Semin Neurol 2012; 32(03): 227-236
DOI: 10.1055/s-0032-1329201
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Idiopathic Inflammatory Myopathies

Mazen M. Dimachkie
1   Department of Neurology, The University of Kansas Medical Center, Kansas City, Kansas
,
Richard J. Barohn
1   Department of Neurology, The University of Kansas Medical Center, Kansas City, Kansas
› Author Affiliations
Further Information

Publication History

Publication Date:
01 November 2012 (online)

Abstract

The idiopathic inflammatory myopathies are a group of rare disorders including polymyositis (PM), dermatomyositis (DM), and autoimmune necrotizing myopathies (NMs). The idiopathic inflammatory myopathies share many similarities. They present acutely, subacutely, or chronically with marked proximal and symmetric muscle weakness, except for associated distal and asymmetric weakness in inclusion body myositis. The idiopathic inflammatory myopathies also share a variable degree of creatine kinase (CK) elevation and a nonspecifically abnormal electromyogram demonstrating an irritative myopathy. The muscle pathology demonstrates inflammatory exudates of variable distribution within the muscle fascicle. Despite these similarities, the idiopathic inflammatory myopathies are a heterogeneous group. The overlap syndrome (OS) refers to the association of PM, DM, or NM with connective tissue disease, such as scleroderma or systemic lupus erythematosus. In addition to elevated antinuclear antibodies (ANA), patients with OS may be weaker in the proximal arms than the legs mimicking the pattern seen in some muscular dystrophies. In this review, we focus on DM, PM, and NM and examine current and promising therapies.

 
  • References

  • 1 Dimachkie MM, Barohn RJ. Idiopathic inflammatory myopathies. Front Neurol Neurosci 2009; 26: 126-146
  • 2 van der Meulen MFG, Bronner IM, Hoogendijk JE , et al. Polymyositis: an overdiagnosed entity. Neurology 2003; 61 (3) 316-321
  • 3 Chahin N, Engel AG. Correlation of muscle biopsy, clinical course, and outcome in PM and sporadic IBM. Neurology 2008; 70 (6) 418-424
  • 4 Kuo CF, See LC, Yu KH , et al. Incidence, cancer risk and mortality of dermatomyositis and polymyositis in Taiwan: a nationwide population study. Br J Dermatol 2011; 165 (6) 1273-1279
  • 5 Quinter SD, Chiu YE, Lyon VB, Holland KE, Ruggeri SY, Drolet BA. Inverse Gottron's papules: an unusual cutaneous manifestation of juvenile dermatomyositis. Pediatr Dermatol 2012; 29 (5) 641-644
  • 6 Schmeling H, Stephens S, Goia C , et al. Nailfold capillary density is importantly associated over time with muscle and skin disease activity in juvenile dermatomyositis. Rheumatology (Oxford) 2011; 50 (5) 885-893
  • 7 Shirani Z, Kucenic MJ, Carroll CL , et al. Pruritus in adult dermatomyositis. Clin Exp Dermatol 2004; 29 (3) 273-276
  • 8 Hundley JL, Carroll CL, Lang W , et al. Cutaneous symptoms of dermatomyositis significantly impact patients' quality of life. J Am Acad Dermatol 2006; 54 (2) 217-220
  • 9 Hoogendijk JE, Amato AA, Lecky BR , et al. 119th ENMC international workshop: trial design in adult idiopathic inflammatory myopathies, with the exception of inclusion body myositis, 10-12 October 2003, Naarden, The Netherlands. Neuromuscul Disord 2004; 14 (5) 337-345
  • 10 Bohan A, Peter JB. Polymyositis and dermatomyositis (second of two parts). N Engl J Med 1975; 292 (8) 403-407
  • 11 Amato AA, Griggs RC. Unicorns, dragons, polymyositis and other mythical beasts. Neurol (Tokyo) 2003; 61: 288-289
  • 12 Amato AA, Barohn RJ. Idiopathic inflammatory myopathies. Neurol Clin 1997; 15 (3) 615-648
  • 13 Dalakas MC, Hohlfeld R. Polymyositis and dermatomyositis. Lancet 2003; 362 (9388) 971-982
  • 14 Pal S, Sanyal D. Jaw muscle weakness: a differential indicator of neuromuscular weakness–preliminary observations. Muscle Nerve 2011; 43 (6) 807-811
