ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 227

Long Term Outcome of Interstitial Lung Disease in Idiopathic Inflammatory Myopathies and Amyopathic Dermatomyositis

Machiko Mizushima1, Hidehiro Yamada2, Yoshioki Yamasaki1, Masaomi Yamasaki3, Minoru Satoh4 and Shoichi Ozaki5, 1Internal Medicine, St. Marianna University School of Medicine, Kawasaki, Japan, 2Internal Medicine, St. Marianna University, Kawasaki, Japan, 3Internal Medicine, St Marianna University, Yokohama City Seibu Hospital, Yokohama, Japan, 4Medicine, University of Florida, Gainesville, FL, 5Division of Rheumatology and Allergy, Department of Internal Medicine, St. Marianna University School of Medicine, St. Marianna University School of Medicine, Kawasaki, Japan

Meeting: 2012 ACR/ARHP Annual Meeting

Keywords: ,

Session Information

Title: Muscle Biology, Myositis and Myopathies: Clinical and Therapuetic Aspects of Idiopathic Inflammatory Myopathies

Session Type: Abstract Submissions (ACR)

Background/Purpose: The aim of this study was to assess the long term clinical course and outcome of interstitial lung disease (ILD) in polymyositis/dermatomyositis (PM/DM) and to determine predictive factors for the outcome of PM/DM-associated ILD.

Methods: Among 228 patients with IIM and clinically amyopathic dermatomyositis (cADM), 140 patients with ILD were identified by medical records search at our hospital. Pulmonary high-resolution computed tomography (HRCT) scan was available in 93 patients and their clinical features were analyzed. 

Results: Mean follow up period (SD) of the 93 patients was 68 (55) months. Clinical course of ILD was monophasic resolution in 24 patients (25.8%), chronic stable in 30 (32.3%), relapsing in 32 (34.4%) and fatal progressive within one year in 7 (7.5%) (Table 1). Univariate analysis indicated older age (p =0.444), dermatomyositis (p =0.0393), cough (p =0.0167), periungual erythema (p =0.0123), lower values of aldolase (p =0.047) and AST (p =0.031) were associated with the fatal ILD. Female (p =0.0045), concurrent malignancy (p =0.0037), and the absence of anti-aminoacyl-tRNA synthetase (ARS) antibodies (p =0.0292) were associated with the monophasic resolving ILD. Overall 5-years survival rate of PM/DM-associated ILD was 84%. anti-ARS antibodies positivity was associated with chronic & relapsing course of ILD. HRCT findings and its extension were not associated with the clinical course.

Table 1. Clinical characteristics in cADM/PM/DM patients according to clinical course of ILD.

resolved (n=24)

chronic stable (n=30)

chronic relpsing (n=32)

fatal (n=7)

Female (%)

91.7

63.3

50.0

85.7

age at onsea of ILD (SD)

54 (12) 51 (15) 49 (10) 60 (7)

cADM/DM/PM (%)

12.5/62.5/25

10/50/40

6.3/59.4/34.4

0/100/0

periungual erythema (%)

58.3

46.7

37.5

100

malignancy (%)

33.3

6.7

6.3

14.3

anti-ARS antibody positive (%)

20.8

50

56.3

0

CK (SD)

891.7 (1233)

2063.6 (3779) 2314 (2129) 552.6 (414)

aldolase (SD)

17.6 (22) 48.3 (78) 50.2 (49) 7 (1)

KL-6 (SD)

1152.5 (1819) 1956.7 (794) 1133.5 (976) 907.4 (526)

%VC (SD)

77.1 (15) 86.1 (21) 73.2 (15) 79.1 (20)

alveolar to arterial PO2 difference (SD)

18 (13) 30.1 (41.5) 29.4 (31.2) 50.8 (27.1)

Groud-glass opacities  (%)

50    

63.3 53.1 71.4

consolidation (%)

66.7 26.7 53.1 71.4

traction bronchiectasis (%)

95.8 93.3 62.5 71.4

linear opacity (%)

87.5 80 87.5 85.7

honeycombing (%)

0 3.3 93.8 0

follow up period months (SD)

94.3 (52.9) 46.4 (36.7) 90.5 (56.1) 1.9 (1.6)

Conclusion: Patients with IIM-associated ILD presenting with predictive factors for poor outcome may require more aggressive therap.

Disclosure:

M. Mizushima,
None;

H. Yamada,
None;

Y. Yamasaki,
None;

M. Yamasaki,
None;

M. Satoh,
None;

S. Ozaki,
None.

« Back to 2012 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/long-term-outcome-of-interstitial-lung-disease-in-idiopathic-inflammatory-myopathies-and-amyopathic-dermatomyositis/