[Antisynthetase syndrome: a report of four cases and literature review].
The antisynthetase syndrome (ASS) includes inflammatory myopathy (polymyositis or dermatomyositis), interstitial lung disease (ILD), arthritis, Raynaud's phenomenon, and mechanic's hands, associated with antibodies against aminoacyl-tRNA-synthetases, the most well-recognized being the anti-Jo1 antibody (anti-histidyl-tRNAsynthetase). We report four cases of antisynthetase syndrome and review the clinical characteristics and prognosis factors dominated by ILD. We report the cases of four women with a mean age of 42 years (28-62 years). The interstitial lung disease was found in four cases and was objectified by CT-scan in all cases. The pulmonary symptoms were consisted of dyspnea (one case) and respiratory distress (one case). The anti-Jo1 antibodies were present in the four patients. The myopathy was concomitant with pulmonary involvement (two cases), preceded it in 6 months (one case) and in the course of evolution and after 1 month (one case). All patients received corticosteroid treatment. The immunosuppressive treatment was necessary for two patients because of the severity of the pulmonary involvement. The outcome was favorable in two patients, partially favorable in a patient who presented pulmonary fibrosis. However, one patient died after developing brain abscesses.
Service de médecine interne, CHU Hédi-Chaker, 3029 Sfax, Tunisie. fetenfrikha@yahoo.fr
F
F
Frikha
N
N
Saidi
M
M
Snoussi
R
R
Ben Salah
M
M
Ben Ayed
E
E
Daoud
Y
Y
Hentati
S
S
Makni
Z
Z
Mnif
T
T
Boudawara
H
H
Masmoudi
Z
Z
Bahloul
D000328:Adult; D000974:Antibodies, Antinuclear / Q000097:blood; D004417:Dyspnea / Q000209:etiology; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D017563:Lung Diseases, Interstitial / Q000175:diagnosis / Q000276:immunology; D008875:Middle Aged; D009220:Myositis / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D012128:Respiratory Distress Syndrome / Q000209:etiology
D000974:Antibodies, Antinuclear; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; C046750:Jo-1 antibody