therapy. However, there are no defined guidelines or best treatment protocols agreed internationally and the treatment thus remains largely empirical. There are only a few prospective, double-blinded, placebo-controlled trials in the treatment of PM [21] and DM. [22]

Corticosteroids, prednisolone, is the initial drug of choice. High dose of prednisolone is maintained until muscle strength normalizes, improvement in strength reaches a plateau or serum creatine kinase (CK) normalizes, which usually takes three to six months. Subsequently the prednisolone dose is tapered over a few weeks. Most of the patients need to remain on at least a small dose of prednisolone to remain in sustained remission. [23] Significant improvement is noted in 60-80% of the patients. [16],[17] In patients with severe disease, weakness of the bulbar and respiratory muscles or delay in the diagnosis, a more rapid response may be obtained by commencing treatment with intravenous methylprednisolone followed by oral prednisolone. [11] Initial combination therapy with prednisolone and azathioprine has been used to achieve better disease control and allowing quicker steroid withdrawal. In addition combination therapy appears to reduce the risk of relapse [24],[25] and also improve long-term outcome. [26] It appears that patients of PM and DM with associated ILD may require initial combination therapy. [13],[20] Similarly, patients with anti-synthetase syndrome who often have refractory myositis and ILD, or those with anti-SRP antibodies who typically have a severe necrotizing myopathy with little inflammation may require an aggressive immunosuppressive therapy, usually a combination therapy. [11] It may be prudent to avoid methotrexate in patients with ILD as it is one of the adverse events of methotrexate.

In a proportion of patients with PD or DM (20-30%) it is not possible to achieve complete remission with corticosteroids and relapses are not uncommon. Patients who are resistant to steroids or who relapse with reduction of steroid dosage require other immunosuppressive or immunomodulatory treatment. In a controlled trial intravenous immunoglobulin (IVIg) has been shown to be effective in patients with DM as an add-on treatment [22] and in uncontrolled trials in patients with PM and overlap syndromes. [27],[28] In patients with refractoriness to steroids initial IVIg dosage is 2 g/kg over a five-day period (0.4 g/kg/ day), followed by monthly three-day courses for three to six months during which period prednisolone and other immunosuppressive agents are also controlled. Treatment needs to be individualized. [11] In an open study IVIg as an add-on treatment with mycophenolate mofetil in patients with severe PM/DM (steroid-dependency, refractoriness to steroids and/or immunosuppressants, and life-threatening disease) has been shown to be effective, moreover as a safe and steroid-sparing agent. [29] IVIg treatment has been shown to be safe and an effective salvage therapy for refractory ILD associated with PM/ DM in certain cases. [20]

Methotrexate and azathioprine are the two immuno suppressive agents used in patients refractory to steroids or who relapse during prednisolone taper. Another advantage of these drugs is their potential steroid-sparing effect. Methotrexate has been shown to be effective in DM and PM, mostly in the retrospective studies [30],[31],[32],[33],[34],[35] and is administered only one day a week. Careful attention should be paid to the serious adverse events associated with methotrexate treatment: Bone marrow, renal and liver toxicity and ILD. Retrospective studies [31],[36] and an open-label follow-up study of the prospective double-blind study [26] indicate that azathioprine is an effective drug in DM and PM. Complete blood counts and liver function tests need to be followed closely. If there is no improvement with either of the drugs after six to eight weeks other options have to be considered which include mycophnolate, cyclosporine, tacrolimus, cyclophosphamide, and tumor necrosis Factor-a blockers (infliximab, etanercept). [23] Cyclophosphamide is usually reserved for patients refractory to most other modalities. [37],[38],[39],[40] B-cell depletion therapy with rituximab used alone or in combination with other immunosuppressive therapies is another viable option in the treatment of refractory DM [41],[42],[43],[44] and PM, [45],[46] in both myositis-specific autoantibody-positive and-negative patients.

PM and DM are potentially treatable diseases and an early institution of appropriate therapies is likely to be associated with early and higher remission rates and probably reduced morbidity and mortality. Better understanding of the underlying immunopathogenesis of PM and DM, as well as carefully performed clinical trials is going to be necessary to make better recommendations in the future.

 » References Top

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