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Prognostic factors for myositis-associated interstitial lung disease.

Fujisawa T, Hozumi H, Kono M, Enomoto N, Hashimoto D, Nakamura Y, Inui N, Yokomura K, Koshimizu N, Toyoshima M, Shirai T, Yasuda K, Hayakawa H, Suda T - PLoS ONE (2014)

Bottom Line: PM) were significantly associated with poor outcome in univariate Cox proportional hazards models.PM) as significant predictors of overall mortality.Acute/subacute form, older age, lower level of FVC and diagnosis of CADM predict poor outcome in PM/DM/CADM-ILD.

View Article: PubMed Central - PubMed

Affiliation: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.

ABSTRACT

Background: Interstitial lung disease (ILD) is a common manifestation of polymyositis (PM), dermatomyositis (DM), and clinically amyopathic dermatomyositis (CADM); however, little is known about the factors influencing the prognosis for PM/DM/CADM-associated ILD. (PM/DM/CADM-ILD). The aim of the present study is to assess prognostic factors for PM/DM/CADM-ILD.

Methods: The clinical features and survival of 114 consecutive patients diagnosed with PM/DM/CADM-ILD (39 men and 75 women; median age, 56 years) were analyzed retrospectively.

Results: The study group included 30 PM-associated ILD, 41 DM-associated ILD, and 43 CADM-associated ILD cases. The clinical presentation of ILD was acute/subacute form in 59 patients (51.8%) and chronic form in 55 patients (48.2%). The major pulmonary symptoms were dyspnea, cough, and fever. High-resolution computed tomography frequently revealed ground-glass opacities, traction bronchiectasis, and consolidation. Most of the patients were treated with corticosteroids or corticosteroids in combination with immunosuppressive agents. The all-cause mortality was 27.2%. Acute/subacute form, % forced vital capacity (FVC), age, % of neutrophils in bronchoalveolar lavage (BAL) fluid, and a diagnosis of CADM (vs. PM) were significantly associated with poor outcome in univariate Cox proportional hazards models. Multivariate Cox proportional hazards analysis validated acute/subacute ILD, %FVC, age, and diagnosis of CADM (vs. PM) as significant predictors of overall mortality. Patients with acute/subacute ILD had a much lower survival rate than those with the chronic form (p<0.001). Patients with CADM-ILD had a lower survival rate than those with PM-ILD (pā€Š=ā€Š0.034).

Conclusions: Acute/subacute form, older age, lower level of FVC and diagnosis of CADM predict poor outcome in PM/DM/CADM-ILD.

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Related in: MedlinePlus

Survival curves of patients with PM-ILD, DM-ILD and CADM-ILD.Patients with CADM-ILD have a significantly lower survival rate than those of PM-ILD (log-rank, pā€Š=ā€Š0.0034).
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pone-0098824-g002: Survival curves of patients with PM-ILD, DM-ILD and CADM-ILD.Patients with CADM-ILD have a significantly lower survival rate than those of PM-ILD (log-rank, pā€Š=ā€Š0.0034).

Mentions: The results of the univariate Cox proportional hazards models are shown in Table 7. Acute/subacute form, %FVC, age, % of neutrophils in BAL fluid, and a diagnosis of CADM (vs. PM) were significantly associated with a worse prognosis. None of the HRCT findings was associated with poor outcomes. In the 38 cases with surgical lung biopsy, univariate Cox proportional hazards models analysis revealed that pathological patterns (UIP or NSIP) were not associated with a worse prognosis (UIP vs NISP, hazard ratio 1.944, pā€Š=ā€Š0.588). Multivariate Cox proportional hazards model analysis revealed that acute/subacute form, age, %FVC, and a diagnosis of CADM (vs. PM) were independent predictors of overall mortality (Table 8). Comparison of the survival rate between the acute/subacute and chronic forms is shown in Figure 1. Patients with the acute/subacute form of ILD had a significantly lower survival rate than did those with the chronic form (five-year survival, 52% vs. 87%, respectively; p<0.0001). The survival curves for patients with PM-ILD, DM-ILD, and CADM-ILD are shown in Figure 2. The survival rate was significantly lower in the patients with CADM-ILD than in those with PM-ILD (pā€Š=ā€Š0.034). The survival analysis suggested that the outcome in patients with DM-ILD was worse than that in patients with PM-ILD but better than that in patients with CADM-ILD (five-year survival: PM-ILD, 82%; DM-ILD, 71%; CADM-ILD, 59%), although no significant differences in survival rate were found between PM-ILD and DM-ILD or between DM-ILD and CADM-ILD.


