Abstract
Objective
To investigate the mortality and the causes of death in Chinese patients with polymyositis (PM) and dermatomyositis (DM).
Methods
The clinical data of all consecutive adult PM/DM patients in Rheumatology and clinical immunology department of Peking University First Hospital from January 2007 to Apr2016 were collected. The primary causes of death were identified, the standardized mortality ratio (SMR) and years of life lost (YLL) were calculated based on the National Bureau of Statistics of China for the general population, the survival in the first decade was performed using Kaplan-Meier analysis, and the predictors of mortality were evaluated by multivariable cox regression.
Results
A total of 85 PM and226 DM cases were included and 68 patients died. Infection (52.3%) was the leading cause of death. The overall age and sex adjusted SMR was 6.0(95%CI 3.5–8.5) for PM, and 9.0(95%CI 6.8–11.2) for DM. The YLL of women and men were 12.2 and 18.3 years respectively for PM, and 37.5 and 28.4 years respectively for DM. The 10-year survival of patients with ILD, malignancy or infection was significantly worse than those without, respectively. The independent predictors of mortality for PM/DM patients were age at disease onset, malignancy and infection.
Conclusions
Mortality of PM/DM patients in China is substantial, especially in females, and those with ILD, malignancy or infection. Infection was the leading cause of death. Patients with older age at onset, infection, ILD, and malignancy need to be paid more attention.
Key Points • This is the first comprehensive report about the mortality situation with a large population in PM/DM patients in China including SMR, YLL, and cause of death, Kaplan-Meier survival analysis and Cox regression analysis for mortality risk factors. • The specific SMRs for PM/DM patients with malignancy and interstitial lung disease were also reported in this study. To our knowledge, only two studies worldwide reported SMRs in PM/DM patients and no figure about YLL was reported so far. • Overall, the mortality figures in this study were higher than those from the western countries, and the leading cause of death was different from the western countries. |
Similar content being viewed by others
Data availability
The datasets analyzed during the current study are available from the corresponding author upon reasonable request.
References
Dalakas MC (2015) Inflammatory muscle diseases. N Engl J Med 372:1734–1747
Lilleker JB, Vencovsky J, Wang G, Wedderburn LR, Diederichsen LP, Schmidt J, Oakley P, Benveniste O, Danieli MG, Danko K, Thuy NTP, Vazquez-del Mercado M, Andersson H, de Paepe B, deBleecker J, Maurer B, McCann L, Pipitone N, McHugh N, Betteridge ZE, New P, Cooper RG, Ollier WE, Lamb JA, Krogh NS, Lundberg IE, Chinoy H, all EuroMyositis contributors (2018) The EuroMyositis registry: an international collaborative tool to facilitate myositis research. Ann Rheum Dis 77(1):30–39
Dobloug GC, Svensson J, Lundberg IE, Holmqvist M (2018) Mortality in idiopathic inflammatory myopathy: results from a Swedish nationwide population-based cohort study. Ann Rheum Dis 77(1):40–47
Marie I (2012) Morbidity and mortality in adult polymyositis and dermatomyositis. Curr Rheumatol Rep 14:275–285
Nuno-Nuno L, Joven BE, Carreira PE, Maldonado-Romero V, Larena-Grijalba C, Cubas IL et al (2017) Mortality and prognostic factors in idiopathic inflammatory myositis: a retrospective analysis of a large multicenter cohort of Spain. Rheumatol Int 37(11):1853–1861
Mustafa KN, Dahbour SS (2010) Clinical characteristics and outcomes ofpatients with idiopathic inflammatory myopathies from Jordan 1996–2009. Clin Rheumatol 29:1381–1385
Yu KH, Wu YJ, Kuo CF, See LC, Shen YM, Chang HC, Luo SF, Ho HH, Chen IJ (2011) Survival analysis of patients withdermatomyositis and polymyositis: analysis of 192 Chinese cases. Clin Rheumatol 30:1595–1601
Schiopu E, Phillips K, Macdonald PM, Crofford LJ, Somers EC (2012) Predictors ofsurvival in a cohort of patients with polymyositis and dermatomyositis: effect of corticosteroids, methotrexate, and azathioprine. Arthritis Res Ther 14:R22
Yamasaki Y, Yamada H, Ohkubo M, Yamasaki M, Azuma K, Ogawa H, Mizushima M, Ozaki S (2011) Longterm survivaland associated risk factors in patients with adult-onset idiopathic inflammatory myopathies and amyopathic dermatomyositis:experience in a single institute in Japan. J Rheumatol 38:1636–1643
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
Sontheimer RD (2002) Would a new name hasten the acceptanceof amyopathic dermatomyositis (dermatomyositissine´ myositis) as a distinctive subset within the idiopathicinflammatorydermatomyopathies spectrum of clinical illness? J Am Acad Dermatol 46:626–636
Lundberg IE, Miller FW, Tjärnlund A et al (2016) Diagnosis and classification of idiopathicinflammatory myopathies. J Intern Med 280:39–51
American Thoracic Society, European Respiratory Society (2002) American Thoracic Society/European RespiratorySociety international multidisciplinary consensus classificationof the idiopathic interstitial pneumonias. Am J Respir Crit Care Med 165:277–304
Sato S, Masui K, Nishina N, Kawaguchi Y, Kawakami A, Tamura M et al (2018) Initial predictors of poor survival in myositis-associated interstitial lung disease: a multicentre cohort of 497 patients. Rheumatology 57:1212–1221
