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A Rare Presentation of Systemic Sclerosis.

Joud Enabi1, Maha Tahir1, Srikanth Mukkera2

  • 1Internal Medicine, Texas Tech University Health Sciences Center, Odessa, USA.

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Summary

Systemic sclerosis (SSc) can affect heart valves, though aortic valve issues are rare. This case highlights SSc-related aortic regurgitation requiring valve replacement, emphasizing early detection for better patient outcomes.

Keywords:
aortic regurgitationautoimmune diseaserheumatologysystemic sclerodermasystemic sclerosis

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Area of Science:

  • Cardiology
  • Rheumatology
  • Autoimmune Diseases

Background:

  • Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by fibrosis affecting various organs.
  • Cardiac involvement is common in SSc, often manifesting as valvular disease, particularly mitral valve dysfunction.

Observation:

  • A 47-year-old male with progressive SSc presented with cardiac complications.
  • A left atrial appendage biopsy confirmed cardiac involvement, showing fibrosis and thickening.

Findings:

  • The patient developed aortic regurgitation secondary to SSc-related cardiac changes.
  • Aortic valve replacement was necessitated due to the severity of the regurgitation.

Implications:

  • This case highlights an uncommon but significant association between Systemic Sclerosis and aortic valve disease.
  • Recognizing this association is crucial for timely diagnosis and management of SSc patients.
  • Prompt identification and treatment can mitigate severe cardiac complications and improve patient prognosis.