RT Journal Article
SR Electronic
T1 Differences in clinical features and prognosis of interstitial lung diseases between polymyositis and dermatomyositis.
JF The Journal of Rheumatology
JO J Rheumatol
FD The Journal of Rheumatology
SP 58
OP 64
VO 32
IS 1
A1 Tomoyuki Fujisawa
A1 Takafumi Suda
A1 Yutaro Nakamura
A1 Noriyuki Enomoto
A1 Kyotaro Ide
A1 Mikio Toyoshima
A1 Hiroshi Uchiyama
A1 Ryoji Tamura
A1 Masaaki Ida
A1 Takeshi Yagi
A1 Kazumasa Yasuda
A1 Hitoshi Genma
A1 Hiroshi Hayakawa
A1 Kingo Chida
A1 Hirotoshi Nakamura
YR 2005
UL http://www.jrheum.org/content/32/1/58.abstract
AB OBJECTIVE: To assess the difference in clinical features and prognosis of patients with interstitial lung disease (ILD) comparing polymyositis (PM) and dermatomyositis (DM). METHODS: Medical records of 28 ILD patients with PM/DM (16 PM-ILD, 12 DM-ILD) were reviewed retrospectively. RESULTS: Serum CPK concentrations were significantly higher in PM-ILD than in DM-ILD. Bronchoalveolar lavage analysis showed that the percentages of lymphocytes and eosinophils were significantly higher in DM-ILD than in PM-ILD. Ten patients (5 PM-ILD, 5 DM-ILD) underwent surgical lung biopsy, and 3 (3 DM-ILD) had an autopsy. Nonspecific interstitial pneumonia (NSIP) was found in 7 (4 PM-ILD, 3 DM-ILD) and usual interstitial pneumonia (UIP) in 3 (1 PM-ILD, 2 DM-ILD). Interestingly, diffuse alveolar damage (DAD) was found in 3 patients with DM-ILD, who all died of deterioration of ILD; but no one with PM-ILD had DAD. Corticosteroid treatment alone achieved a favorable response in 6 patients (37.5%) with PM-ILD, but in only one (8.3%) with DM-ILD. Administration of cyclosporine in the early phase of onset benefited 4 corticosteroid-resistant patients with DM-ILD. Conclusively, survival in DM-ILD was significantly worse than that in PM-ILD. CONCLUSION: DM-ILD is more refractory to corticosteroid therapy, resulting in poorer prognosis compared with PM-ILD. These data indicate that intensive therapy, including cyclosporine, should be considered for DM-ILD.