Nephrogenic systemic fibrosis: a pathologic study of autopsy cases
Authors
Koreishi, Aashiyana F.Nazarian, Rosalynn M.
Saenz, Adam J.
Klepeis, Veronica E.
McDonald, Anna G.
Farris, Alton Brad.
Colvin, Robert B.
Duncan, Lyn M.
Mandal, Rajni V.
Kay, Jonathan
UMass Chan Affiliations
Department of Medicine, Division of RheumatologyDocument Type
Journal ArticlePublication Date
2009-12-01Keywords
AdultAged
*Autopsy
Cause of Death
Fatal Outcome
Female
Humans
Male
Middle Aged
Nephrogenic Fibrosing Dermopathy
Pneumonia
Risk Factors
Thromboembolism
Musculoskeletal Diseases
Rheumatology
Skin and Connective Tissue Diseases
Metadata
Show full item recordAbstract
CONTEXT: Nephrogenic systemic fibrosis (NSF) is a rare but serious disorder initially described as a purely dermatologic process. Isolated autopsy reports have described multiorgan involvement by this disease. OBJECTIVE: To further illustrate the varied and systemic involvement of NSF by describing the autopsy experience at the Massachusetts General Hospital. DESIGN: We describe the findings in a series of 4 autopsy cases of patients diagnosed with NSF. This report describes the history of renal dysfunction, exposure to gadolinium-containing contrast agents, specific laboratory parameters, and the extent of systemic involvement identified by postmortem examination. RESULTS: Causes of death included systemic thromboembolic disease (n = 3) and pneumonia (n = 1). Laboratory parameters and type, dose, or timing of gadolinium-containing contrast-agent exposure did not correlate with clinical findings and outcomes. All patients demonstrated cutaneous manifestations of the disease and nephrocalcinosis, with some exhibiting calcification and fibrosis of the dura, thyroid, and heart including the cardiac conduction system, on postmortem examination. Soft tissue calcification was associated with concurrent hyperparathyroidism or high serum parathyroid hormone levels. CONCLUSIONS: Thromboembolic disease can be a significant clinical complication of NSF. Patients with NSF may also develop characteristic histologic features of fibrosis and calcification in multiple organs, with significant morbidity and mortality. This autopsy series highlights the variability of systemic manifestations of NSF.Source
Arch Pathol Lab Med. 2009 Dec;133(12):1943-8. doi: 10.1043/1543-2165-133.12.1943. Link to article on publisher's siteDOI
10.1043/1543-2165-133.12.1943Permanent Link to this Item
http://hdl.handle.net/20.500.14038/48768PubMed ID
19961249Related Resources
Link to Article in PubMedae974a485f413a2113503eed53cd6c53
10.1043/1543-2165-133.12.1943