Prognostic factors for myositis-associated interstitial lung disease.

Fujisawa T, Hozumi H, Kono M, Enomoto N, Hashimoto D, Nakamura Y, Inui N, Yokomura K, Koshimizu N, Toyoshima M, Shirai T, Yasuda K, Hayakawa H, Suda T - PLoS ONE (2014)

Survival curves of patients with PM-ILD, DM-ILD and CADM-ILD.Patients with CADM-ILD have a significantly lower survival rate than those of PM-ILD (log-rank, pā€Š=ā€Š0.0034).
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4048238&req=5

pone-0098824-g002: Survival curves of patients with PM-ILD, DM-ILD and CADM-ILD.Patients with CADM-ILD have a significantly lower survival rate than those of PM-ILD (log-rank, pā€Š=ā€Š0.0034).
Mentions: The results of the univariate Cox proportional hazards models are shown in Table 7. Acute/subacute form, %FVC, age, % of neutrophils in BAL fluid, and a diagnosis of CADM (vs. PM) were significantly associated with a worse prognosis. None of the HRCT findings was associated with poor outcomes. In the 38 cases with surgical lung biopsy, univariate Cox proportional hazards models analysis revealed that pathological patterns (UIP or NSIP) were not associated with a worse prognosis (UIP vs NISP, hazard ratio 1.944, pā€Š=ā€Š0.588). Multivariate Cox proportional hazards model analysis revealed that acute/subacute form, age, %FVC, and a diagnosis of CADM (vs. PM) were independent predictors of overall mortality (Table 8). Comparison of the survival rate between the acute/subacute and chronic forms is shown in Figure 1. Patients with the acute/subacute form of ILD had a significantly lower survival rate than did those with the chronic form (five-year survival, 52% vs. 87%, respectively; p<0.0001). The survival curves for patients with PM-ILD, DM-ILD, and CADM-ILD are shown in Figure 2. The survival rate was significantly lower in the patients with CADM-ILD than in those with PM-ILD (pā€Š=ā€Š0.034). The survival analysis suggested that the outcome in patients with DM-ILD was worse than that in patients with PM-ILD but better than that in patients with CADM-ILD (five-year survival: PM-ILD, 82%; DM-ILD, 71%; CADM-ILD, 59%), although no significant differences in survival rate were found between PM-ILD and DM-ILD or between DM-ILD and CADM-ILD.

Bottom Line: PM) were significantly associated with poor outcome in univariate Cox proportional hazards models.PM) as significant predictors of overall mortality.Acute/subacute form, older age, lower level of FVC and diagnosis of CADM predict poor outcome in PM/DM/CADM-ILD.

View Article: PubMed Central - PubMed

Affiliation: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.

ABSTRACT

Background: Interstitial lung disease (ILD) is a common manifestation of polymyositis (PM), dermatomyositis (DM), and clinically amyopathic dermatomyositis (CADM); however, little is known about the factors influencing the prognosis for PM/DM/CADM-associated ILD. (PM/DM/CADM-ILD). The aim of the present study is to assess prognostic factors for PM/DM/CADM-ILD.

Methods: The clinical features and survival of 114 consecutive patients diagnosed with PM/DM/CADM-ILD (39 men and 75 women; median age, 56 years) were analyzed retrospectively.

Results: The study group included 30 PM-associated ILD, 41 DM-associated ILD, and 43 CADM-associated ILD cases. The clinical presentation of ILD was acute/subacute form in 59 patients (51.8%) and chronic form in 55 patients (48.2%). The major pulmonary symptoms were dyspnea, cough, and fever. High-resolution computed tomography frequently revealed ground-glass opacities, traction bronchiectasis, and consolidation. Most of the patients were treated with corticosteroids or corticosteroids in combination with immunosuppressive agents. The all-cause mortality was 27.2%. Acute/subacute form, % forced vital capacity (FVC), age, % of neutrophils in bronchoalveolar lavage (BAL) fluid, and a diagnosis of CADM (vs. PM) were significantly associated with poor outcome in univariate Cox proportional hazards models. Multivariate Cox proportional hazards analysis validated acute/subacute ILD, %FVC, age, and diagnosis of CADM (vs. PM) as significant predictors of overall mortality. Patients with acute/subacute ILD had a much lower survival rate than those with the chronic form (p<0.001). Patients with CADM-ILD had a lower survival rate than those with PM-ILD (pā€Š=ā€Š0.034).

Conclusions: Acute/subacute form, older age, lower level of FVC and diagnosis of CADM predict poor outcome in PM/DM/CADM-ILD.

Show MeSH
Related in: MedlinePlus