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      Drugs & Diseases > Pediatrics: General Medicine

      Juvenile Dermatomyositis Medication

      Updated: May 12, 2021
      • Author: Ann M Reed, MD; Chief Editor: Lawrence K Jung, MD  more...
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      Medication

      Medication Summary

      Systemic corticosteroids are the mainstay of treatment for juvenile dermatomyositis (JDM). Other immunosuppressive and immunomodulatory agents are used as steroid-sparing agents, to lower the risk of steroid-related complications.

      Long-term use of corticosteroids is associated with toxicities such as cataracts, hypertension, a cushingoid appearance, growth failure, menstrual irregularities, avascular necrosis, and metabolic complications. [2] The risk of osteopenia and osteoporosis is also increased with long-term corticosteroid use, [107, 108] as are the risks of insulin resistance, lipodystrophy, and hypertriglyceridemia. Screening for metabolic disorders is recommended during routine follow-up visits. [109]

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      Corticosteroids

      Class Summary

      Corticosteroids are the mainstay of therapy. These agents have anti-inflammatory properties and cause profound and varied metabolic effects. In addition, these agents modify the body's immune response to diverse stimuli.

      Prednisone

      Prednisone is a first-line therapy for JDM. It may decrease inflammation by reversing increased capillary permeability and suppressing polymorphonuclear neutrophil (PMN) activity. Administration in IV pulses may be beneficial and may be associated with lower frequency of calcinosis.

      Methylprednisolone (Depo-Medrol, Medrol, Solu-Medrol)

      Methylprednisolone is used as an anti-inflammatory or immunosuppressant agent in the treatment of a variety of diseases.

      Prednisolone (Orapred ODT, Prelone, Pred Forte, Omnipred)

      Prednisolone decreases autoimmune reactions, possibly by suppressing key components of the immune system. This agent does not need to undergo hepatic metabolism.

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      Immunosuppressive and Immunomodulatory Agents

      Class Summary

      These agents inhibit immune reactions that result from diverse stimuli.

      Methotrexate (Rheumatrex, Trexall)

      Methotrexate has benefits in both muscle and skin disease. Its mechanism of action in treatment of inflammatory reactions is unknown. It may affect immune function. Methotrexate ameliorates symptoms of inflammation (eg, pain, swelling, stiffness).

      This agent is an antimetabolite that inhibits DNA synthesis and cell reproduction in malignant cells. It may suppress the immune system. A satisfactory response may be seen 3-6 wk following initiation of treatment. Gradually adjust dose to attain satisfactory response.

      It is used early in the course as a steroid-sparing agent to lower the risk of steroid-related complications.

      Cyclosporine (Neoral, Sandimmune, Gengraf)

      An 11-amino acid cyclic peptide and natural product of fungi, cyclosporine acts on T-cell replication and activity. It is a specific modulator of T-cell function and depresses cell-mediated immune responses by inhibiting helper T-cell function. Preferential and reversible inhibition of T lymphocytes in G0 or G1 phase of cell cycle has been suggested.

      Cyclosporine binds to cyclophilin, an intracellular protein, which, in turn, prevents formation of interleukin 2 and the subsequent recruitment of activated T cells. It has about 30% bioavailability but this widely varies. Cyclosporine specifically inhibits T-lymphocyte function, with minimal activity against B cells. Maximum suppression of T-lymphocyte proliferation requires that drug be present during first 24 h of antigenic exposure.

      Cyclosporine suppresses some humoral immunity and, to a greater extent, cell-mediated immune reactions (eg, delayed hypersensitivity, allograft rejection, experimental allergic encephalomyelitis, and graft-vs-host disease) for various organs.

      Mycophenolate (CellCept, Myfortic)

      Mycophenolate is useful for both skin and muscle disease. It inhibits purine synthesis and proliferation of human lymphocytes.

      Infliximab (Remicade)

      Infliximab is a tumor necrosis factor (TNF) antagonist that is a chimeric human-murine monoclonal antibody. It blocks the effects of TNF-alpha by inhibiting receptor binding.

      Immune globulin intravenous (Carimune, Gammagard S/D, Gamunex, Octagam, Gammaplex)

      Intravenous immune globulin (IVIg) is used for patients in whom corticosteroids and immunosuppressive agents have failed. IVIg downregulates proinflammatory cytokines, including interferon-gamma; blocks Fc receptors on macrophages; suppresses inducer T and B cells and augments suppressor T cells; blocks complement cascade; promotes remyelination; may increase CSF IgG (10%).

      Hydroxychloroquine (Plaquenil)

      Hydroxychloroquine may allow partial or complete control of the skin manifestations in JDM. Anecdotal reports suggest that morbilliform drug reactions are more common in patients with JDM than in other collagen vascular diseases. This agent inhibits chemotaxis of eosinophils and locomotion of neutrophils and impairs complement-dependent antigen-antibody reactions.

      Cyclophosphamide

      Cyclophosphamide is an alkylating agent of the nitrogen mustard family that exerts cytotoxic effects by binding to nucleic acids and crosslinking DNA and RNA strands and inhibiting protein synthesis.

      Azathioprine (Imuran)

      Azathioprine is an imidazolyl derivative of 6-mercaptopurine. Many of its biological effects are similar to those of the parent compound. Both compounds are rapidly eliminated from blood and are oxidized or methylated in erythrocytes and liver. No azathioprine or mercaptopurine is detectable in urine 8 h after ingestion.

      Azathioprine antagonizes purine metabolism and inhibits synthesis of DNA, RNA, and proteins. The mechanism whereby azathioprine affects autoimmune diseases is unknown.

      This agent works primarily on T cells. It suppresses cell-mediated hypersensitivities and causes variable alterations in antibody production. Immunosuppressive, delayed hypersensitivity, and cellular cytotoxicity are suppressed to a greater degree than antibody responses.

      Azathioprine works very slowly; a 6-12 mo trial may be needed before the drug takes effect. Up to 10% of patients may have an idiosyncratic reaction that disallows use of this agent. Do not allow WBC count to drop below 3000/mL or the lymphocyte count to drop below 1000/mL.

      Azathioprine is available in tablet form for oral administration and in 100-mg vials for IV injection.

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      Calcium Channel Blockers

      Class Summary

      Consider using these agents for calcinosis cutis.

      Diltiazem (Cardizem, Cardizem CD, Tiazac, Dilacor XR, Cartia XT)

      During depolarization, diltiazem inhibits the influx of extracellular calcium across both the myocardial and vascular smooth muscle cell membranes. Serum calcium levels remain unchanged. The resultant decrease in intracellular calcium inhibits the contractile processes of myocardial smooth muscle cells, resulting in dilation of the coronary and systemic arteries and improved oxygen delivery to the myocardial tissue.

      Diltiazem decreases conduction velocity in the AV node. It also increases the refractory period via blockade of calcium influx. This, in turn, stops reentrant phenomena.

      This agent decreases myocardial oxygen demand by reducing peripheral vascular resistance, reducing heart rate by slowing conduction through SA and AV nodes, and reducing LV inotropy. It slows AV nodal conduction time and prolongs the AV nodal refractory period, which may convert supraventricular tachycardia or slow the rate in atrial fibrillation. It also has vasodilator activity but may be less potent than other agents. Total peripheral resistance, systemic blood pressure, and afterload are decreased.

      Calcium channel blockers provide control of hypertension associated with less impairment of function of the ischemic kidney. Calcium channel blockers may have beneficial long-term effects, but this remains uncertain.

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