  • 15 Ellis E, Ann Tan J, Lester S , et al. Necrotizing myopathy: clinicoserologic associations. Muscle Nerve 2012; 45 (2) 189-194
  • 16 Suzuki S, Ohta M, Shimizu Y, Hayashi YK, Nishino I. Anti-signal recognition particle myopathy in the first decade of life. Pediatr Neurol 2011; 45 (2) 114-116
  • 17 Grable-Esposito P, Katzberg HD, Greenberg SA, Srinivasan J, Katz J, Amato AA. Immune-mediated necrotizing myopathy associated with statins. Muscle Nerve 2010; 41 (2) 185-190
  • 18 Tymms KE, Webb J. Dermatopolymyositis and other connective tissue diseases: a review of 105 cases. J Rheumatol 1985; 12 (6) 1140-1148
  • 19 Marie I, Hatron PY, Dominique S, Cherin P, Mouthon L, Menard JF. Short-term and long-term outcomes of interstitial lung disease in polymyositis and dermatomyositis: a series of 107 patients. Arthritis Rheum 2011; 63 (11) 3439-3447
  • 20 Hochberg MC, Feldman D, Stevens MB, Arnett FC, Reichlin M. Antibody to Jo-1 in polymyositis/dermatomyositis: association with interstitial pulmonary disease. J Rheumatol 1984; 11 (5) 663-665
  • 21 Targoff IN, Miller FW, Medsger Jr TA, Oddis CV. Classification criteria for the idiopathic inflammatory myopathies. Curr Opin Rheumatol 1997; 9 (6) 527-535
  • 22 Maoz CR, Langevitz P, Livneh A , et al. High incidence of malignancies in patients with dermatomyositis and polymyositis: an 11-year analysis. Semin Arthritis Rheum 1998; 27 (5) 319-324
  • 23 Sigurgeirsson B, Lindelöf B, Edhag O, Allander E. Risk of cancer in patients with dermatomyositis or polymyositis. A population-based study. N Engl J Med 1992; 326 (6) 363-367
  • 24 Lakhanpal S, Bunch TW, Ilstrup DM, Melton III LJ. Polymyositis-dermatomyositis and malignant lesions: does an association exist?. Mayo Clin Proc 1986; 61 (8) 645-653
  • 25 Scaling ST, Kaufman RH, Patten BM. Dermatomyositis and female malignancy. Obstet Gynecol 1979; 54 (4) 474-477
  • 26 Rider LG, Miller FW. Classification and treatment of the juvenile idiopathic inflammatory myopathies. Rheum Dis Clin North Am 1997; 23 (3) 619-655
  • 27 Chen YJ, Wu CY, Huang YL, Wang CB, Shen JL, Chang YT. Cancer risks of dermatomyositis and polymyositis: a nationwide cohort study in Taiwan. Arthritis Res Ther 2010; 12 (2) R70
  • 28 Miller T, Al-Lozi MT, Lopate G, Pestronk A. Myopathy with antibodies to the signal recognition particle: clinical and pathological features. J Neurol Neurosurg Psychiatry 2002; 73 (4) 420-428
  • 29 Zöller B, Li X, Sundquist J, Sundquist K. Risk of subsequent coronary heart disease in patients hospitalized for immune-mediated diseases: a nationwide follow-up study from Sweden. PLoS ONE 2012; 7 (3) e33442
  • 30 Eimer MJ, Brickman WJ, Seshadri R , et al. Clinical status and cardiovascular risk profile of adults with a history of juvenile dermatomyositis. J Pediatr 2011; 159 (5) 795-801
  • 31 Titulaer MJ, Soffietti R, Dalmau J , et al. Screening for tumours in paraneoplastic syndromes: report of an EFNS task force. Eur J Neurol 2011; 18 (1) 19-e3
  • 32 Fathi M, Barbasso Helmers S, Lundberg IE. KL-6: a serological biomarker for interstitial lung disease in patients with polymyositis and dermatomyositis. J Intern Med 2012; 271 (6) 589-597
  • 33 Gono T, Kawaguchi Y, Sugiura T , et al. Interleukin-18 is a key mediator in dermatomyositis: potential contribution to development of interstitial lung disease. Rheumatology (Oxford) 2010; 49 (10) 1878-1881
  • 34 Ichimura Y, Matsushita T, Hamaguchi Y , et al. Anti-NXP2 autoantibodies in adult patients with idiopathic inflammatory myopathies: possible association with malignancy. Ann Rheum Dis 2012; 71 (5) 710-713
  • 35 Joffe MM, Love LA, Leff RL , et al. Drug therapy of the idiopathic inflammatory myopathies: predictors of response to prednisone, azathioprine, and methotrexate and a comparison of their efficacy. Am J Med 1993; 94 (4) 379-387
