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Ascending aortic aneurysm in a man with scleroderma.

Robert R Attaran1, David Guarraia

  • 1The University of Arizona Affiliated Hospitals, 1501 N Campbell Avenue, Tucson, AZ 85724, USA. rattaran@email.arizona.edu

Clinical Rheumatology
|March 31, 2006
PubMed
Summary

Scleroderma rarely affects large blood vessels. This case highlights a large ascending aortic aneurysm in a patient with diffuse scleroderma, a condition seldom reported with thoracic aortic aneurysms.

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Area of Science:

  • Cardiovascular Medicine
  • Rheumatology
  • Vascular Surgery

Background:

  • Scleroderma, a connective tissue disease, primarily affects the skin and internal organs.
  • Macrovascular complications, particularly aortic aneurysms, are infrequently reported in scleroderma patients.
  • This study focuses on a rare presentation of scleroderma with a significant aortic aneurysm.

Observation:

  • A 56-year-old male presented with dyspnea and was diagnosed with antibody-negative, rapidly progressive diffuse cutaneous scleroderma.
  • Physical examination revealed sclerosis and induration of extremities, face, thorax, and abdomen.
  • The patient exhibited Raynaud's phenomenon, arthralgia, dysphagia, pulmonary fibrosis, pericardial effusion, and cardiomyopathy.

Findings:

  • A 5.6-cm ascending aortic aneurysm was diagnosed in the patient.

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  • This represents a rare instance of macrovascular involvement in scleroderma.
  • Only one prior English-language report exists detailing a thoracic aortic aneurysm associated with scleroderma.

    • Implications:

    • This case underscores the importance of considering macrovascular complications in scleroderma.
    • Further research is needed to understand the link between scleroderma and aortic aneurysms.
    • Early detection and management of cardiovascular risks in scleroderma patients are crucial.