A case of digital vasculitis in anti-synthetase syndrome (Anti-OJ subtype)

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.

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Publication Details

Journal
BMC Rheumatology
Published
2025-03-27
DOI
https://doi.org/10.1186/s41927-025-00484-0
Primary Topic
Inflammatory Myopathies and Dermatomyositis
Type
article
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article

A case of digital vasculitis in anti-synthetase syndrome (Anti-OJ subtype)

Deniz Ak, Richard Stratton
BMC Rheumatology
Inflammatory Myopathies and Dermatomyositis
article

A case of digital vasculitis in anti-synthetase syndrome (Anti-OJ subtype)

Deniz Ak, Richard Stratton
article en

Abstract

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.

BMC RheumatologyVol. 9(1)
The Royal Free Hospital (GB), University College London (GB)
Good health and well-being
Openalex Percentile: Top 91%
Inflammatory Myopathies and Dermatomyositis
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