Juvenile Dermatomyositis Treatment & Management
- Author: Ann M Reed, MD; Chief Editor: Lawrence K Jung, MD more...
A multidisciplinary approach is required to prevent and reduce long-term morbidity in juvenile dermatomyositis (JDM). Various therapies are used to treat skin manifestations of JDM. For active muscle disease, oral corticosteroids are the mainstay of treatment. Second-line agents are routinely added for steroid-sparing effects and for recalcitrant or refractory disease.
Cyclophosphamide at a dose of 0.5-1 g/m2 may be given with bladder protection on a monthly basis and adjusted for leukopenia for patients with significant morbidity such as skin or GI ulcerations, respiratory disease, or both.
For patients with dysphonia, a referral to a speech therapist is appropriate. Children with dysphagia should meet with an occupational therapist for instruction on food consistency and proper positioning of the head, chin, and tongue.
Because of the rarity of the disease, patients with JDM should be transferred to a tertiary care center for experienced clinical care in the initial course of the disease and for periodic follow-up. Patients should be followed on a regular basis, with monitoring of muscle enzymes and muscle strength every 3-6 months. Remission occurs when stable improvement or normalization of muscle strength and muscle enzymes is observed over 6 months.
Consultations may be indicated with the following:
Treatment of Skin Disease
Photosensitive rashes in patients with JDM may be exacerbated by sunlight exposure; sun avoidance, and judicious use of sunscreens that protect against ultraviolet A and B rays. U
Topical corticosteroids are used to treat skin manifestations of JDM. Hydroxychloroquine at a dose no greater than 5-7 mg/kg/day may be beneficial. Topical tacrolimus (0.1%) has been used to treat cutaneous disease, but this use is falling out of favor. Methotrexate, which is an established second-line agent for muscle involvement in JDM, may also be effective for cutaneous manifestations.
Medical therapy for calcinosis in JDM has been controversial due to inconsistent responses. Diltiazem at doses of 240-480 mg (3-6 mg/kg/day) has been used with varying success rates.[1, 95, 96] Other agents used with little success include bisphosphonates, aluminum hydroxide, probenecid, and local corticosteroid injections. Calcinosis cutis may regress over time.
Areas of calcinosis that cause significant disfigurement, pain, or compromised physical function may be resected surgically. Resection is not usually recommended, however, as areas of resection may be complicated by draining sinuses, infections, and ulcerations; the calcinosis may recur.
Treatment of Muscle Disease
For active muscle disease, oral corticosteroids are the mainstay of treatment. High doses (1-2 mg/kg/day) are initially used until improvement or for 4-6 weeks, with a slow taper to avoid relapse. Weaning of steroids often occurs over 1-2 years. For refractory or severe disease, pulse therapy with intravenous methylprednisolone is used at a dose of 30 mg/kg, with a maximum dose of 1 g daily for 3 days with efficacy. Calcium and vitamin D supplementation is recommended for patients on long-term corticosteroid therapy.
Second-line agents are routinely added for steroid-sparing effects and for recalcitrant or refractory disease. Methotrexate has been the most widely accepted agent and is used at doses of 10-20 mg/m2 per week orally or subcutaneously with 1 mg/d of folic acid supplementation.
Methotrexate administration may be started at the outset of severe disease (eg, moderate to severe weakness, manifestations of vasculopathy) or started if patients fail to respond to high-dose corticosteroids within 6 weeks. The use of methotrexate in conjunction with a tapering course of prednisone may be effective in reducing the long-term cumulative dose of corticosteroids.[98, 96]
Cyclosporin A has been used as an alternative, effective, steroid-sparing agent in JDM. Preliminary data also suggest that the use of cyclosporine A in combination with methotrexate may be associated with further improvement in clinical outcome.
Intravenous immunoglobulin (IVIG) may also be used in patients with JDM who are steroid-resistant or steroid-dependent at a dose of 1 g/kg on 2 consecutive days, or 2 g/kg in one day, then every month thereafter, generally for a 6-month course. Other second-line agents include azathioprine and mycophenolate mofetil.
