J Korean Med Sci.  2016 Mar;31(3):389-396. 10.3346/jkms.2016.31.3.389.

Elevated Erythrocyte Sedimentation Rate Is Predictive of Interstitial Lung Disease and Mortality in Dermatomyositis: a Korean Retrospective Cohort Study

• Go DJ
• Lee EY
• Lee EB
• Song YW
• Konig MF
• Park JK

• KMID: 2363499
• DOI: http://doi.org/10.3346/jkms.2016.31.3.389

Abstract

Interstitial lung disease (ILD) is a major cause of death in patients with dermatomyositis (DM). This study was aimed to examine the utility of the erythrocyte sedimentation rate (ESR) as a predictor of ILD and prognostic marker of mortality in patients with DM. One hundred-and-fourteen patients with DM were examined, including 28 with clinically amyopathic DM (CADM). A diagnosis of ILD was made based on high resolution computed tomography (HRCT) scans. The association between elevated ESR and pulmonary impairment and mortality was then examined. ILD was diagnosed in 53 (46.5%) of 114 DM patients. Cancer was diagnosed in 2 (3.8%) of 53 DM patients with ILD and in 24 (92.3%) of those without ILD (P < 0.001). The median ESR (50.0 mm/hour) in patients with ILD was significantly higher than that in patients without ILD (29.0 mm/hour; P < 0.001). ESR was inversely correlated with forced vital capacity (Spearman rho = - 0.303; P = 0.007) and carbon monoxide diffusing capacity (rho = - 0.319; P = 0.006). DM patients with baseline ESR > or = 30 mm/hour had significantly higher mortality than those with ESR < 30 mm/hour (P = 0.002, log-rank test). Patients with a persistently high ESR despite immunosuppressive therapy was associated with higher mortality than those with a normalized ESR (P = 0.039, log-rank test). Elevated ESR is associated with increased mortality in patients with DM due to respiratory failure. Thus, monitoring ESR should be an integral part of the clinical care of DM patients.

Keyword

Erythrocyte Sedimentation Rate; Interstitial Lung Disease; Dermatomyositis

MeSH Terms

Adult
Asian Continental Ancestry Group
Blood Sedimentation
Carbon Monoxide/metabolism
Cohort Studies
Dermatomyositis/blood/*diagnosis/mortality
Disease Progression
Erythrocytes/*cytology
Female
Follow-Up Studies
Humans
Immunosuppressive Agents/therapeutic use
Lung Diseases, Interstitial/*complications/diagnosis
Male
Middle Aged
Predictive Value of Tests
Prognosis
Republic of Korea
Respiratory Function Tests
Retrospective Studies
Risk Factors
Survival Analysis
Carbon Monoxide
Immunosuppressive Agents

Figure

• 

  Fig. 1 Diagnostic flow chart for patients with dermatomyositis (DM). DM, dermatomyositis; ESR, erythrocyte sedimentation rate; ILD, interstitial lung disease; CDM, classic dermatomyositis; CADM, clinically amyopathic dermatomyositis.

• 

  Fig. 2 Association between elevation of the baseline ESR and pulmonary impairment in patients with DM. (A) ESR levels at the time of DM diagnosis were higher in patients with ILD than in those without. (B) Differences in the baseline ESR were more pronounced in the CADM subgroup. The highest ESR level was observed in CADM-ILD patients, whereas there was no significant difference in the baseline ESR between CADM patients with ILD and classic DM patients with ILD (P = 0.235). (C) ESR was inversely correlated with FVC and DLCO. By contrast, there was no correlation between ESR and the extent of muscle injury, as measured by serum creatine kinase (CK) and aldolase levels. Box plots represent the median values (horizontal bars inside the box) and the 25th and 75th percentiles (bottom and top borders of each box, respectively). The whiskers represent the 5th and 95th percentiles, respectively. ρ = Spearman rank correlation coefficient. CDM, classic dermatomyositis; CADM, clinically amyopathic dermatomyositis; FVC, forced vital capacity; DLCO, diffusing capacity of the lung for carbon monoxide.

• 

  Fig. 3 Elevated ESR was predictive of mortality. (A) All-cause mortality for patients with a baseline ESR ≥ 30 mm/hour was higher than that for patients with a baseline ESR < 30 mm/hour. No deaths were observed in DM patients with a normal baseline ESR, even after 8 years of follow-up. (B, C) In both DM patients with ILD and DM patients without ILD, a baseline ESR < 30 mm/hour was associated with an excellent survival rate. (D) The ESR decreased with treatment in a time-dependent manner. (E) Patients with a persistently elevated ESR at 6 months (ESR ≥ 30 mm/hour) showed worse survival than patients whose ESR normalized after treatment.

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