A 54 y.o. male presents with shortness of breath

he has a history of nephrogenic systemic fibrosis and hypothyroidism

His CXR appears above. What could be wrong?

The patient had nephrogenic systemic fibrosis from gadolinium diagnosed in 1998, he developed end stage renal disease and underwent a transplant in 1990 that failed.  He had multiple finger osteomyelitis of the R hand and underwent amputations of the fingertips.  He presented 9/21 with maggots in a R lateral ankle wound which was debrided.  He was unable to walk and confined to a wheelchair.

Nephrogenic systemic fibrosis almost always occurs in patient with renal insufficiency who have had imaging studies with gadolinium.  It can be found in the tissues of these patients on biopsy.  The condition was first described in 2008. Since then many more cases have been reported some occurring as late as 8 years after exposure to gadolinium. It has been hypothesized that patients with Hep C are at higher risk because of increased fibroblast growth factor.

maggots were removed from this ankle ulcer leaving a fairly clean wound

Extracutaneous manifestations may also occur.  Visceral fibrosis can involve the heart, lungs, skeletal muscle and other organs.  This contributes to the development of pulmonary hypertension, cardiomyopathy and skeletal muscle weakness.

Our patient was in the ED from 13:19 on 11/29 to 6:21 on 11/30.  He came in complaining of shortness of breath.  He had not missed dialysis and his lactate increased during his ED stay  to 24.9. His troponis increased to 109. He arrested at 6:15 am on 11/30.  He was placed on pressors and sent to an ICU.  He was dialyzed at 15:35 on 11/30.  Following dialysis he arrested and expired.

He was shown to have a very dilated RV on US and PE protocol was done.  It was negative for pneumonia or PE.  The pt had severe pulmonary hypertension and thickened LV probably related to his systemic fibrosis with cardiogenic shock as the probable cause of the arrest.

Knopp E, Cowper S. nephrogenic systemic fibrosis: early recognition  and treatment. Semin Dial 2008;21:123-8.

Cowper S, Kuo P Bucala R. nephrogenic systemic fibrosis and gadolinium exposure: association and lessons for idiopathic fibrosing disorders. Arthritis rheum. 2007;56:3173-5.

Basak P, Jesmajian S. Nephrogenic systemic fibrosis: current concepts. Indian Journal of Dermatol. 2011 Jan-Feb;56(1):59-64.