Outcome of anti-PL12 positive patients with antisynthetase syndrome

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• 作者：Isabelle Marie¹ ; ^{isabelle.marie@chu-rouen.fr} ; Sé ; verine Josse¹ ; Olivier Decaux² ; Sté ; phane Dominique³ ; Cé ; dric Landron⁴ ; Pascal Roblot⁴ ; Sté ; phane Jouneau² ; Olivier Vittecoq⁵ ; Fabienne Jouen⁶
• 刊名：La Presse M?¡ì|dicale
• 出版年：2013
• 出版时间：June, 2013
• 年：2013
• 卷：42
• 期：6_part_P1
• 页码：e153-e158
• 全文大小：176 K

文摘

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Summary

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.