Schwartz T, Diederichsen LP, Lundberg IE et al (2016) Cardiac involvement in adult and juvenile idiopathic inflammatory myopathies. RMD Open 2:e000291
Gosselink R, Kovacs L, Decramer M (1999) Respiratory muscle involvement in multiple sclerosis. Eur Respir J 13:449–454
Lee PP, Lee TL, Ho MH, Wong WH, Lau YL (2007) Recurrent major infections injuvenile-onset systemic lupus erythematosus–a close linkwith long-term disease damage. Rheumatology 46:1290–1296
Andersen PK, Borgan O, Gill RD, Keiding N (1993) Statistical models based on counting processes. Springer-Verlag, New York, p 322
Mok CC, Kwok CL, Ho LY, Chan PT, Yip SF (2011) Life expectancy, standardized mortality ratios, and causes of death in six rheumatic diseases in Hong Kong, China. Arthritis Rheum 63:1182–1189
Hao Y, Hudson M, Baron M, Carreira P, Stevens W, Rabusa C et al (2017) Early mortality in a multinational systemic sclerosis inception cohort. Arthritis Rheum 69:1067–1077
Chiang CL (1968) The life table and its construction. In: Introduction to stochastic processes in Bioststatistics. John Wiley & Sons, New York, pp 189–214
Newell C (1994) Methods and Models in Demography. John Wiley & Sons, Chichester(UK), pp 63–81
Kuo CF, See LC, Yu KH, Chou IJ, Chang HC, Chiou MJ, Luo SF (2011) Incidence, cancer risk and mortality of dermatomyositis and polymyositis in Taiwan: a nationwide population study. Br J Dermatol 165:1273–1279
Chen IJ, JanWu YJ, Lin CW, Fan KW, Luo SF, Ho HH et al (2009) Interstitial lung disease in polymyositis and dermatomyositis. Clin Rheumatol 28:639–646
Obert J, Freynet O, Nunes H, Brillet PY, Miyara M, Dhote R, Valeyre D, Naccache JM (2016) Outcome and prognostic factors in a French cohort of patients with myositis-associated interstitial lung disease. Rheumatol Int 36(12):1727–1735
Chen W, Zheng R, Baade PD, Zhang S, Zeng H, Bray F, Jemal A, Yu XQ, He J (2016) Cancer statistics in China, 2015. CA Cancer J Clin 66:115–132
Wu C, Wang Q, He L, Yang E, Zeng X (2018) Hospitalization mortality and associated risk factors in patients with polymyositis and dermatomyositis: a retrospective case-control study. PLoS One 13(2):e0192491
Marie I, Menard JF, Hachulla E, Cherin P, Benveniste O, Tiev K et al (2011) Infectious complications in polymyositis and dermatomyositis: a series of 279 patients. Semin Arthritis Rheum 41:48–60
Chen IJ, Tsai WP, Wu YJ, Luo SF, Ho HH, Liou LB et al (2010) Infections in polymyositis and dermatomyositis: analysis of 192 cases. Rheumatology 49:2429–2437
Schiopu E, Phillips K, MacDonald PM, Crofford LJ, Somers EC (2012) Predictors of survival in a cohort ofpatients with polymyositis and dermatomyositis: effect of corticosteroids, methotrexate and azathioprine. Arthritis Res Ther 14:R22
Marie I, Hachulla E, Chérin P, Hellot MF, Herson S, Levesque H et al (2005) Opportunistic infectionsin polymyositis and dermatomyositis. Arthritis Rheum 53:155–165
Gordon PA, Winer JB, Hoogendijk JE et al (2012) Immunosuppressant andimmunomodulatory treatment for dermatomyositis and polymyositis. Cochrane Database Syst Rev 8:CD003643
Vermaak E, Tansley SL, McHugh NJ (2015) The evidence for immunotherapy indermatomyositis and polymyositis: a systematic review. Clin Rheumatol 34:2089–2095
Redondo-Benito C, Villar-Gomez, Trallero-Araguas, Fernández-Codina, Pinal-Fernandez, Rodrigo-Pendás J, Selva-O'Callaghan (2018) Opportunistic infections in patients with idiopathic inflammatory myopathies. Int J Rheum Dis 21(2):487–496
Brandt D, Gershwin ME (2006) Common variable immunedeficiency and autoimmunity. Autoimmun Rev 5:465–470
Tha-In T, Bayry J, Metselaar HJ et al (2008) Modulation of thecellular immune system by intravenousimmunoglobulin. Trends Immunol 29:608–615
Kaveri SV, Lacroix-Desmazes S, Bayry J (2008) The antiinflammatory IgG. N Engl J Med 359:307–309
Jordan SC, Toyoda M, Vo AA (2009) Intravenous immunoglobulin a natural regulator of immunity and inflammation. Transplantation 88:1–6
Lundberg IE, Tjarnlund A, Bottai M, Werth VP, Pilkington C, de Visser M et al (2017) 2017 European league against rheumatism/American College of Rheumatology Classification Criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups. Arthritis Rheum 69(12):2271–2282
Acknowledgments
We thank our patients with PM/DM participating in this study.
Author information
Authors and Affiliations
Contributions
ZLZ:study conception and design, analysis and interpretation of data, article revision and approval of the final version. YJH: study conception and design, analysis and interpretation of data, drafting of the article and approval of the final version. XLY: acquisition of data, analysis and interpretation of data, drafting of the article, article revision and approval of the final version. Others: acquisition of data, article revision and approval of the final version.
Corresponding author
Ethics declarations
Ethics approval and consent to participate
The ethics approval was obtained from the human research ethics committees of Peking University First Hospital.
Disclosures
None.
Additional information
Publisher’s note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Electronic supplementary material
Rights and permissions
About this article
Cite this article
Yang, X., Hao, Y., Zhang, X. et al. Mortality of Chinese patients with polymyositis and dermatomyositis. Clin Rheumatol 39, 1569–1579 (2020). https://doi.org/10.1007/s10067-019-04910-w
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10067-019-04910-w