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Pericardial involvement in systemic sclerosis
R J Byers1, D A Marshall, A J Freemont
1Department of Pathological Sciences, University of Manchester.
Insights
Systemic sclerosis frequently causes chronic pericarditis and myocardial fibrosis, suggesting it is a primary cardiac manifestation rather than secondary to kidney disease.
Area of Science:
- Rheumatology
- Cardiology
- Pathology
Background:
- Systemic sclerosis is a multisystem autoimmune disease characterized by fibrosis.
- Cardiac involvement is a significant cause of morbidity and mortality in systemic sclerosis.
- The specific pathology of pericardial involvement in systemic sclerosis requires further elucidation.
Purpose of the Study:
- To investigate the frequency and histological features of pericardial disease in systemic sclerosis.
- To differentiate systemic sclerosis-related pericarditis from other causes, such as uraemic pericarditis.
- To explore the potential role of fibrosis and mast cells in the pathogenesis of cardiac involvement.
Main Methods:
- Histological examination of pericardial autopsy sections from 44 systemic sclerosis patients and 19 controls.
- Staining techniques included haematoxylin and eosin, acid toluidine blue, and elastic van Gieson.
- Quantification of mast cells and fibrosis using mast cell counts and Chalkley scoring.
Main Results:
- Chronic pericarditis was observed in 77.5% of systemic sclerosis cases, compared to 1 control.
- Increased pericardial fibrosis was noted in systemic sclerosis patients; mast cell numbers were similar.
- Myocardial fibrosis was present in 37.5% of systemic sclerosis cases, absent in controls.
Conclusions:
- Pericarditis and myocardial fibrosis are significantly more prevalent in systemic sclerosis.
- These findings suggest pericarditis in systemic sclerosis is a primary cardiac manifestation.
- The results underscore the importance of cardiac evaluation in systemic sclerosis management.
Objective:
To determine the frequency and histological characteristics of pericardial involvement in systemic sclerosis.
Method:
Necropsy sections of pericardium from 44 patients with systemic sclerosis were studied, together with sections from 19 age/sex matched controls. Sections were stained with haematoxylin and eosin, acid toluidine blue, and elastic van Gieson. Mast cells were counted in 10 random high power fields and the degree of fibrosis was quantified using a Chalkley count.
Results:
Chronic pericarditis was seen in 31 (77.5%) of the systemic sclerosis cases, but in only one of the controls. The characteristic changes of uraemic pericarditis were not seen. The degree of fibrosis was greater in those with systemic sclerosis, though numbers of mast cells, thought to be important in fibrogenesis, were similar in both groups. Myocardial fibrosis was seen in 15 (37.5%) of systemic sclerosis cases but in none of the controls.
Conclusion:
The incidence of pericarditis and myocardial fibrosis is much greater than in controls. The results indicate that pericarditis is a primary disease (rather than secondary to uraemia).