Biologic agents such as the anti–tumor necrosis factor agents have had mixed results. More recently, infliximab has shown major clinical benefit in 5 patients with refractory JDM. Another promising agent for JDM is the anti-CD20 monoclonal antibody rituximab. In one trial of adults with refractory polymyositis and adults and children with refractory dermatomyositis, 200 patients were randomized to either rituximab early or rituximab late. Glucocorticoid and immunosuppressive therapy were allowed at entry. While no significant differences in outcomes between treatment groups were observed, 83% of refractory adult and juvenile myositis patients met the International Myositis Assessment and Clinical Studies Group definition of improvement.
Rehabilitation plays an active role in the first-line management of myositis to restore and maintain muscle strength and endurance and to prevent contractures. If the myositis is active, then passive range of motion exercises are recommended; pool therapy may also be helpful.
When the disease is stabilized, isometric and isotonic exercises have been recommended. However, one study indicated that an intensive muscular training program involving resistance training may be beneficial and safe without worsening of muscle inflammation.
For patients with severe contractures, physical and occupational therapy with assistive devices is recommended.
Diet and Activity
No special diet is advised for most patients with JDM, although calcium and vitamin D supplementation are usually recommended for those using corticosteroids. Patients who have dysphagia may have a consistency-limited diet as recommended by a physical therapist.
Vigorous physical activity should be avoided if the child has instabilities, severe fatigue, or concerns of falling or aspiration. Bed rest is not indicated. Protective measures against sun exposure are recommended.
Rider LG, Miller FW. Classification and treatment of the juvenile idiopathic inflammatory myopathies. Rheum Dis Clin North Am. 1997 Aug. 23(3):619-55. [Medline].
Rider LG, Miller FW. Idiopathic inflammatory muscle disease: clinical aspects. Baillieres Best Pract Res Clin Rheumatol. 2000 Mar. 14(1):37-54. [Medline].
Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med. 1975 Feb 13. 292(7):344-7. [Medline].
Bohan A, Peter JB. Polymyositis and dermatomyositis (second of two parts). N Engl J Med. 1975 Feb 20. 292(8):403-7. [Medline].
Brown VE, Pilkington CA, Feldman BM, Davidson JE,. An international consensus survey of the diagnostic criteria for juvenile dermatomyositis (JDM). Rheumatology (Oxford). 2006 Aug. 45(8):990-3. [Medline].
Spencer CH, Hanson V, Singsen BH, Bernstein BH, Kornreich HK, King KK. Course of treated juvenile dermatomyositis. J Pediatr. 1984 Sep. 105(3):399-408. [Medline].
Constantin T, Ponyi A, Orban I, Molnar K, Derfalvi B, Dicso F. National registry of patients with juvenile idiopathic inflammatory myopathies in Hungary--clinical characteristics and disease course of 44 patients with juvenile dermatomyositis. Autoimmunity. 2006 May. 39(3):223-32. [Medline].
Gerami P, Walling HW, Lewis J, Doughty L, Sontheimer RD. A systematic review of juvenile-onset clinically amyopathic dermatomyositis. Br J Dermatol. 2007 Oct. 157(4):637-44. [Medline].
Hiketa T, Matsumoto Y, Ohashi M, Sasaki R. Juvenile dermatomyositis: a statistical study of 114 patients with dermatomyositis. J Dermatol. 1992 Aug. 19(8):470-6. [Medline].
Engel AG, Arahata K, Emslie-Smith A. Immune effector mechanisms in inflammatory myopathies. Res Publ Assoc Res Nerv Ment Dis. 1990. 68:141-57. [Medline].
Engel AG, Arahata K. Mononuclear cells in myopathies: quantitation of functionally distinct subsets, recognition of antigen-specific cell-mediated cytotoxicity in some diseases, and implications for the pathogenesis of the different inflammatory myopathies. Hum Pathol. 1986 Jul. 17(7):704-21. [Medline].
Crowe WE, Bove KE, Levinson JE, Hilton PK. Clinical and pathogenetic implications of histopathology in childhood polydermatomyositis. Arthritis Rheum. 1982 Feb. 25(2):126-39. [Medline].
Dalakas MC, Hohlfeld R. Polymyositis and dermatomyositis. Lancet. 2003 Sep 20. 362(9388):971-82. [Medline].
Li CK, Varsani H, Holton JL, Gao B, Woo P, Wedderburn LR. MHC Class I overexpression on muscles in early juvenile dermatomyositis. J Rheumatol. 2004 Mar. 31(3):605-9. [Medline].
Greenberg SA. Proposed immunologic models of the inflammatory myopathies and potential therapeutic implications. Neurology. 2007 Nov 20. 69(21):2008-19. [Medline].
