Interstitial lung disease in polymyositis and dermatomyositis.
OBJECTIVE
To assess prevalence, characteristics, and long-term outcome of interstitial lung disease (ILD) in polymyositis (PM) and dermatomyositis (DM). To determine predictive variables of ILD course in PM/DM, and to define both clinical and biochemical features associated with ILD onset in PM/DM.


METHODS
The medical records of 156 consecutive PM/DM patients in 3 medical centers were reviewed.


RESULTS
Thirty-six PM/DM patients (23.1%) developed ILD. We observed that 19.4% of patients with ILD had resolution of pulmonary disorders, whereas 25% experienced ILD deterioration. Morbidity and mortality rates were as high as 13.9% and 36.4%, respectively, in PM/DM patients with ILD. Parameters of PM/DM that related to ILD poor outcome were identified as follows: Hamman-Rich-like pattern, initial diffusing capacity of carbon monoxide <45%, neutrophil alveolitis, and histologic usual interstitial pneumonia. Additionally, for the group with ILD, polyarthritis, higher values of erythrocyte sedimentation rate and C-reactive protein, presence of anti-Jo-1 antibody, and characteristic microangiopathy were significantly more frequent.


CONCLUSIONS
Our series underlines the high frequency of ILD in PM/DM patients, resulting in increased morbidity and mortality rates. It also indicates that PM/DM patients should routinely be screened for ILD, even those patients without anti-Jo-1 antibody, because 69% of our ILD patients were seronegative for the anti-Jo-1 antibody. Our findings further suggest that PM/DM patients presenting with factors predictive of ILD poor outcome may require more aggressive therapy.
Marie
I
I
Centre Hospitalier Universitaire de Rouen-Boisguillaume, Rouen Cedex, France.
Hachulla
E
E
Chérin
P
P
Dominique
S
S
Hatron
P-Y
PY
Hellot
M-F
MF
Devulder
B
B
Herson
S
S
Levesque
H
H
Courtois
H
H
D000974:Antibodies, Antinuclear / Q000097:blood; D001706:Biopsy; D001799:Blood Sedimentation; D002097:C-Reactive Protein / Q000032:analysis; D003882:Dermatomyositis / Q000276:immunology / Q000401:mortality*; D005260:Female; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000276:immunology / Q000401:mortality* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D017285:Polymyositis / Q000276:immunology / Q000401:mortality*; D011237:Predictive Value of Tests; D011658:Pulmonary Fibrosis / Q000276:immunology / Q000401:mortality / Q000473:pathology; D012189:Retrospective Studies; D012307:Risk Factors; D016019:Survival Analysis; D014638:Vanadates
D000974:Antibodies, Antinuclear; C046750:Jo-1 antibody; C076715:pervanadate; D014638:Vanadates; D002097:C-Reactive Protein