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Pulseless Systemic Lupus Erythematosus: A Rare Presentation.

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Summary

Systemic lupus erythematosus (SLE) can cause rare medium vessel vasculopathy, affecting arteries like the brachial and radial arteries. This case highlights an unusual presentation of SLE-related vascular disease in a young woman.

Keywords:
Absent pulseslupus erythematosusmedium vessel vasculopathy

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Area of Science:

  • Rheumatology
  • Vascular Medicine
  • Immunology

Background:

  • Vascular disease is a common complication and leading cause of mortality in patients with systemic lupus erythematosus (SLE).
  • Presentations of SLE-related vascular complications can be subtle and require thorough clinical and radiological evaluation.
  • Medium vessel vasculopathy is an uncommon manifestation of SLE.

Observation:

  • A young female presented with classic SLE symptoms including photosensitive malar rash, oral ulcers, intermittent fever, and joint pain.
  • Clinical examination revealed unilateral absence of radial and brachial artery pulses.
  • Laboratory tests confirmed positive antinuclear antibody, anti-Smith antibody, and direct Coomb's test, with a negative antiphospholipid antibody panel.

Findings:

  • Color Doppler flow imaging demonstrated irregular wall thickening and luminal narrowing in the right upper limb arteries.
  • Computed tomography aortogram confirmed extensive wall thickening and luminal narrowing of the right brachial and radial arteries.
  • The findings were consistent with medium vessel vasculopathy.

Implications:

  • This case underscores the importance of considering vasculopathy in SLE patients, even with atypical presentations.
  • Early and accurate diagnosis through comprehensive imaging is crucial for managing SLE-related vascular complications.
  • Recognizing rare manifestations like medium vessel vasculopathy can improve patient outcomes and guide treatment strategies.