Abstract
The objective of this study was to assess the long-term outcome of polymyositis (PM) and dermatomyositis (DM) and the factors predictive of this outcome in a nationwide series in Finland. One hundred and seventy-six patients with PM and 72 patients with DM diagnosed in Finland in 1969–1985 were selected from the national hospital discharge register according to the diagnostic criteria of Bohan and Peter and followed up until death or till the end of August 1995. Gender, age, delay of therapy, serum creatine kinase, erythrocyte sedimentation rate, initial dose of corticosteroids and duration of cytostatic therapy were assessed as factors prognostic of death. The 5-year survival rate for PM was 75% [95% confidence interval (CI): 68–81%] and that for DM 63% (50–73%), and the respective 10-year survival rates were 55% (47–62%) and 53% (41–64%). The median survival for polymyositis was 11.0 years (95% CI: 9.5–13.3) and that for DM 12.3 years (5.5–20.7). The standardized mortality ratio for the combined group of PM and DM showed approximately threefold mortality compared to the general population. Compared to PM patients, the subjects with DM had a 1.47-fold (95% CI: 0.99–2.12) age- and sex-adjusted risk of mortality. Except for age in both groups and the delay in diagnosis in the PM group, no other individual factor reached significance as a predictor of death. However, cancer had a hazard ratio (HR) of 2.16 for death (95% CI: 0.95–4.50) in the DM group and 1.99 (95% CI: 1.01–3.94) in the PM group. A comparison of the causes of death in the PM and DM groups showed that the patients with DM had a greater risk of dying from cancer (age-adjusted HR 5.11, 95% CI: 2.31–11.3). According to this nationwide analysis of survival and its prognostic factors in patients with PM and DM, the latter group had an increased age-adjusted risk for mortality compared to the former. The difference seems to be mostly explained by the fact that the patients with DM had a higher risk of dying from cancer.
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References
Medsger TA Jr, Robinson H, Masi AT (1971) Factors affecting survivorship in polymyositis: a life-table study of 124 patients. Arthritis Rheum 14:249–258
Carpenter JR., Bunch TW, Engel AG, O’Brien PC (1977) Survival in polymyositis: corticosteroids and risk factors. J Rheumatol 4:207–214
Henriksson KG, Sandstedt P (1982) Polymyositis—treatment and prognosis: a study of 107 patients. Acta Neurol Scand 65:280–300
Hochberg MC, Lopez-Acuna D, Gittelsohn AM (1983) Mortality from polymyositis and dermatomyositis in the United States, 1968–1978. Arthritis Rheum 26:1465–1471
Benbassat J, Gefel D, Larholt K, Sukenik S, Morgenstern V, Zlotnick A (1985) Prognostic factors in polymyositis/derma-tomyositis. A computer-assisted analysis of ninety-two cases. Arthritis Rheum 28:249–255
Maugars YM, Berthelot JM, Abbas AA, Mussini JM, Nguyen JM, Prost AM (1996): Long-term prognosis of 69 patients with dermatomyositis or polymyositis. Clin Exp Rheumatol 14:263–274
Marie I, Hachulla E, Hatron P-Y, Hellot M-F, Levesque H, Devulder B, Courtois H (2001) Polymyositis and dermatomyositis: short term and long term outcome, and predictive factors of prognosis. J Rheumatol 28:2230–2237
Bohan A, Peter JB, Bowman RL, Pearson CM (1977) Computer-assisted analysis of 153 patients with polymyositis and dermatomyositis. Medicine 56:255–286
Sigurgeirsson B, Lindelöf B, Edhag O, Allander EA (1992) Risk of cancer in patients with dermatomyositis or polymyositis. A population-based study. N Engl J Med 6:363–367
Bohan A, Peter JB (1975) Polymyositis and dermatomyositis (first of two parts). N Engl J Med 292:344–347
Bohan A, Peter JB (1975) Polymyositis and dermatomyositis (second of two parts). N Engl J Med 292:403–407
Marie I, Hatron P-Y, Levesque H, Hachulla E, Hellot M-F, Michon-Pasturel U, Courtois H, Devulder B (1999) Influence of age on characteristics of polymyositis and dermatomyositis in adults. Medicine (Baltimore) 78:139–147
Fudman EJ, Schnitzer TJ (1986) Dermatomyositis without creatine kinase elevation. A poor prognostic sign. Am J Med 80:329–332
Lakhanpal S, Bunch TW, Ilstrup DM, Melton LJ III (1986) Polymyositis-dermatomyositis and malignant lesions: does an association exist? Mayo Clin Proc 61:645–653
Nicholls D (1987) Dermatomyositis without creatine kinase elevation. Am J Med 83:182–183
Basset-Seguin N, Roujeau JC, Gherardi R, Guillaume JC, Revuz J, Touraine R (1990) Prognostic factors and predictive signs of malignancy in adult dermatomyositis. Arch Dermatol 126:633–637
O’Leary PA, Waismann M (1940) Dermatomyositis: a study of 40 cases. Arch Dermatol Syphilol 41:1001–1019
Metzger AL, Bohan A, Goldberg LS, Bluestone R, Pearson CM (1974) Polymyositis and dermatomyositis: combined methotrexate and corticosteroid therapy. Ann Intern Med 81:182–189
Bunch TW (1981) Prednisone and azathioprine for polymyositis: Long-term followup. Arthritis Rheum 24:45–48
Bong JJ, Lansdown M (2002) Figure. BMJ 324:1344
Airio A, Pukkala E, Isomäki H (1995) Elevated cancer incidence in patients with dermatomyositis: a population based study. J Rheumatol 22:1300–1303
Chow WH, Gridley G, Mellemkjaer L, McLauglin JK, Olsen JH, Fraumeni JF Jr (1995) Cancer risk following polymyositis and dermatomyositis: a nationwide cohort study in Denmark. Cancer Causes Control 6:9–13
Hill CL, Zhang Y, Sigurgeirsson B, Pukkala E, Mellemkjaer L, Airio A, Evans SR, Felson DT (2001) Frequency of specific cancer types in dermatomyositis and polymyositis: a population-based study. Lancet 357:96–100
Marie I, Hatron P-Y, Hachulla E, Wallaert B, Michon-Pasturel U, Devulder B (1998) Pulmonary involvement in polymyositis and in dermatomyositis. J Rheumatol 25:1336–1343
Tymms KE, Webb J (1985) Dermatopolymyositis and other connective tissue diseases: areview of 105 cases. J Rheumatol 12:1140–1148
Hernandez-Cruz B, Sifuentes-Osornio J, Ponce-de-Leon Ro-sales S, Ponce-de-Leon Garduno A, Diaz-Jouanen E (1999) Mycobacterium tuberculosis infection in patients with systemic rheumatic diseases. A case series. Clin Exp Rheumatol 17:289–296
Haq I, Isenberg DA (2002) Myositis and swollen knees: disease or treatment complication? Ann Rheum Dis 61:544–546
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Published online: 14 February 2006
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Airio, A., Kautiainen, H. & Hakala, M. Prognosis and mortality of polymyositis and dermatomyositis patients. Clin Rheumatol 25, 234–239 (2006). https://doi.org/10.1007/s10067-005-1164-z
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DOI: https://doi.org/10.1007/s10067-005-1164-z