Lopez de Padilla CM, Vallejo AN, McNallan KT, Vehe R, Smith SA, Dietz AB. Plasmacytoid dendritic cells in inflamed muscle of patients with juvenile dermatomyositis. Arthritis Rheum. 2007 May. 56(5):1658-68. [Medline].
Kissel JT, Mendell JR, Rammohan KW. Microvascular deposition of complement membrane attack complex in dermatomyositis. N Engl J Med. 1986 Feb 6. 314(6):329-34. [Medline].
Dalakas MC. Muscle biopsy findings in inflammatory myopathies. Rheum Dis Clin North Am. 2002 Nov. 28(4):779-98, vi. [Medline].
Chevrel G, Page G, Granet C, Streichenberger N, Varennes A, Miossec P. Interleukin-17 increases the effects of IL-1 beta on muscle cells: arguments for the role of T cells in the pathogenesis of myositis. J Neuroimmunol. 2003 Apr. 137(1-2):125-33. [Medline].
Bettelli E, Korn T, Oukka M, Kuchroo VK. Induction and effector functions of T(H)17 cells. Nature. 2008 Jun 19. 453(7198):1051-7. [Medline].
Dong C. TH17 cells in development: an updated view of their molecular identity and genetic programming. Nat Rev Immunol. 2008 May. 8(5):337-48. [Medline].
López De Padilla CM, Vallejo AN, Lacomis D, McNallan K, Reed AM. Extranodal lymphoid microstructures in inflamed muscle and disease severity of new-onset juvenile dermatomyositis. Arthritis Rheum. 2009 Apr. 60(4):1160-72. [Medline].
Tezak Z, Hoffman EP, Lutz JL, Fedczyna TO, Stephan D, Bremer EG. Gene expression profiling in DQA1*0501+ children with untreated dermatomyositis: a novel model of pathogenesis. J Immunol. 2002 Apr 15. 168(8):4154-63. [Medline]. [Full Text].
Nagaraju K, Rider LG, Fan C, Chen YW, Mitsak M, Rawat R, et al. Endothelial cell activation and neovascularization are prominent in dermatomyositis. J Autoimmune Dis. 2006 Feb 20. 3:2. [Medline]. [Full Text].
Liu YJ. IPC: professional type 1 interferon-producing cells and plasmacytoid dendritic cell precursors. Annu Rev Immunol. 2005. 23:275-306. [Medline].
Greenberg SA, Pinkus JL, Pinkus GS, Burleson T, Sanoudou D, Tawil R. Interferon-alpha/beta-mediated innate immune mechanisms in dermatomyositis. Ann Neurol. 2005 May. 57(5):664-78. [Medline].
McNiff JM, Kaplan DH. Plasmacytoid dendritic cells are present in cutaneous dermatomyositis lesions in a pattern distinct from lupus erythematosus. J Cutan Pathol. 2008 May. 35(5):452-6. [Medline].
Symmons DP, Sills JA, Davis SM. The incidence of juvenile dermatomyositis: results from a nation-wide study. Br J Rheumatol. 1995 Aug. 34(8):732-6. [Medline].
Pachman LM, Hayford JR, Chung A, Daugherty CA, Pallansch MA, Fink CW. Juvenile dermatomyositis at diagnosis: clinical characteristics of 79 children. J Rheumatol. 1998 Jun. 25(6):1198-204. [Medline].
Massa M, Costouros N, Mazzoli F, et al. Self Epitopes shared between human skeletal myosin and Streptococcus pyogenes M5 protein are targets of immune responses in active juvenile dermatomyositis. Arthritis Rheum. 2002. 46(11):3015-3025.
Christensen ML, Pachman LM, Schneiderman R, Patel DC, Friedman JM. Prevalence of Coxsackie B virus antibodies in patients with juvenile dermatomyositis. Arthritis Rheum. 1986 Nov. 29(11):1365-70. [Medline].
Lewkonia RM, Horne D, Dawood MR. Juvenile dermatomyositis in a child infected with human parvovirus B19. Clin Infect Dis. 1995 Aug. 21(2):430-2. [Medline].
Zampieri S, Ghirardello A, Iaccarino L, Briani C, Sarzi-Puttini P, Atzeni F. Polymyositis-dermatomyositis and infections. Autoimmunity. 2006 May. 39(3):191-6. [Medline].
