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Outcome of anti-PL12 positive patients with antisynthetase syndrome.[Profil évolutif du syndrome des antisynthétases avec anticorps anti-PL12]

Abstract : OBJECTIVES: The aim of the present study was to assess the outcome in anti-PL12 patients with antisynthetase syndrome (ASS). METHODS: The medical records of anti-PL12 (n=5) patients with ASS were retrospectively analyzed without prior selection. To exclude false-positive patients, we included patients who were successively tested positive for anti-PL12 antibody at least twice by immunodot and/or Western blot. RESULTS: Anti-PL12 patients experienced: myositis (n=2), Raynaud's phenomenon (n=2), mechanic's hands (n=1), joint impairment (n=4), digestive involvement (n=2), and interstitial lung disease (ILD) (n=4). The two patients with myositis exhibited deterioration of muscle manifestations despite therapy. As regards outcome of ILD, patients developed resolution (n=1), stabilization (n=1) or deterioration (n=2) of pulmonary status. One patient died of pyogenic pneumonia. CONCLUSION: Our series underscores that the presence of anti-PL12 antibody is associated with a particular phenotype of ASS characterized by: (1) less frequent although severe/steroid refractory myositis; (2) less common mechanic's hands and calcinosis cutis; (3) both frequent and severe ILD. Taken together, our findings suggest that PM/DM patients should routinely undergo the search for anti-PL12 antibody as this autoantibody appears to impact patients' prognosis. Furthermore, ILD patients with anti-PL12 antibody should routinely undergo clinical screening for underlying ASS.
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https://www.hal.inserm.fr/inserm-00872701
Contributor : Morgane Le Corre <>
Submitted on : Monday, October 14, 2013 - 11:55:42 AM
Last modification on : Wednesday, April 1, 2020 - 12:38:02 PM

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Isabelle Marie, Séverine Josse, Olivier Decaux, Stéphane Dominique, Cédric Landron, et al.. Outcome of anti-PL12 positive patients with antisynthetase syndrome.[Profil évolutif du syndrome des antisynthétases avec anticorps anti-PL12]. La Presse medicale, Paris, Masson et Cie, 2013, 42 (6 Pt 1), pp.e153-8. ⟨10.1016/j.lpm.2012.12.003⟩. ⟨inserm-00872701⟩

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