Ak Deniz, Stratton Richard J
Rheumatology, Royal Free Hospital London, London, UK.
Division of Medicine, University College London, London, UK.
BMC Rheumatol. 2025 Mar 27;9(1):36. doi: 10.1186/s41927-025-00484-0.
Anti-synthetase syndrome is a rare autoimmune disorder characterised by the presence of autoantibodies against aminoacyl transfer RNA synthetases. We report a unique case of a 54-year-old woman with anti-OJ anti-synthetase syndrome, characterised by the atypical occurrence of digital vasculitis in conjunction with the classic manifestations of anti-synthetase syndrome. Our patient presented with digital vasculitis affecting the right third and fourth fingers, rapidly evolving interstitial lung disease of the organising pneumonia subtype, sub-clinical myositis, arthritis and mechanic's hands. Notably, she had no prior history of Raynaud's phenomenon. Serological tests revealed positive anti-OJ antibodies and weakly positive anti-MI2 antibodies. Our patient's condition was managed with intravenous methylprednisolone then after stepped down to prednisolone and mycophenolate mofetil with successful therapeutic response.Current literature primarily highlights Raynaud's phenomenon and vasculopathy-related ischemia, whether occlusive or non-occlusive in anti-synthetase syndrome. This case study identifies digital vasculitis as a distinctive complication of anti-synthetase syndrome, anti-OJ subtype. It emphasises the importance of recognising vascular complications, including vasculitis, even when classic signs like Raynaud's phenomenon are absent. Further research is crucial to fully understand the range of vascular manifestations associated with anti-synthetase syndrome.
抗合成酶综合征是一种罕见的自身免疫性疾病，其特征是存在针对氨酰基转移RNA合成酶的自身抗体。我们报告了一例独特的54岁女性抗OJ抗合成酶综合征病例，其特征是在抗合成酶综合征的典型表现基础上出现了非典型的指端血管炎。我们的患者表现为累及右手第三和第四指的指端血管炎、迅速进展的机化性肺炎亚型间质性肺病、亚临床型肌炎、关节炎和技工手。值得注意的是，她既往无雷诺现象病史。血清学检查显示抗OJ抗体阳性和抗MI2抗体弱阳性。我们的患者先接受静脉注射甲泼尼龙治疗，之后逐渐减量至泼尼松龙和霉酚酸酯，治疗反应良好。目前的文献主要强调抗合成酶综合征中雷诺现象以及血管病变相关的缺血，无论是闭塞性还是非闭塞性的。本病例研究确定指端血管炎是抗合成酶综合征抗OJ亚型的一种独特并发症。它强调了即使在没有雷诺现象等典型体征的情况下，认识包括血管炎在内的血管并发症的重要性。进一步的研究对于全面了解抗合成酶综合征相关的血管表现范围至关重要。
