Very rare: prevalence 5/100,000 ? 

Group of inflammatory diseases of unknown origin characterized by the presence of autoantibodies directed against enzymes involved in the translation of tRNA-proteins (anti-aminoacyl transfer RNA synthetases) and called 'antisynthetases' (especially anti-JO-1, more rarely: anti-PL-7, anti-PL-12, anti-JE, anti-OJ or anti-KS).

The sex ratio (women to men) is 3/2.

Clinical signs:

-         inflammatory myopathy (polymyositis or dermatomyositis): mostly proximal, bilateral and symmetrical; very frequently associated with an interstitial pneumonitis (80%), which can evolve to fibrosis: cough, dyspnea, sometimes dysphagia (motility disorder of the lower third of the  esophagus ) sometimes: myocarditis with pericardial effusion

-         polyarthritis

-         Raynaud's phenomenon but rarely severe

-         pigmented hyperkeratosis  associated with fissures of the glabrous skin of the hands: 'mechanic's hands ".

-         fever.

Treatment: corticosteroids and in case of loss of effect, immunoglobulins or immunosuppressants.

In case of serious pulmonary damage in young subjects, bipulmonary lung transplantation may be proposed.

Anesthetic implications: 

check lung (restrictive disease, decrease in DLCO) and cardiac function (echocardiography); sequelae of corticosteroid therapy; avoid intraoperative hypothermia. Monitoring of muscle relaxation: variable response to non-depolarizing muscle relaxants due to the decrease in muscle mass and inflammation; in case of acute myositis (high CPK) it is wise to avoid succinylcholine.

References : 

-        Tillie-Leblond I, Colin G, Lelong J, Cadranel J.
Atteintes pulmonaires des polymyosites et dermatomyosites. 
Rev Mal Respir 2006 ; 23 : 671-80.

-         Dieval C, Ribeiro E, Mercié P, Blanco P, Duffau P, Longy-Boursier M. 
Le syndrome des antisynthétases : étude rétrospective à propos d’une série de 14 patients. 
Rev Med Int 2012 ; 33 : 76-9.

Updated: November 2019