  • 36 Suzuki S. [Anti-SRP myopathy]. Rinsho Shinkeigaku 2011; 51 (11) 961-963
  • 37 Kao AH, Lacomis D, Lucas M, Fertig N, Oddis CV. Anti-signal recognition particle autoantibody in patients with and patients without idiopathic inflammatory myopathy. Arthritis Rheum 2004; 50 (1) 209-215
  • 38 Hanisch F, Müller T, Stoltenburg G, Zierz S. Unusual manifestations in two cases of necrotizing myopathy associated with SRP-antibodies. Clin Neurol Neurosurg 2012; 114 (7) 1104-1106
  • 39 Christopher-Stine L, Casciola-Rosen LA, Hong G, Chung T, Corse AM, Mammen AL. A novel autoantibody recognizing 200-kd and 100-kd proteins is associated with an immune-mediated necrotizing myopathy. Arthritis Rheum 2010; 62 (9) 2757-2766
  • 40 Degardin A, Morillon D, Lacour A, Cotten A, Vermersch P, Stojkovic T. Morphologic imaging in muscular dystrophies and inflammatory myopathies. Skeletal Radiol 2010; 39 (12) 1219-1227
  • 41 Kissel JT, Mendell JR, Rammohan KW. Microvascular deposition of complement membrane attack complex in dermatomyositis. N Engl J Med 1986; 314 (6) 329-334
  • 42 Emslie-Smith AM, Engel AG. Microvascular changes in early and advanced dermatomyositis: a quantitative study. Ann Neurol 1990; 27 (4) 343-356
  • 43 Pestronk A, Schmidt RE, Choksi R. Vascular pathology in dermatomyositis and anatomic relations to myopathology. Muscle Nerve 2010; 42 (1) 53-61
  • 44 De Visser M, Emslie-Smith AM, Engel AG. Early ultrastructural alterations in adult dermatomyositis. Capillary abnormalities precede other structural changes in muscle. J Neurol Sci 1989; 94 (1-3) 181-192
  • 45 Greenberg SA, Bradshaw EM, Pinkus JL , et al. Plasma cells in muscle in inclusion body myositis and polymyositis. Neurology 2005; 65 (11) 1782-1787
  • 46 Zhou X, Dimachkie MM, Xiong M, Tan FK, Arnett FC. cDNA microarrays reveal distinct gene expression clusters in idiopathic inflammatory myopathies. Med Sci Monit 2004; 10 (7) BR191-BR197
  • 47 Walsh RJ, Kong SW, Yao Y , et al. Type I interferon-inducible gene expression in blood is present and reflects disease activity in dermatomyositis and polymyositis. Arthritis Rheum 2007; 56 (11) 3784-3792
  • 48 Liao AP, Salajegheh M, Nazareno R, Kagan JC, Jubin RG, Greenberg SA. Interferon β is associated with type 1 interferon-inducible gene expression in dermatomyositis. Ann Rheum Dis 2011; 70 (5) 831-836
  • 49 Wong D, Kea B, Pesich R , et al. Interferon and biologic signatures in dermatomyositis skin: specificity and heterogeneity across diseases. PLoS ONE 2012; 7 (1) e29161
  • 50 Salaroli R, Baldin E, Papa V , et al. Validity of internal expression of the major histocompatibility complex class I in the diagnosis of inflammatory myopathies. J Clin Pathol 2012; 65 (1) 14-19
  • 51 Greenberg SA, Pinkus GS, Amato AA, Pinkus JL. Myeloid dendritic cells in inclusion-body myositis and polymyositis. Muscle Nerve 2007; 35 (1) 17-23
  • 52 Greenberg SA, Pinkus JL, Pinkus GS , et al. Interferon-alpha/beta-mediated innate immune mechanisms in dermatomyositis. Ann Neurol 2005; 57 (5) 664-678
  • 53 Greenberg SA, Higgs BW, Morehouse C , et al. Relationship between disease activity and type 1 interferon- and other cytokine-inducible gene expression in blood in dermatomyositis and polymyositis. Genes Immun 2012; 13 (3) 207-213
  • 54 Emslie-Smith AM, Engel AG. Necrotizing myopathy with pipestem capillaries, microvascular deposition of the complement membrane attack complex (MAC), and minimal cellular infiltration. Neurology 1991; 41 (6) 936-939
  • 55 Needham M, Fabian V, Knezevic W, Panegyres P, Zilko P, Mastaglia FL. Progressive myopathy with up-regulation of MHC-I associated with statin therapy. Neuromuscul Disord 2007; 17 (2) 194-200
  • 56 Niewold TB, Wu SC, Smith M, Morgan GA, Pachman LM. Familial aggregation of autoimmune disease in juvenile dermatomyositis. Pediatrics 2011; 127 (5) e1239-e1246
