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Nephrogenic systemic fibrosis: a pathologic study of autopsy cases
Aashiyana F Koreishi1, Rosalynn M Nazarian, Adam J Saenz
1Department of Pathology, James Homer Wright Pathology Laboratories, Massachusetts General Hospital and Harvard Medical School, Boston, USA.
Archives of Pathology & Laboratory Medicine
|December 8, 2009
Summary
Nephrogenic systemic fibrosis (NSF) can affect multiple organs beyond the skin, leading to complications like thromboembolic disease. Autopsy findings reveal widespread fibrosis and calcification in NSF patients.
Area of Science:
- Nephrology
- Pathology
- Radiology
Background:
- Nephrogenic systemic fibrosis (NSF) is a rare, severe condition initially recognized as a skin disorder.
- Autopsy studies suggest multiorgan involvement in NSF.
Observation:
- This study details 4 autopsy cases of NSF patients from Massachusetts General Hospital.
- Key data included renal dysfunction, gadolinium contrast exposure, lab results, and postmortem systemic findings.
Findings:
- Thromboembolic disease and pneumonia were causes of death.
- Systemic involvement included cutaneous manifestations, nephrocalcinosis, and calcification/fibrosis in the dura, thyroid, and heart.
- Soft tissue calcification correlated with hyperparathyroidism.
Implications:
- Thromboembolic events are a significant complication of NSF.
- NSF can lead to characteristic histologic changes in multiple organs, increasing morbidity and mortality.
- This series underscores the diverse systemic manifestations of NSF.
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