Albani, S. Infection and molecular mimicry in autoimmune diseases of childhood. Clin Exp Rheumatol. 1994. 12 (Suppl 10):S35-41.
Baechler EC, Bauer JW, Slattery CA, Ortmann WA, Espe KJ, Novitzke J, et al. An interferon signature in the peripheral blood of dermatomyositis patients is associated with disease activity. Mol Med. 2007 Jan-Feb. 13(1-2):59-68. [Medline]. [Full Text].
Walsh RJ, Kong SW, Yao Y, Jallal B, Kiener PA, Pinkus JL, et al. Type I interferon-inducible gene expression in blood is present and reflects disease activity in dermatomyositis and polymyositis. Arthritis Rheum. 2007 Nov. 56(11):3784-92. [Medline]. [Full Text].
Greenberg SA, Pinkus JL, Pinkus GS, et al. Interferon-alpha/beta-mediated innate immune mechanisms in dermatomyositis. Ann Neurol. 2005 May. 57(5):664-78. [Medline].
Dourmishev AL, Dourmishev LA. Dermatomyositis and drugs. Adv Exp Med Biol. 1999. 455:187-91. [Medline].
Kass E, Straume S, Mellbye OJ, Munthe E, Solheim BG. Dermatomyositis associated with BCG vaccination. Scand J Rheumatol. 1979. 8(3):187-91. [Medline].
Adams C, August CS, Maguire H, Sladky JT. Neuromuscular complications of bone marrow transplantation. Pediatr Neurol. 1995 Jan. 12(1):58-61. [Medline].
Reed AM, Pachman L, Ober C. Molecular genetic studies of major histocompatibility complex genes in children with juvenile dermatomyositis: increased risk associated with HLA-DQA1 *0501. Hum Immunol. 1991 Dec. 32(4):235-40. [Medline].
Reed AM, Stirling JD. Association of the HLA-DQA1*0501 allele in multiple racial groups with juvenile dermatomyositis. Hum Immunol. 1995 Nov. 44(3):131-5. [Medline].
Mamyrova G, O'Hanlon TP, Monroe JB, Carrick DM, Malley JD, Adams S, et al. Immunogenetic risk and protective factors for juvenile dermatomyositis in Caucasians. Arthritis Rheum. 2006 Dec. 54(12):3979-87. [Medline]. [Full Text].
Reed AM, Picornell YJ, Harwood A, Kredich DW. Chimerism in children with juvenile dermatomyositis. Lancet. 2000 Dec 23-30. 356(9248):2156-7. [Medline].
Artlett CM, Ramos R, Jiminez SA, Patterson K, Miller FW, Rider LG. Chimeric cells of maternal origin in juvenile idiopathic inflammatory myopathies. Childhood Myositis Heterogeneity Collaborative Group. Lancet. 2000 Dec 23-30. 356(9248):2155-6. [Medline].
Pachman LM, Liotta-Davis MR, Hong DK, Kinsella TR, Mendez EP, Kinder JM. TNFalpha-308A allele in juvenile dermatomyositis: association with increased production of tumor necrosis factor alpha, disease duration, and pathologic calcifications. Arthritis Rheum. 2000 Oct. 43(10):2368-77. [Medline].
Fedczyna TO, Lutz J, Pachman LM. Expression of TNFalpha by muscle fibers in biopsies from children with untreated juvenile dermatomyositis: association with the TNFalpha-308A allele. Clin Immunol. 2001 Aug. 100(2):236-9. [Medline].
Rider LG, Artlett CM, Foster CB, Ahmed A, Neeman T, Chanock SJ, et al. Polymorphisms in the IL-1 receptor antagonist gene VNTR are possible risk factors for juvenile idiopathic inflammatory myopathies. Clin Exp Immunol. 2000 Jul. 121(1):47-52. [Medline]. [Full Text].
Kelly JC. Juvenile Dermatomyositis May Be Triggered by UV Exposure. Medscape Medical News. Available at http://www.medscape.com/viewarticle/807438. Accessed: March 19, 2015.
Shah M, Targoff IN, Rice MM, Miller FW, Rider LG. Brief report: ultraviolet radiation exposure is associated with clinical and autoantibody phenotypes in juvenile myositis. Arthritis Rheum. 2013 Jul. 65(7):1934-41. [Medline]. [Full Text].
