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Large vessel occlusive disease associated with CREST syndrome and scleroderma
P Youssef1, H Englert, J Bertouch
1Department of Rheumatology, Prince Henry Hospital, Sydney, Australia.
Insights
This study reports on scleroderma patients with severe macrovascular disease despite few risk factors, suggesting a link between scleroderma and large vessel complications.
Area of Science:
- Rheumatology
- Vascular Medicine
- Dermatology
Background:
- Scleroderma and CREST syndrome are autoimmune diseases affecting connective tissues.
- Macrovascular disease is typically associated with traditional vascular risk factors.
Observation:
- Three patients with CREST syndrome and one with diffuse scleroderma presented with severe macrovascular disease.
- These patients had minimal traditional vascular risk factors.
- Macrovascular disease developed over 10 years after initial Raynaud's phenomenon in CREST syndrome patients.
Findings:
- The patients experienced significant morbidity from macrovascular disease in their arms and legs.
- Pathological findings revealed severe arterial narrowing due to acellular material, not atherosclerosis.
- This represents an 11% incidence in female scleroderma patients, a threefold increase over expected rates.
Implications:
- These findings suggest a potential association between CREST syndrome and scleroderma with macrovascular disease.
- This highlights the need for increased vigilance for vascular complications in these patients.
- Further research is warranted to elucidate the underlying mechanisms linking scleroderma to macrovascular disease.
Objectives:
To report the cases of three patients with CREST syndrome and one patient with diffuse scleroderma who had severe macrovascular disease and only minimal vascular risk factors.
Methods:
The medical histories, physical examinations, and results of clinical investigations were reviewed in four patients.
Results:
These four patients had severe morbidity from macrovascular disease of the arms and legs in the presence of minimal underlying vascular risk factors. These patients represent 11% of the women with scleroderma seen at our hospital since 1974. This is a greater than threefold increase above the expected proportion of symptomatic vascular disease seen in population studies. In the patients with CREST syndrome, large vessel disease was first seen more than 10 years after the onset of Raynaud's phenomenon, which was the first manifestation of the disease. A pathological specimen of the ulnar artery from one patient showed severe luminal narrowing by an acellular material with no evidence of atheroma.
Conclusions:
These cases suggest an association of both the CREST syndrome and scleroderma with macrovascular disease.