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.
Hervier
Baptiste
B
Internal Medicine Department, CHU Nantes Hotel Dieu, Place Alexis Ricordeau, Nantes Cedex, France. bhervier@yahoo.fr
Wallaert
Benoit
B
Hachulla
Eric
E
Adoue
Daniel
D
Lauque
Dominique
D
Audrain
Marie
M
Camara
Boubou
B
Fournie
Bernard
B
Couret
Bertrand
B
Hatron
Pierre-Yves
PY
Dubucquoi
Sylvain
S
Hamidou
Mohamed
M
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