  • 57 Bronner IM, Hoogendijk JE, Wintzen AR , et al. Necrotising myopathy, an unusual presentation of a steroid-responsive myopathy. J Neurol 2003; 250 (4) 480-485
  • 58 Mammen AL, Chung T, Christopher-Stine L , et al. Autoantibodies against 3-hydroxy-3-methylglutaryl-coenzyme A reductase in patients with statin-associated autoimmune myopathy. Arthritis Rheum 2011; 63 (3) 713-721
  • 59 Mammen AL, Pak K, Williams EK , et al. Rarity of anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase antibodies in statin users, including those with self-limited musculoskeletal side effects. Arthritis Care Res (Hoboken) 2012; 64 (2) 269-272
  • 60 Amato AA, Barohn RJ. Inclusion body myositis: old and new concepts. J Neurol Neurosurg Psychiatry 2009; 80 (11) 1186-1193
  • 61 Bunch TW, Worthington JW, Combs JJ, Ilstrup DM, Engel AG. Azathioprine with prednisone for polymyositis. A controlled, clinical trial. Ann Intern Med 1980; 92 (3) 365-369
  • 62 Miller FW, Leitman SF, Cronin ME , et al. Controlled trial of plasma exchange and leukapheresis in polymyositis and dermatomyositis. N Engl J Med 1992; 326 (21) 1380-1384
  • 63 Dalakas MC, Illa I, Dambrosia JM , et al. A controlled trial of high-dose intravenous immune globulin infusions as treatment for dermatomyositis. N Engl J Med 1993; 329 (27) 1993-2000
  • 64 Miller J, Walsh Y, Saminaden S, Lecky BRF, Winer JB. Randomised double blind controlled trial of methotrexate and steroids compared with azathioprine and steroids in the treatment of idiopathic inflammatory myopathy. J Neurol Sci 2002; 199 (Suppl. 01) S53
  • 65 Vencovský J, Jarosová K, Machácek S , et al. Cyclosporine A versus methotrexate in the treatment of polymyositis and dermatomyositis. Scand J Rheumatol 2000; 29 (2) 95-102
  • 66 Villalba L, Hicks JE, Adams EM , et al. Treatment of refractory myositis: a randomized crossover study of two new cytotoxic regimens. Arthritis Rheum 1998; 41 (3) 392-399
  • 67 Muscle Study Group. A randomized, pilot trial of etanercept in dermatomyositis. Ann Neurol 2011; 70 (3) 427-436
  • 68 Evans WE, Hon YY, Bomgaars L , et al. Preponderance of thiopurine S-methyltransferase deficiency and heterozygosity among patients intolerant to mercaptopurine or azathioprine. J Clin Oncol 2001; 19 (8) 2293-2301
  • 69 Booth RA, Ansari MT, Loit E , et al. Assessment of thiopurine S-methyltransferase activity in patients prescribed thiopurines: a systematic review. Ann Intern Med 2011; 154 (12) 814-823 , W-295–W-298
  • 70 Cherin P, Piette JC, Wechsler B , et al. Intravenous gamma globulin as first line therapy in polymyositis and dermatomyositis: an open study in 11 adult patients. J Rheumatol 1994; 21 (6) 1092-1097
  • 71 Danieli MG, Malcangi G, Palmieri C , et al. Cyclosporin A and intravenous immunoglobulin treatment in polymyositis/dermatomyositis. Ann Rheum Dis 2002; 61 (1) 37-41
  • 72 Patwa HS, Chaudhry V, Katzberg H, Rae-Grant AD, So YT. Evidence-based guideline: intravenous immunoglobulin in the treatment of neuromuscular disorders: report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology 2012; 78 (13) 1009-1015
  • 73 Rowin J, Amato AA, Deisher N, Cursio J, Meriggioli MN. Mycophenolate mofetil in dermatomyositis: is it safe?. Neurology 2006; 66 (8) 1245-1247
  • 74 Levine TD. Rituximab in the treatment of dermatomyositis: an open-label pilot study. Arthritis Rheum 2005; 52 (2) 601-607
  • 75 Noss EH, Hausner-Sypek DL, Weinblatt ME. Rituximab as therapy for refractory polymyositis and dermatomyositis. J Rheumatol 2006; 33 (5) 1021-1026
  • 76 Oddis CV, Sciurba FC, Elmagd KA, Starzl TE. Tacrolimus in refractory polymyositis with interstitial lung disease. Lancet 1999; 353 (9166) 1762-1763
  • 77 Cagnoli M, Marchesoni A, Tosi S. Combined steroid, methotrexate and chlorambucil therapy for steroid-resistant dermatomyositis. Clin Exp Rheumatol 1991; 9 (6) 658-659