Mendez EP, Lipton R, Ramsey-Goldman R, Roettcher P, Bowyer S, Dyer A. US incidence of juvenile dermatomyositis, 1995-1998: results from the National Institute of Arthritis and Musculoskeletal and Skin Diseases Registry. Arthritis Rheum. 2003 Jun 15. 49(3):300-5. [Medline].
Pachman LM, Lipton R, Ramsey-Goldman R, Shamiyeh E, Abbott K, Mendez EP. History of infection before the onset of juvenile dermatomyositis: results from the National Institute of Arthritis and Musculoskeletal and Skin Diseases Research Registry. Arthritis Rheum. 2005 Apr 15. 53(2):166-72. [Medline].
Bitnum S, Daeschner CW Jr, Travis LB, Dodge WF, Hopps HC. Dermatomyositis. J Pediatrics. 1964. 64:101-31.
Huber AM, Feldman BM. Long-term outcomes in juvenile dermatomyositis: how did we get here and where are we going?. Curr Rheumatol Rep. 2005/12. 7(6):441-6.
Huber AM, Lang B, LeBlanc CM, Birdi N, Bolaria RK, Malleson P. Medium- and long-term functional outcomes in a multicenter cohort of children with juvenile dermatomyositis. Arthritis Rheum. 2000 Mar. 43(3):541-9. [Medline].
Miller LC, Michael F, Kim Y. Childhood dermatomyositis: Clinical course and long-term follow-up. Clin Pediatr. 1987. 26(11):5661-6.
Taieb A, Guichard C, Salamon R, Maleville J. Prognosis in juvenile dermatopolymyositis: a cooperative retrospective study of 70 cases. Pediatr Dermatol. 1985 Jul. 2(4):275-81. [Medline].
Huber AM, Feldman BM, Rennebohm RM, Hicks JE, Lindsley CB, Perez MD. Validation and clinical significance of the Childhood Myositis Assessment Scale for assessment of muscle function in the juvenile idiopathic inflammatory myopathies. Arthritis Rheum. 2004 May. 50(5):1595-603. [Medline].
Rider LG. Calcinosis in juvenile dermatomyositis: pathogenesis and current therapies. Pediatric Rheumatology Online Journal. 2003. 1(2):[Full Text].
Boulman N, Slobodin G, Rozenbaum M, Rosner I. Calcinosis in rheumatic diseases. Semin Arthritis Rheum. 2005 Jun. 34(6):805-12. [Medline].
Hundley JL, Carroll CL, Lang W, Snively B, Yosipovitch G, Feldman SR. Cutaneous symptoms of dermatomyositis significantly impact patients' quality of life. J Am Acad Dermatol. 2006 Feb. 54(2):217-20. [Medline].
Sallum AM, Pivato FC, Doria-Filho U, et al. Risk factors associated with calcinosis of juvenile dermatomyositis. J Pediatr (Rio J). 2008. 84(1):68-74.
Peloro TM, Miller OF 3rd, Hahn TF, Newman ED. Juvenile dermatomyositis: a retrospective review of a 30-year experience. J Am Acad Dermatol. 2001 Jul. 45(1):28-34. [Medline].
Braunstein EM, White SJ. Pneumatosis intestinalis in dermatomyositis. Br J Radiol. 1980 Oct. 53(634):1011-2. [Medline].
Stefanski JC, Shetty AK. Abdominal pain in a girl with juvenile dermatomyositis. Clin Pediatr (Phila). 1998. 37(9):561-563.
Santmyire-Rosenberger B, Dugan EM. Skin involvement in dermatomyositis. Curr Opin Rheumatol. 2003 Nov. 15(6):714-22. [Medline].
Smith RL, Sundberg J, Shamiyah E, Dyer A, Pachman LM. Skin involvement in juvenile dermatomyositis is associated with loss of end row nailfold capillary loops. J Rheumatol. 2004 Aug. 31(8):1644-9. [Medline].
García-Patos V, Bartralot R, Fonollosa V, Arnal C, Boronat M, Gelpí C. Childhood sclerodermatomyositis: report of a case with the anti-PM/Scl antibody and mechanic's hands. Br J Dermatol. 1996 Oct. 135(4):613-6. [Medline].
