Natural history of hepatic sarcoidosis and its response to treatment.
BACKGROUND
Liver involvement in sarcoidosis is variable and can occur in the absence of pulmonary disease. Data on the natural history of hepatic sarcoid and response to therapy are lacking. This study investigates hepatic dysfunction complicating lung disease and significant liver involvement presenting independent of pulmonary sarcoid.


METHODS
One hundred and eighty patients were included in the study. The minimum follow-up was 2 years.


RESULTS
Fifty per cent of the study population had derangement of liver function attributable to hepatic sarcoid. Twenty-three patients (13%) had liver involvement without lung disease. Sixty-three patients were administered corticosteroids; approximately one-third had a complete clinical response, one-third a partial response and one-third showed no response. Fourteen patients (8%) were cirrhotic at presentation, and two progressed to cirrhosis despite steroid therapy. Sixteen patients received a second-line immunosuppressive agent; one-half of these showed a response to treatment augmentation (four patients azathioprine, three patients methotrexate, one patient both drugs). Six patients required liver transplantation, with disease recurrence in one recipient. In four patients, sarcoid as the aetiology of end-stage liver disease was diagnosed only on examination of the explanted liver.


CONCLUSIONS
Sarcoidosis can cause end-stage chronic liver disease, which is often unrecognized until examination of the explanted liver. Response to conventional immunosuppression is variable and unpredictable. Transplantation is feasible and safe in this population but recurrence is possible.
Kennedy
Patrick T F
PT
Department of Gastroenterology, Chelsea and Westminster Hospital, London, UK. patrick.kennedy@ucl.ac.uk
Zakaria
Nada
N
Modawi
Salma B
SB
Papadopoulou
Anthie M
AM
Murray-Lyon
Iain
I
du Bois
Roland M
RM
Jervoise N Andreyev
H
H
Devlin
John
J
D000328:Adult; D000368:Aged; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008103:Liver Cirrhosis / Q000209:etiology; D008107:Liver Diseases / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D012507:Sarcoidosis / Q000175:diagnosis* / Q000628:therapy; D017565:Sarcoidosis, Pulmonary / Q000150:complications; D012720:Severity of Illness Index; D016896:Treatment Outcome
D005938:Glucocorticoids; D007166:Immunosuppressive Agents