Prognostic Factors Affecting Survival Rate in Inflammatory Myositis

Hye-In Kim; Hee Kyung Baek; Jin Kyu Jung; Yong Min Jo; Sang Yeob Lee; Sung Won Lee; Won Tae Chung

doi:10.4078/jkra.2009.16.2.108

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Kim, Baek, Jung, Jo, Lee, Lee, and Chung: Prognostic Factors Affecting Survival Rate in Inflammatory Myositis

Original Article

J Korean Rheum Assoc 2009;16(2):108-114.

Published online: 20 June 2009

DOI: https://doi.org/10.4078/jkra.2009.16.2.108

Prognostic Factors Affecting Survival Rate in Inflammatory Myositis

Hye-In Kim, Hee Kyung Baek, Jin Kyu Jung, Yong Min Jo, Sang Yeob Lee, Sung Won Lee, Won Tae Chung

Department of Internal Medicine, Dong-A University College of Medicine, Busan, Korea

Received 27 February 2009 Revised 8 May 2009 Accepted 8 May 2009

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Objective

To assess the 10-year cumulative survival outcome of polymyositis (PM) and dermatomyositis (DM) as well as the factors associated with the the outcome.

Methods

Eighty five patients with PM and twenty one patients with DM were diagnosed at our university medical center between 1997 and 2007. Thirty six patients with PM and 13 patients with DM were followed up until death or until the end of January, 2008. Gender, age, AST, ALT, CPK, LDH, ESR, CRP, aldolase, drugs of therapy, combined ILD, and cancer, and duration of remission after therapy were assessed as prognostic factors of death by the Kaplan-Meier curve and Cox regression model.

Results

The respective 10-year survival rate for PM and DM was 80.8% (95% confidence interval (CI): 73.3∼87.2) and 55.9% (95% CI: 40.7∼71.1), respectively. The median survival for PM and DM was 11.3 years (95% CI: 9.8∼12.9) and 7.0 years (95% CI: 3.6∼10.5), respectively. Compared to DM patients, the subjects with PM had a 167.26 fold (95% CI: 7.59∼ 3683.19) combined ILD adjusted risk of mortality (p＜0.05) and no other individual factor reached significance as a predictor of death. However, cancer had a hazard ratio (HR) of 17.00 (95% CI: 1.06∼281.79) and 2.45 (95% CI: 0.78∼12.45) for death in the PM and DM group, respectively.

Conclusion

According to an analysis of the survival and its prognostic factors in patients with PM and DM, ILD is a risk factor for mortality in PM and cancer was risk factor for mortality in both PM and DM.

Keywords: Polymyositis, Dermatomyositis, Prognostic factor

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Fig. 1.

Cumulative survival curve in polymyositis.

Fig. 2.

Cumulative survival curve in dermatomyositis.

Table 1.

Characteristics and risk factors of patients with inflammatory muscle disease

	PM (n=36)	DM (n=13)
Age (years)	47.22±14.37	51.31±14.32
Gender (M/F)	18/18	7/6
Treatment drug (n) Prs/Prs＋immunosuppressive drug	23 (64%)/13 (36%)	9 (69%)/4 (31%)
ANA Positive/Negative (n)	14 (39%)/22 (61%)	4 (31%)/9 (69%)
AST (IU/L)	250.08±212.93	151.46±78.02
ALT (IU/L)	209.19±134.23	107.61±51.92
Aldolase (U/L)	15.15±13.08	7.93±5.13
LDH (U/L)	2,142.50±1,895.58	1,050.15±5,01.59
CPK (U/L)	6,155.69±5,926.41	1,549.69±2,029.7
ESR (mm/hr)	67.97±37.35	75.84±39.15
CRP (mg/dL)	4.43±6.03	3.62±4.04
Treatment duration for remission (month)	2.51±0.61	1.23±0.43
ILD (with/without) (n)	13/23	5/8
Cancer (n)	2 (5.6%)	3 (23%)

Values are mean±SD. Prs: prednisolon, eANA: anti-nuclear antibody, PM: polymyositis, DM: dermatomyositis, PTS: prednisolon, ANA: antinuclear antibody, AST: aspartate aminotransferase, ALT: alanine aminotransferase, LDH: lactate dehydrogenase, CPK: creatin phosphkinase, ESR: erythrocyte sedimentation rate, CRP: C-reactive protein, ILD: interstitial lung disease

Table 2.

Statistically significance of prognostic factors with mortality in inflammatory muscle disease

	p-value
	PM&DM (n=49)	PM (n=36)	DM (n=13)
Age	0.127	0.29	0.28
Gender	0.49	0.86	0.28
Tx drug	0.98	0.74	0.91
ANA	0.52	0.65	0.61
Anti-Jo1	0.92	0.38	0.43
LDH	0.20	0.35	0.42
AST	0.46	0.47	0.36
ALT	0.17	0.33	0.53
Aldolase	0.53	0.41	0.24
CPK	0.14	0.28	0.28
ESR	0.62	0.68	0.59
CRP	0.29	0.38	0.59
Tx duration for remission	0.36	0.89	0.77
ILD	0.035	0.04	0.42
Cancer	0.00	0.01	0.001

PM: polymyositis, DM: dermatomyositis, Tx: treatment, ANA: antinuclear antibody, LDH: lactate dehydrogenase, AST: aspartate aminotransferase, ALT: alanine aminotransferase, CPK: creatin phosphate kinase, ESR: erythrocyte sedimentation rate, CRP: C-reactive protein, ILD: interstitial lung disease

Table 3.

Anti-Jo1 antibody is related to interstitial lung disease of polymyositis but not in dermatomyositis

	p-value
	PM with ILD (n=9)	DM with ILD (n=9)
Anti-Jo1 Ab	0.003 (n=6)	0.462 (n=1)

ILD: interstitial lung disease, PM: polymyositis, DM: dermatomyositis, anti Jo-1 Ab: anti Jo-1 antibody

Table 4.

Hazard ratio in inflammatory muscle disease. Interstitial lung disease, age and treatment drug influence mortality in polymyositis. In dermatomyositis, there are not significant factors in mortality

Risk factor	PM		DM
Risk factor	Hazard ratio (95% C.I.)	p-value	Hazard ratio (95% C.I.)	p-value
Age	0.98 (0.08∼1.17)	0.66	NR	NR
Tx drug	0.01 (0.00∼0.25)	0.56	NR	NR
ILD	167.26 (7.59∼3,683.19)	0.001	NR	NR
Cancer	17.00 (1.06∼271.79)	0.04	NR	NR

ILD: interstitial lung disease, C.I.: confidence interval, Tx: treatment

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