Pachman LM, Veis A, Stock S, Abbott K, Vicari F, Patel P, et al. Composition of calcifications in children with juvenile dermatomyositis: association with chronic cutaneous inflammation. Arthritis Rheum. 2006 Oct. 54(10):3345-50. [Medline]. [Full Text].
Wananukul S, Pongprasit P, Wattanakrai P. Calcinosis cutis presenting years before other clinical manifestations of juvenile dermatomyositis: report of two cases. Australas J Dermatol. 1997 Nov. 38(4):202-5. [Medline].
Blane CE, White SJ, Braunstein EM, Bowyer SL, Sullivan DB. Patterns of calcification in childhood dermatomyositis. AJR Am J Roentgenol. 1984 Feb. 142(2):397-400. [Medline].
Targoff IN. Myositis specific autoantibodies. Curr Rheumatol Rep. 2006 Jun. 8(3):196-203. [Medline].
Dolezalova P, Young SP, Bacon PA, Southwood TR. Nailfold capillary microscopy in healthy children and in childhood rheumatic diseases: a prospective single blind observational study. Ann Rheum Dis. 2003 May. 62(5):444-9. [Medline]. [Full Text].
Lamminen A, Jääskeläinen J, Rapola J, Suramo I. High-frequency ultrasonography of skeletal muscle in children with neuromuscular disease. J Ultrasound Med. 1988 Sep. 7(9):505-9. [Medline].
Love LA, Leff RL, Fraser DD, et al. A new approach to the classification of idiopathic inflammatory myopathy: myositis-specific autoantibodies define useful homogenous patient groups. Medicine (Baltimore). 1991. 70(6):360-74.
Chmiel JF, Wessel HU, Targoff IN, Pachman LM. Pulmonary fibrosis and myositis in a child with anti-Jo-1 antibody. J Rheumatol. 1995 Apr. 22(4):762-5. [Medline].
Rider LG, Miller FW, Targoff IN, Sherry DD, Samayoa E, Lindahl M. A broadened spectrum of juvenile myositis. Myositis-specific autoantibodies in children. Arthritis Rheum. 1994 Oct. 37(10):1534-8. [Medline].
Leff RL, Burgess SH, Miller FW, Love LA, Targoff IN, Dalakas MC. Distinct seasonal patterns in the onset of adult idiopathic inflammatory myopathy in patients with anti-Jo-1 and anti-signal recognition particle autoantibodies. Arthritis Rheum. 1991 Nov. 34(11):1391-6. [Medline].
Targoff IN, Mamyrova G, Trieu EP, et al. A novel autoantibody to a 155-kd protein is associated with dermatomyositis. Arthritis Rheum. 2006. 54(11):3682-3689.
Gunawardena H, Wedderburn LR, North J, et al. Clinical associations of autoantibodies to a p155/140 kDa doublet protein in juvenile dermatomyositis. Rheumatology (Oxford). 2008. 47(3):324-328.
Fraser DD, Frank JA, Dalakas M, Miller FW, Hicks JE, Plotz P. Magnetic resonance imaging in the idiopathic inflammatory myopathies. J Rheumatol. 1991 Nov. 18(11):1693-700. [Medline].
Summers RM, Brune AM, Choyke PL, Chow CK, Patronas NJ, Miller FW. Juvenile idiopathic inflammatory myopathy: exercise-induced changes in muscle at short inversion time inversion-recovery MR imaging. Radiology. 1998 Oct. 209(1):191-6. [Medline].
Miles L, Bove KE, Lovell D, Wargula JC, Bukulmez H, Shao M. Predictability of the clinical course of juvenile dermatomyositis based on initial muscle biopsy: a retrospective study of 72 patients. Arthritis Rheum. 2007 Oct 15. 57(7):1183-91. [Medline].
Wedderburn LR, Varsani H, Li CK, et al. International consensus on a proposed score system for muscle biopsy evaluation in patients with juvenile dermatomyositis:a tool for potential use in clinical trials. Arthritis Rheum. 2007. 57(7):1192-201.
Feldman BM, Ayling-Campos A, Luy L, Stevens D, Silverman ED, Laxer RM. Measuring disability in juvenile dermatomyositis: validity of the childhood health assessment questionnaire. J Rheumatol. 1995 Feb. 22(2):326-31. [Medline].
Pachman LM, Abbott K, Sinacore JM, Amoruso L, Dyer A, Lipton R. Duration of illness is an important variable for untreated children with juvenile dermatomyositis. J Pediatr. 2006 Feb. 148(2):247-53. [Medline].
