A 28 y.o. male has lesions in the liver; what could cause this?

Hint; he has hilar adenopathy shown below

sarcoid1.JPG
 hilar adenopathy

hilar adenopathy

Our patient had neurosarcoidosis.  The lesions in the liver and lymph nodes were granulomas.  He had presented initially with progressive leg weakness over 3 months and urinary retention.  The pt had cauda equina syndrome with involvement of the sacral nerve roots on MRI as well as diffuse leptomeningeal enhancement of the entire spine. 5-10% of patients with sarcoid develop neurosarcoidosis.

 

 involvement of sacral roots on MRI

involvement of sacral roots on MRI

Sarcoidosis is a poorly understood disease thought to be an immune response to something; possibly environmental, possibly infectious, possibly genetic, and possibly related to other immune diseases.  It is more common in celiac disease and common variable immune deficiency. The disease has a prevalence of 40/100,000 in the general population but seems to be dependent on race.  It is more common in Sweden and Iceland where the prevalence is 60 per 100,000. The disease is also more common among those of African American descent.  In Japanese patients ophthalmologic and cardiac involvement are more likely.

The presentation is widely variable.  It can present as cranial nerve palsies with vestibulocochlear nerve involvement causing  deafness or vertigo, optic nerve involvement causing  loss of vision, glossopharyngeal nerve involvement causing difficulty swallowing or facial nerve involvement. The granulomas can form in the pituitary and cortex of the brain.  10% of patients presenting with sarcoid present with seizures due to brain involvement.

It can present as mediastinal adenopathy with no other involvement. It can also present as e nodosum which is an inflammation of fat cells causing raised nodules on the shins.

  e nodosum

 e nodosum

Our patient had a biopsy of a lymph node confirming sarcoid and was treated with high dose steroids. His symptoms greatly improved and he was discharged. Long term treatment is anticipated.

 

Joseph FG, Scolding  NJ. Sarcoidosis of the nervous system. 2007 Practical Neurology 7(4) 234-44.

Li Y, Pabst S, Kubisch C, Grohe, Wollnik B. First independent replications study confirms the strong genetic association of ANXA11 with sarcoidosis. Thorx 2010. 65(10) 939-40.

Syed J, Myers R. sarcoid heart disease. Can J Cardiol 2004. 20 (1) 89-93.