Multi system disorder seen only in patients with CKD (GFR <15; rarely with a GFR of 15–29, but FDA currently warns against using gadolinium-based contrast agents in patients with a GFR <30), acute kidney injury, and after kidney transplantation.
Nephrogenic systemic fibrosis was first recognized in hemodialysis patients during the 1990s and there is a strong link to use of contrast agents containing gadolinium. Incidence is estimated around 1–4% in the highest risk (ESRD) population that has received gadolinium, and lower in patients with less severe kidney dysfunction.
Clinical Findings
Nephrogenic systemic fibrosis affects several organ systems, including: the skin, muscles, lungs, and cardiovascular system.
The most common manifestation is a debilitating fibrosing skin disorder that can range from skin-colored to erythematous papules, which coalesce to brawny patches. The skin can be thick and woody in areas and is painful out-of-proportion to findings on examination.
Treatment
Several case reports and series have described benefit for patients after treatment with corticosteroids, photopheresis, plasmapheresis, and sodium thiosulfate. The true effectiveness of these interventions is still unclear.
REFERENCES
Mixed apneas are characterized by absent respiratory effort and airflow in the first section of…
Although rare, the differential diagnosis of hypoventilation and hypercapnia respiratory failure includes hypothyroidism. It is…
B-type natriuretic peptide (BNP) is a hormone created in response to cardiac wall stretch due…
A common finding described on computed tomography (CT) imaging. A disease with a peribronchovascular distribution…
Though often used synonymously; Trapped Lung and Lung Entrapment technically describe separate entities along the…
It is not clearly understood why patients with pulmonary hypertension (PH) develop pericardial effusions. However,…
This website uses cookies.