Huber AM, Dugan EM, Lachenbruch PA, Feldman BM, Perez MD, Zemel LS. Preliminary validation and clinical meaning of the Cutaneous Assessment Tool in juvenile dermatomyositis. Arthritis Rheum. 2008 Feb 15. 59(2):214-21. [Medline].
Riley P, Maillard SM, Wedderburn LR, Woo P, Murray KJ, Pilkington CA. Intravenous cyclophosphamide pulse therapy in juvenile dermatomyositis. A review of efficacy and safety. Rheumatology (Oxford). 2004. 43(4):491-496.
Hollar CB, Jorizzo JL. Topical tacrolimus 0.1% ointment for refractory skin disease in dermatomyositis: a pilot study. J Dermatolog Treat. 2004 Jan. 15(1):35-9. [Medline].
Zieglschmid-Adams ME, Pandya AG, Cohen SB, Sontheimer RD. Treatment of dermatomyositis with methotrexate. J Am Acad Dermatol. 1995 May. 32(5 Pt 1):754-7. [Medline].
Ichiki Y, Akiyama T, Shimozawa N, Suzuki Y, Kondo N, Kitajima Y. An extremely severe case of cutaneous calcinosis with juvenile dermatomyositis, and successful treatment with diltiazem. Br J Dermatol. 2001 Apr. 144(4):894-7. [Medline].
Stringer E, Feldman BM. Advances in the treatment of juvenile dermatomyositis. Curr Opin Rheumatol. 2006 Sep. 18(5):503-6. [Medline].
Huang JL. Long-term prognosis of patients with juvenile dermatomyositis initially treated with intravenous methylprednisolone pulse therapy. Clin Exp Rheumatol. 1999 Sep-Oct. 17(5):621-4. [Medline].
Ramanan AV, Campbell-Webster N, Ota S, Parker S, Tran D, Tyrrell PN. The effectiveness of treating juvenile dermatomyositis with methotrexate and aggressively tapered corticosteroids. Arthritis Rheum. 2005 Nov. 52(11):3570-8. [Medline].
Reiff A, Rawlings DJ, Shaham B, Franke E, Richardson L, Szer IS. Preliminary evidence for cyclosporin A as an alternative in the treatment of recalcitrant juvenile rheumatoid arthritis and juvenile dermatomyositis. J Rheumatol. 1997 Dec. 24(12):2436-43. [Medline].
Riley P, McCann LJ, Maillard SM, Woo P, Murray KJ, Pilkington CA. Effectiveness of infliximab in the treatment of refractory juvenile dermatomyositis with calcinosis. Rheumatology (Oxford). April 2008. [Full Text].
Cooper MA, Willingham DL, Brown DE, French AR, Shih FF, White AJ. Rituximab for the treatment of juvenile dermatomyositis: a report of four pediatric patients. Arthritis Rheum. 2007 Sep. 56(9):3107-11. [Medline].
Oddis CV, Reed AM, Aggarwal R, Rider LG, Ascherman DP, Levesque MC, et al. Rituximab in the treatment of refractory adult and juvenile dermatomyositis and adult polymyositis: A randomized, placebo-phase trial. Arthritis Rheum. 2012 Nov 2. [Medline].
Alexanderson H, Dastmalchi M, Esbjörnsson-Liljedahl M, Opava CH, Lundberg IE. Benefits of intensive resistance training in patients with chronic polymyositis or dermatomyositis. Arthritis Rheum. 2007 Jun 15. 57(5):768-77. [Medline].
Stewart WA, Acott PD, Salisbury SR, Lang BA. Bone mineral density in juvenile dermatomyositis: assessment using dual x-ray absorptiometry. Arthritis Rheum. 2003 Aug. 48(8):2294-8. [Medline].
Perez MD, Abrams SA, Koenning G, Stuff JE, O'Brien KO, Ellis KJ. Mineral metabolism in children with dermatomyositis. J Rheumatol. 1994 Dec. 21(12):2364-9. [Medline].
Huemer C, Kitson H, Malleson PN, Sanderson S, Huemer M, Cabral DA. Lipodystrophy in patients with juvenile dermatomyositis--evaluation of clinical and metabolic abnormalities. J Rheumatol. 2001 Mar. 28(3):610-5. [Medline].