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

**Authors:** Deniz AK, Richard J. Stratton

PMC · DOI: 10.1186/s41927-025-00484-0 · BMC Rheumatology · 2025-03-27

## TL;DR

This paper presents a rare case of digital vasculitis in a patient with anti-OJ anti-synthetase syndrome, highlighting a new vascular complication.

## Contribution

The paper introduces digital vasculitis as a novel and atypical manifestation of anti-OJ anti-synthetase syndrome.

## Key findings

- Digital vasculitis occurred in a patient with anti-OJ anti-synthetase syndrome without Raynaud’s phenomenon.
- The patient showed successful response to treatment with corticosteroids and mycophenolate mofetil.
- The case suggests the need for increased awareness of vascular complications in anti-synthetase syndrome.

## 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.

## Linked entities

- **Chemicals:** methylprednisolone (PubChem CID 6741), prednisolone (PubChem CID 5755), mycophenolate mofetil (PubChem CID 5281078)
- **Diseases:** anti-synthetase syndrome (MONDO:0019344), interstitial lung disease (MONDO:0015925), organising pneumonia (MONDO:0015264), arthritis (MONDO:0005578)

## Full-text entities

- **Diseases:** vasculopathy (MESH:D000090122), anti-OJ anti-synthetase syndrome (MESH:D016736), autoimmune disorder (MESH:D001327), Anti-synthetase syndrome (MESH:D020159), Raynaud's phenomenon (MESH:D011928), interstitial lung disease (MESH:D017563), organising pneumonia (MESH:D011014), Anti-OJ (MESH:D006679), vascular complications (MESH:D003925), digital vasculitis (MESH:D014657), arthritis (MESH:D001168), ischemia (MESH:D007511), myositis (MESH:D009220)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

3 figures with captions in the complete paper: https://tomesphere.com/paper/PMC11948678/full.md

## References

3 references — full list in the complete paper: https://tomesphere.com/paper/PMC11948678/full.md

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Source: https://tomesphere.com/paper/PMC11948678