Clinical manifestations of anti-synthetase syndrome positive for anti-alanyl-tRNA synthetase (anti-PL12) antibodies: a retrospective study of 17 cases.
OBJECTIVE
To describe the clinical manifestations of the anti-synthetase syndrome (ASS) specifically associated with anti-alanyl-tRNA (anti-PL12) synthetase antibodies.


METHODS
In a retrospective study, 17 patients (eight males, nine females, mean age = 60.3 years) with ASS symptoms confirmed by two consecutive tests (cyto-dot and/or immunoblot, or both), with positive results for anti-PL12 antibodies, were included.


RESULTS
All patients presented with interstitial lung disease (ILD), which was associated with mild myositis in 41% of the cases. RP and general impairment were common, whereas rheumatic and dermatological symptoms were uncommon. Four patients suffered from SS, and four others had an atypical oesophageal involvement. The long-term course was assessable for 10 patients (follow-up of 41.1 months). Five patients required immunosuppressive drugs. Two patients are waiting for a lung transplant because of disproportionate and refractory pulmonary hypertension.


CONCLUSIONS
The severity of anti-PL12 ASS varied because of the constant pulmonary involvement. ILD was the predominant prognosis factor, which was notable in cases associated with pulmonary hypertension.
Internal Medicine Department, CHU Nantes Hotel Dieu, Place Alexis Ricordeau, Nantes Cedex, France. bhervier@yahoo.fr
Baptiste
B
Hervier
Benoit
B
Wallaert
Eric
E
Hachulla
Daniel
D
Adoue
Dominique
D
Lauque
Marie
M
Audrain
Boubou
B
Camara
Bernard
B
Fournie
Bertrand
B
Couret
Pierre-Yves
PY
Hatron
Sylvain
S
Dubucquoi
Mohamed
M
Hamidou
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000412:Alanine-tRNA Ligase / Q000037:antagonists & inhibitors* / Q000276:immunology; D001323:Autoantibodies / Q000276:immunology*; D005260:Female; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000150:complications* / Q000276:immunology; D008297:Male; D008875:Middle Aged; D009220:Myositis / Q000150:complications* / Q000276:immunology; D012189:Retrospective Studies; D013577:Syndrome
D001323:Autoantibodies; D000412:Alanine-tRNA Ligase