Abstract
The objective of the current report was to determine the relapse rates and characterize the nature of relapses during the disease course of adult patients with idiopathic inflammatory myopathies (IIM). A retrospective cohort study of 53 medical records of patients with polymyositis (PM), dermatomyositis (DM), connective tissue disease (CTD)-associated myositis, and malignancy-associated myositis at an academic rheumatology center was performed. Medical records were reviewed to determine clinical presentation, initial treatment, and clinical follow-up, with an emphasis on relapses. Relapses were defined as a sustained elevation in serum creatine kinase (CK) levels in the absence of an alternative etiology. Patients were followed for an average of 65±43 months. All patients received corticosteroids, and 35 patients received additional immunosuppressive medications as part of their initial treatment. Serum CK levels normalized in 51 patients, and muscle strength normalized in 43 patients. Biochemical relapse was observed in 33 patients (65%). Patients with PM and CTD-associated myositis had a higher relapse rate compared to DM and malignancy-associated myositis patients. Multiple relapses were observed in 17 patients. Relapses tended to occur within the first 2 years after treatment initiation and during the tapering phase of treatment. No risk factors were unequivocally identified, although advanced age and increased duration of symptoms prior to treatment initiation had nonsignificant associations with increased risk of relapse. In conclusion, initial treatment of IIM results in a high rate of normalization of serum CK and muscle weakness. However, physicians should be aware of the high rate of relapse in patients with IIM.
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Joffe MM, Love LA, Leff RL, Fraser DD, Targoff IN, Hicks JE, Plotz PH, Miller FW (1993) Drug therapy of the idiopathic inflammatory myopathies: predictors of response to prednisone, azathioprine, and methotrexate and a comparison of their efficacy. Am J Med 94:379–387
Medsger TA, Robinson H, Masi AT (1971) Factors affecting survivorship in polymyositis. Arthritis Rheum 14:249–258
Maugras YM, Berthelot J, Abbas AA, Mussini J, Nguyen J, Prost AM (1996) Long-term prognosis of 69 patients with dermatomyositis or polymyositis. Clin Exp Rheumatol 14:263–274
Benbassat J, Gefel D, Larholtz K, Sukenik S, Morgenstern V, Zlotnick A (1985) Prognostic factors in polymyositis/dermatomyositis. A computer-assisted analysis of ninety-two cases. Arthritis Rheum 28:249–255
DeVere R, Bradley WG (1975) Polymyositis: its presentation, morbidity and mortality. Brain 98:637–666
Rose AL, Walton AL (1966) Polymyositis: a survey of 89 cases with particular reference to treatment and prognosis. Brain:747–768
Oddis CV, Conte CG, Steen VD, Medsger TA (1990) Incidence of polymyositis–dermatomyositis: a 20-year study of hospital diagnosed cases in allegheny count, pa 1963–1982. J Rheumatol 17:1329–1334
Phillips BA, Zilko P, Garlepp MJ, Mastaglia FL (1998) Frequency of relapses in patients with polymyositis and dermatomyositis. Muscle Nerve 21:1668–1672
Hoffman GS, Franck WA, Raddatz DA, Stallones L (1983) Presentation, treatment, and prognosis of idiopathic inflammatory muscle disease in a rural hospital. Am J Med 75:433–438
Henriksson KG, Sandstedt P (1982) Polymyositis—treatment and prognosis. A study of 107 patients. Acta Neurol Scand 65:280–300
Mosca M, Neri R, Pasero G, Bombardieri S (2000) Treatment of idiopathic inflammatory myopathies: a retrospective analysis of 63 caucasian patients longitudinally followed at a single clinical center. Clin Exp Rheumatol 18:451–456
Bohan A, Peter JB (1975) Polymyositis and dermatomyositis (first of two parts). N Engl J Med 292:344–347
Dalakas MC, Hohlfeld R (2003) Polymyositis and dermatomyositis. Lancet 362:971–982
Mastaglia FL, Phillips BA (2002) Idiopathic inflammatory myopathies: epidemiology, classification, and diagnostic criteria. Rheum Dis Clin North Am 28:723–741
Dalakas MC (2002) Muscle biopsy findings in inflammatory myopathies. Rheum Dis Clin North Am 28:779–798, vi
Oddis CV, Medsger TA Jr (1988) Relationship between serum creatine kinase level and corticosteroid therapy in polymyositis–dermatomyositis. J Rheumatol 15:807–811
Sultan SM, Ioannou Y, Moss K, Isenberg DA (2002) Outcome in patients with idiopathic inflammatory myositis: morbidity and mortality. Rheumatology (Oxford) 41:22–26
Lee W, Zimmermann B 3rd, Lally EV (1997) Relapse of polymyositis after prolonged remission. J Rheumatol 24:1641–1644
Koh ET, Seow A, Ong B, Ratnagopal P, Tjia H, Chng HH (1993) Adult onset polymyositis/dermatomyositis: clinical and laboratory features and treatment response in 75 patients. Ann Rheum Dis 52:857–861
Henriksson KG, Lindvall B (1990) Polymyositis and dermatomyositis 1990—diagnosis, treatment and prognosis. Prog Neurobiol 35:181–193
Lundberg I, Hedfors E (1991) Clinical course of patients with anti-rnp antibodies. A prospective study of 32 patients. J Rheumatol 18:1511–1519
Miro O, Laguno M, Grau JM (1999) Relapses in idiopathic inflammatory myopathies. Muscle Nerve 22: 1159–1160; discussion 1160–1151
Arnett FC, Whelton JC, Zizic TM, Stevens MB (1973) Methotrexate therapy in polymyositis. Ann Rheum Dis 32:536–546
Villalba L, Hicks JE, Adams EM, Sherman JB, Gourley MF, Leff RL, Thornton BC, Burgess SH, Plotz PH, Miller FW (1998) Treatment of refractory myositis: a randomized crossover study of two new cytotoxic regimens. Arthritis Rheum 41:392–399
Bunch TW, Worthington JW, Combs JJ, Ilstrup DM, Engel AG (1980) Azathioprine with prednisone for polymyositis. Ann Intern Med 92:365–369
Acknowledgements
We would like to thank Dr. Jeffrey N. Katz for his critical review of this manuscript. Furthermore, we would like to thank the Robert Brigham Arthritis and Musculoskeletal Clinical Research Center for its support during this study.
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Agarwal, S.K., Monach, P.A., Docken, W.P. et al. Characterization of relapses in adult idiopathic inflammatory myopathies. Clin Rheumatol 25, 476–481 (2006). https://doi.org/10.1007/s10067-005-0075-3
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DOI: https://doi.org/10.1007/s10067-005-0075-3