Title

Nephrogenic systemic fibrosis: a pathologic study of autopsy cases

UMMS Affiliation

Department of Medicine, Division of Rheumatology

Date

12-1-2009

Document Type

Article

Medical Subject Headings

Adult; Aged; *Autopsy; Cause of Death; Fatal Outcome; Female; Humans; Male; Middle Aged; Nephrogenic Fibrosing Dermopathy; Pneumonia; Risk Factors; Thromboembolism

Disciplines

Musculoskeletal Diseases | Rheumatology | Skin and Connective Tissue Diseases

Abstract

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.

Rights and Permissions

Citation: Arch Pathol Lab Med. 2009 Dec;133(12):1943-8. doi: 10.1043/1543-2165-133.12.1943. Link to article on publisher's site

Related Resources

Link to Article in PubMed

PubMed ID

19961249