  • 78 Oddis CV, Reed AM, Aggarwal R , et al. Rituximab in the treatment of refractory adult and juvenile dermatomyositis (DM) and adult polymyositis (PM) – The RIM study. Arthritis Rheum 2010; 62: 3844
  • 79 Valiyil R, Casciola-Rosen L, Hong G, Mammen A, Christopher-Stine L. Rituximab therapy for myopathy associated with anti-signal recognition particle antibodies: a case series. Arthritis Care Res (Hoboken) 2010; 62 (9) 1328-1334
  • 80 Park JK, Yoo HG, Ahn DS, Jeon HS, Yoo WH. Successful treatment for conventional treatment-resistant dermatomyositis-associated interstitial lung disease with adalimumab. Rheumatol Int 2011; ; Epub ahead of print
  • 81 Brunasso AM, Scocco GL, Massone C. Dermatomyositis during adalimumab therapy for rheumatoid arthritis. J Rheumatol 2010; 37 (7) 1549-1550
  • 82 Mimori T, Nakashima R, Hosono Y. Interstitial lung disease in myositis: clinical subsets, biomarkers, and treatment. Curr Rheumatol Rep 2012; 14 (3) 264-274
  • 83 Morganroth PA, Kreider ME, Werth VP. Mycophenolate mofetil for interstitial lung disease in dermatomyositis. Arthritis Care Res (Hoboken) 2010; 62 (10) 1496-1501
  • 84 Kotani T, Takeuchi T, Makino S , et al. Combination with corticosteroids and cyclosporin-A improves pulmonary function test results and chest HRCT findings in dermatomyositis patients with acute/subacute interstitial pneumonia. Clin Rheumatol 2011; 30 (8) 1021-1028
  • 85 Varjú C, Pethö E, Kutas R, Czirják L. The effect of physical exercise following acute disease exacerbation in patients with dermato/polymyositis. Clin Rehabil 2003; 17 (1) 83-87
  • 86 Alexanderson H, Stenström CH, Jenner G, Lundberg I. The safety of a resistive home exercise program in patients with recent onset active polymyositis or dermatomyositis. Scand J Rheumatol 2000; 29 (5) 295-301
  • 87 Alexanderson H. Exercise effects in patients with adult idiopathic inflammatory myopathies. Curr Opin Rheumatol 2009; 21 (2) 158-163
  • 88 Chung YL, Alexanderson H, Pipitone N , et al. Creatine supplements in patients with idiopathic inflammatory myopathies who are clinically weak after conventional pharmacologic treatment: Six-month, double-blind, randomized, placebo-controlled trial. Arthritis Rheum 2007; 57 (4) 694-702
  • 89 Marie I, Hachulla E, Hatron PY , et al. Polymyositis and dermatomyositis: short term and longterm outcome, and predictive factors of prognosis. J Rheumatol 2001; 28 (10) 2230-2237
  • 90 Bronner IM, van der Meulen MF, de Visser M , et al. Long-term outcome in polymyositis and dermatomyositis. Ann Rheum Dis 2006; 65 (11) 1456-1461
  • 91 Limaye V, Hakendorf P, Woodman RJ, Blumbergs P, Roberts-Thomson P. Mortality and its predominant causes in a large cohort of patients with biopsy-determined inflammatory myositis. Intern Med J 2012; 42 (2) 191-198
  • 92 Airio A, Kautiainen H, Hakala M. Prognosis and mortality of polymyositis and dermatomyositis patients. Clin Rheumatol 2006; 25 (2) 234-239
  • 93 Marie I, Hatron PY, Levesque H , et al. Influence of age on characteristics of polymyositis and dermatomyositis in adults. Medicine (Baltimore) 1999; 78 (3) 139-147
  • 94 Yamasaki Y, Yamada H, Ohkubo M , et al. Longterm survival and associated risk factors in patients with adult-onset idiopathic inflammatory myopathies and amyopathic dermatomyositis: experience in a single institute in Japan. J Rheumatol 2011; 38 (8) 1636-1643
  • 95 Dankó K, Ponyi A, Constantin T, Borgulya G, Szegedi G. Long-term survival of patients with idiopathic inflammatory myopathies according to clinical features: a longitudinal study of 162 cases. Medicine (Baltimore) 2004; 83 (1) 35-42
  • 96 Benveniste O, Drouot L, Jouen F , et al. Correlation of anti-signal recognition particle autoantibody levels with creatine kinase activity in patients with necrotizing myopathy. Arthritis Rheum 2011; 63 (7) 1961-1971