[Idiopathic inflammatory myopathies with anti-PM-Scl antibodies: case series and literature review].
OBJECTIVE
The objectives of this study were to evaluate: (1) the prevalence of anti-PM-Scl antibodies within the framework of antinuclear antibodies detection; and (2) the clinical features and outcome of patients with isolated polymyositis/dermatomyositis.


METHODS
Nine thousand and sixty-four consecutive antinuclear testing data allowed us to evaluate anti-PM-Scl antibody prevalence. Second, we also assessed the characteristics of patients with isolated dermatomyositis/polymyositis and associated anti-PM-Scl antibody.


RESULTS
Over 9064 consecutive antinuclear samples tested for antinuclear antibodies, 3263 (36%) were positive; anti-PM-Scl antibody were positive in nine patients: 0.1% of all sera, 0.2% of sera positive for antinuclear antibodies, 1.2% of sera positive for anti-ENA antibodies. Four of the nine patients with anti-PM-Scl antibody had dermatomyositis (n=3) and polymyositis (n=1). Patients with dermatomyositis/polymyositis and anti-PM-Scl antibody exhibited severe complications, as follows: ventilatory insufficiency (n=2) requiring mechanical ventilation in one case, esophageal involvement requiring enteral feeding (n=1); also, two of these patients had cancer.


CONCLUSIONS
Our case series suggests that the presence of anti-PM-Scl antibody is not a favorable prognostic factor in patients with dermatomyositis/polymyositis. This type of antibody appears to be associated with lung and esophageal involvement; in addition, anti-PM-Scl antibody may co-exist with malignancy in PM/DM patients. Taken together, we suggest that patients with dermatomyositis/polymyositis and anti-PM-Scl antibody require both initial evaluation for lung/digestive manifestations and cancer and close surveillance.
Département de médecine interne, CHU de Rouen, 1, rue de Germont, 76031 Rouen cedex, France. isabelle.marie@chu-rouen.fr
I
I
Marie
L
L
Lahaxe
K
K
Tiev
A-B
AB
Duval-Modeste
O
O
Vittecoq
H
H
Levesque
F
F
Jouen
D000293:Adolescent; D000368:Aged; D000906:Antibodies / Q000032:analysis*; D001324:Autoantigens / Q000276:immunology*; D003882:Dermatomyositis / Q000276:immunology*; D005095:Exoribonucleases; D063326:Exosome Multienzyme Ribonuclease Complex; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D017285:Polymyositis / Q000276:immunology*
D000906:Antibodies; D001324:Autoantigens; D005095:Exoribonucleases; D063326:Exosome Multienzyme Ribonuclease Complex; C077044:EXOSC10 protein, human